Gastric peroral endoscopic myotomy for delayed gastric conduit emptying after pharyngolaryngo-esophagectomy: a case report

Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
CASE REPORT
Gastric peroral endoscopic myotomy for delayed gastric conduit emptying after pharyngo-laryngo-esophagectomy: a case report
Fion SY Chan, MB, BS, FHKAM (Surgery)1; Ian YH Wong, MB, BS, FHKAM (Surgery)1; Desmond KK Chan, MB, BS, FHKAM (Surgery)1; Claudia LY Wong, MB, BS, FHKAM (Surgery)1; Betty TT Law, MB, BS, FHKAM (Surgery)1; Velda LY Chow, MS, FHKAM (Surgery)2; Simon Law, PhD, MS1
1 Division of Esophageal and Upper Gastrointestinal Surgery, Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong
2 Division of Head and Neck Surgery, Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong
 
Corresponding author: Prof Simon Law (slaw@hku.hk)
 
 Full paper in PDF
 
Case report
In September 2016, a 64-year-old man with intrathoracic oesophageal cancer underwent neoadjuvant chemoradiotherapy and minimally invasive esophagectomy with no pyloroplasty. The gastric conduit was placed in the posterior mediastinum. Pathology revealed a moderately differentiated squamous cell carcinoma (ypT2N1M0) with clear margins. One year later he underwent surgery for isolated right cervical lymph node recurrence and tolerated a normal diet after surgery with no gastrointestinal symptoms. At 22 months after the second surgery, the patient developed dysphagia and a cervical oesophageal cancer was identified. Completion pharyngo-laryngo-esophagectomy (PLE) with resection of the residual cervical oesophagus, pharyngo-laryngectomy, and reconstruction with a segment of free jejunum interposed between the neopharynx and gastric conduit was performed. After surgery, the patient developed delayed gastric conduit emptying (DGCE) and reported regurgitation of undigested food soon after diet introduction. There was a persistently high nasogastric output, and non-ionic contrast study showed hold-up of contrast at the level of pylorus (Fig 1a). The patient’s symptoms persisted and he relied on nasoduodenal feeding despite prokinetic agents and pyloric balloon dilatation.
 

Figure 1. (a) Contrast study after pharyngo-laryngo-esophagectomy showing contrast hold-up at level of pylorus (yellow arrow). (b) Contrast study after gastric peroral endoscopic myotomy showing free emptying of contrast, with endoclips (red arrow) and pylorus (yellow arrow) visible
 
Gastric peroral endoscopic myotomy (G-POEM) was performed in February 2020, 4 months after completion PLE. The procedure was performed with the patient in a supine position and under general anaesthesia with endotracheal intubation via end tracheostomy. A high-definition gastroscope (GIF-H190; Olympus, Tokyo, Japan) fitted with a conical shaped transparent cap (DH-28GR; Fujifilm, Tokyo, Japan) and carbon dioxide insufflation were used. After submucosal injection of a mixture of normal saline and indigo carmine at the posterior wall of the gastric conduit, 5 cm proximal to the pylorus, a 2-cm longitudinal mucosal incision was made with DualKnife J (Olympus) using Endocut Q mode (effect 3, cut-duration 2, cut-interval 4) [VIO® 300D; Erbe, Tübingen, Germany]. The endoscope entered the submucosal space to dissect a tunnel caudally until the pyloric ring was well exposed (Fig 2a). Pyloromyotomy was performed and the circular muscle ring completely divided and flattened (Fig 2b). Haemostasis was achieved and the mucosal opening closed with repositioning clips (Single Use Hemoclip; Mednova, Zhejiang, China) [Fig 2c]. The surgery time was 120 minutes and rapid contrast passage to the duodenum was demonstrated on postoperative contrast study (Fig 1b). He resumed an oral diet thereafter.
 

Figure 2. Intra-operative photographs showing pyloric ring muscle well exposed (a) before and (b) after pyloromyotomy, and (c) mucosal incision closed with endoclips
 
Discussion
Pharyngo-laryngo-esophagectomy was first reported by Ong and Lee in 19601 and is regarded as standard treatment for hypopharyngeal and cervical oesophageal cancer. Chemoradiotherapy has gained popularity as an alternative therapeutic strategy to preserve the larynx, but salvage PLE due to incomplete response or cancer recurrence is not uncommonly required.2 Post-PLE DGCE is underreported and the incidence is unknown. Patients frequently complain of bloating, regurgitation, and poor oral intake. According to unpublished results from our prospectively collected database, DGCE was documented in five of 20 patients with PLE for cervical oesophageal cancer over the past 10 years. Of these five patients, pyloroplasty was performed in two, of whom symptoms improved with prokinetic agents alone in one, and endoscopic pyloric balloon dilation was required in the other. For those without pyloric drainage, two patients were managed by G-POEM. The remaining patient was an 83-year-old man on prolonged tube feeding who had pneumonia and died 10 weeks after the surgery.
 
The pathogenesis of DGCE after PLE may differ to that after oesophagectomy without pharyngo-laryngectomy although data are lacking. Experience in the management of DGCE after oesophagectomy (without pharyngo-laryngectomy) serves to guide treatment of post-PLE DGCE. Proposed contributing factors include gastropyloric denervation, dysfunctional gastric peristalsis and use of the whole stomach for reconstruction.3 4
 
The application of G-POEM in PLE patients has not been reported. We report a patient with prior oesophagectomy who developed DGCE only after completion PLE. Symptoms resolved after G-POEM. We postulate that removal of the upper oesophageal sphincter in PLE limits build-up of intragastric pressure, compounding DGCE. Pyloromyotomy reduces pyloric channel pressure and expedites gastric emptying, G-POEM accomplishes this as a minimally invasive method. We hypothesise that gastric conduit emptying after PLE can be viewed as a two-stage process. In the first stage, the food bolus passes passively from the proximal stomach to the antrum. In the second stage, food is evacuated from the antrum through the pylorus to the duodenum. A sufficient pressure gradient within the gastric conduit is required to overcome pyloric resistance. Resection of the pharynx and larynx results in equalisation of pressure between the gastric conduit and the atmosphere. The stomach is also exposed to negative intrathoracic pressure. The outflow resistance due to the intact pylorus assumes more importance after PLE since the paretic stomach fails to build up internal pressure. This explains why symptoms of delayed emptying in our patient emerged only after the pharyngo-laryngectomy, not after the initial oesophagectomy.
 
Pyloroplasty and pyloromyotomy have both been shown effective and safe drainage procedures for gastric conduit after oesophagectomy.5 The G-POEM disrupts the pylorus and improves gastric emptying, theoretically achieving the same outcome and serving as a salvage option for DGCE after PLE. We perform G-POEM according to the same principle applied to POEM for achalasia. The submucosal tunnel is dissected close to the muscle layer for precise pyloromyotomy. Secure mucosal closure permits early diet resumption. However, the aim of pyloromyotomy is to overcome the outlet obstruction without alleviating gastroparesis. Despite improved gastric emptying, symptoms of our patient were not completely eliminated. Patients need to make dietary adjustments to accommodate the new conduit over time while maintaining satisfactory nutrition and body weight.
 
To the best of our knowledge, this is the first report of successful management of DGCE after PLE by G-POEM. A pyloric drainage procedure is advocated since resection of the upper oesophageal sphincter, an integral part of PLE, limits pressure build-up and food emptying within the gastric conduit.
 
Author contributions
Concept or design: FSY Chan, S Law.
Acquisition of data: FSY Chan.
Analysis or interpretation of data: FSY Chan.
Drafting of the manuscript: FSY Chan.
Critical revision of the manuscript for important intellectual content: All authors.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
As an editor of the journal, VLY Chow was not involved in the peer review process. Other authors have disclosed no conflicts of interest.
 
Funding/support
This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
Ethics approval
The study was approved by the Institutional Review Board of The University of Hong Kong/Hospital Authority Hong Kong West Cluster (Ref: UW 16-2023). Consent from patient was obtained.
 
References
1. Ong GB, Lee TC. Pharyngogastric anastomosis after oesophago-pharyngectomy for carcinoma of the hypopharynx and cervical oesophagus. Br J Surg 1960;48:193-200. Crossref
2. Tong DK, Law S, Kwong DL, Wei WI, Ng RW, Wong KH. Current management of cervical esophageal cancer. World J Surg 2011;35:600-7. Crossref
3. Konradsson M, Nilsson M. Delayed emptying of the gastric conduit after esophagectomy. J Thorac Dis 2019;11:S835-44. Crossref
4. Akkerman RD, Haverkamp L, van Hillegersberg R, Ruurda JP. Surgical techniques to prevent delayed gastric emptying after esophagectomy with gastric interposition: a systematic review. Ann Thorac Surg 2014;98:1512-9. Crossref
5. Law S, Cheung MC, Fok M, Chu KM, Wong J. Pyloroplasty and pyloromyotomy in gastric replacement of the esophagus after esophagectomy: a randomized controlled trial. J Am Coll Surg 1997;184:630-6.

Pain management for painful brachial neuritis after COVID-19: a case report

Hong Kong Med J 2022 Apr;28(2):178–80  |  Epub 8 Apr 2022
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
CASE REPORT
Pain management for painful brachial neuritis after COVID-19: a case report
Vivian YT Cheung, MB, BS, FHKAM (Anaesthesiology); Fiona PY Tsui, MB, BS, FHKAM (Anaesthesiology); Joyce MK Cheng, BHlthSc, FHKAN (Perioperative)
Department of Anaesthesia, Pamela Youde Nethersole Eastern Hospital, Hong Kong
 
Corresponding author: Dr Vivian YT Cheung (cyt086@ha.org.hk)
 
 Full paper in PDF
 
 
Case report
In October 2020, a 55-year-old Chinese man travelled from Hong Kong to Paris to attend a family funeral. He had psoriatic arthropathy in remission without chronic pain. In November 2020 while still in France, he and seven family members developed fever and upper respiratory symptoms, confirmed to be coronavirus disease 2019 (COVID-19). The family remained in home isolation and required no medical treatment. The patient self-treated with traditional Chinese medication: Lianhua Qingwen herbal capsules for 1 week. Two weeks later, he returned to Hong Kong after testing negative for COVID-19. In December 2020, during mandatory quarantine on re-entry to Hong Kong, the patient suddenly developed pain that extended from the neck and right interscapular region to the shoulder and down along the ulnar side of the right arm and forearm. The patient described the pain as shooting and drilling in nature, constantly severe, worst at the interscapular region, and aggravated by shoulder movement. He also reported disturbed sleep and numbness over his entire right arm and weakened right hand grip. He had no other joint pain, rash, vesicles, or fever. At this time, he was still resting alone in a quarantine hotel and performing no physical work. Most activities of daily living were manageable but some, such as bathing and dressing, were difficult. Diclofenac 100 mg daily and gabapentin 200 mg 3 times daily prescribed at a COVID-19 Clinic were ineffective. The patient’s younger sister who had recovered in France without medication reported similar symptoms in her left arm.
 
The patient presented to our pain clinic 1 month after pain onset. His motor symptoms had spontaneously improved although disturbing right shoulder and interscapular pain with paraesthesia persisted. There were no muscle wasting, scar, rash, or trophic changes. The patient’s right arm was slightly warmer than the left, and upper limb joints were not swollen or tender and there was full range of movement. He reported decreased sensation to light touch, cold and pinprick over the whole right arm, but his sense of vibration and proprioception were preserved. No touch or mechanical allodynia or hyperalgesia were noted. Apart from slightly weakened thumb opposition, other muscle strength, tendon reflexes and neck examination were unremarkable.
 
Analgesia was changed to pregabalin 75 mg twice per day and etoricoxib 90 mg daily as needed, and the patient was referred for occupational therapy for grip strengthening. Magnetic resonance imaging (MRI) in March 2021 revealed mild T2 hyperintensity at the right brachial plexus, suggestive of resolving neuritis (Fig). There was also cervical spondylosis without significant intervertebral foraminal narrowing or cord compression. Nerve conduction study in March 2021 was normal. Electromyography was not performed due to good neurological recovery.
 

Figure. Magnetic resonance imaging showing mild T2 hyperintensity at the right brachial plexus
 
At a subsequent 3-month follow-up examination in March 2021, the patient reported continued improvement with little or no pain. He reported only intermittent paraesthesia and mild weakness of his right hand and fingers. As a right-hander, he continued to have trouble turning keys and using chopsticks and pens. He coped with his office work with a speech-to-text converter to minimise keyboard usage. He could manage most household chores, including shopping for groceries, and slept well. He was calm and grieving appropriately for the loss of his mother. Pregabalin was gradually reduced, and he was weaned off etoricoxib.
 
Discussion
The Coronavirus family is known for its neurotropism with 36% of COVID-19 infected patients reporting some form of neurological manifestation.1 Mechanisms involve direct neural infection, and indirect inflammatory and immunological reactions. Other possibilities are targeting of neuronal angiotensin-converting enzyme 2, vasculitis, thrombosis and iatrogenic, such as prone position-related effects or neuropathies.2
 
The pathophysiology of acute brachial neuritis is not well understood but the pre-existence of viral infection supports immunological mechanisms. Affected subjects have more lymphocytic activity to brachial (versus sacral) plexus nerve extracts and increased antibodies to peripheral nerve myelin. A hereditary form with mutation-related deficiency in proteins from the septin family has been identified.2 Our patient took Lianhua Qingwen, a traditional Chinese medicine prepared from 13 herbs, shown to bind to angiotensin-converting enzyme 2 and shorten the course of COVID-19 infection.3 Drug-induced plexopathy, although less likely, remains possible.
 
Acute brachial neuritis is self-limiting but classically presents with excruciating pain at the shoulder, neck and interscapular region, followed by shoulder girdle weakness. Diagnosis is clinical and investigations are supportive. Cervical pathology is the major differential diagnosis but was excluded in our patient by MRI that revealed cervical spondylosis without nerve or cord compression or signal changes. Given the rheumatological history in our case, active autoimmune disease was also possible, but he had no such features.
 
To the best of our knowledge three cases of post-COVID-19 brachial neuritis4 5 6 have been reported but none in Hong Kong. Brachial neuritis is rare with an incidence of only 1.64 cases per 100 000 person-years, and underreporting is expected with isolation and restricted healthcare access during COVID-19. Compared to existing three cases, two cases similarly involved middle-aged men with delayed neuropathic symptoms 2 weeks after COVID-19 confirmation. One had similar symptoms to our patient, whereas the other two had either purely sensory components or solely proximal median nerve involvement. Our case and one existing case demonstrated classical MRI changes. Nerve conduction study in our patient did not demonstrate reduced action potential amplitude in affected nerves, which may have been related to its performance at a later course of the disease. Given the rarity of the entity and its occurrence in our patient and his sister, further research to investigate the role of genetic susceptibility to the acute form is warranted. Management of brachial neuritis is supportive and focused on pain control and functional rehabilitation with physiotherapy and occupational therapy. There is limited evidence that steroids and immunoglobulins will hasten recovery so their use should be balanced against the risk of viral replication. Currently, there are no established guidelines for pain management in patients with or recently recovered from COVID-19. Specific precautions should be taken in pain management of these cases.
 
Paracetamol has limited efficacy for neuropathic pain. Care should be taken for patients with severe COVID-19, because viral-induced cytokine storm can suppress cytochrome P450, increasing the risks of hepatotoxicity. Nonsteroidal anti-inflammatory drugs offer effective analgesia for brachial neuritis by suppressing cyclooxygenase and prostaglandin production. Although there were early concerns about ibuprofen-associated decompensation in patients with COVID-19, this has not been supported by the World Health Organization after data review. Meanwhile, cyclooxygenase-2 selective nonsteroidal anti-inflammatory drugs disturb the thromboxane A2–prostacyclin balance, potentially enhancing thrombotic tendency in patients with COVID-19. Our case illustrates the safe use of cyclooxygenase-2 inhibitors in a patient recently recovered from COVID-19. Among antineuropathic agents, gabapentinoids have relatively few adverse effects, lower cardiac toxicity, and fewer drug-drug interactions than tricyclic antidepressants and serotonin-noradrenaline reuptake inhibitors. Our patient was initially prescribed a relatively low dose of gabapentin that may account for its lack of effect. He was changed to pregabalin at a higher equivalent dose, with a better pharmacological profile with linear dose-response relationship and faster onset. Physicians should be alert to the sedative effects of analgesia that may worsen COVID-19-related ventilatory impairment. Opioids should be reserved for severe refractory pain.
 
Pain management for patients with or recently recovered from COVID-19 can be socially challenging. The need for quarantine delays presentation and management, and the associated mental stress and lack of social support may perpetuate pain. Although telemedicine enables remote medical care, controversies remain, and psychological engagement is less effective. Our patient’s appropriate grief reaction and illness coping mechanism minimises risk of chronic pain.
 
Our case report is the first to focus on the clinical management of brachial neuritis in patients with or recently recovered from COVID-19, and the first to identify a possible case series within a family. We hope our report of COVID-19-related brachial neuritis can promote awareness and understanding. Future research should focus on its pathophysiology including genetic susceptibility. Whether COVID-19 vaccination alters the course of acute brachial neuritis warrants further observation.
 
Author contributions
Concept or design: VYT Cheung, FPY Tsui.
Acquisition of data: VYT Cheung, JMK Cheng.
Analysis or interpretation of data: VYT Cheung.
Drafting of the manuscript: VYT Cheung.
Critical revision of the manuscript for important intellectual content: FPY Tsui, JMK Cheng.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Acknowledgement
We would like to thank Dr Annie Chu for contributing to the clinical management, and Drs Mandy Au Yeung and Kendrick Tang for the investigations.
 
Funding/support
This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
Ethics approval
The patient was treated in accordance with the Declaration of Helsinki. The patient provided informed consent for the treatment/procedures, and for publication.
 
References
1. Mao L, Jin H, Wang M, et al. Neurologic manifestations of hospitalized patients with coronavirus disease 2019 in Wuhan, China. JAMA Neurol 2020;77:683-90. Crossref
2. Fernandez CE, Franz CK, Ko JH, et al. Imaging review of peripheral nerve injuries in patients with COVID-19. Radiology 2021;298:E117-30. Crossref
3. Alam S, Sarker MM, Afrin S, et al. Traditional herbal medicines, bioactive metabolites, and plant products against COVID-19: update on clinical trials and mechanism of actions. Front Pharmacol 2021;12:671498. Crossref
4. Mitry MA, Collins LK, Kazam JJ, Kaicker S, Kovanlikaya A. Parsonage-turner syndrome associated with SARS-CoV2 (COVID-19) infection. Clin Imaging 2021;72:8-10. Crossref
5. Siepmann T, Kitzler HH, Lueck C, Platzek I, Reichmann H, Barlinn K. Neuralgic amyotrophy following infection with SARS-CoV-2. Muscle Nerve 2020;62:E68-70. Crossref
6. Cacciavillani M, Salvalaggio A, Briani C. Pure sensory neuralgic amyotrophy in COVID-19 infection. Muscle Nerve 2021;63:E7-E8. Crossref

COVID toe in an adolescent boy: a case report

Hong Kong Med J 2022 Apr;28(2):175–7  |  Epub 17 Mar 2022
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
CASE REPORT
COVID toe in an adolescent boy: a case report
Joshua SC Wong, MB, BS, FHKAM (Paediatrics)1 †; TS Wong, MB, ChB, MRCPCH1 †; Gilbert T Chua, MB, BS, FHKAM (Paediatrics)2 †; Christy Wan, MB, BS1; SH Lau, MB, BS1; Samuel CS Ho, MB, BS1; Jaime S Rosa Duque, MD, PhD2; Ian CK Wong, PhD, FRCPCH3,4; Kelvin KW To, MD, FRCPath5; Winnie WY Tso, FHKAM (Paediatrics)2; Christine S Wong, MRCP, FHKCP6; Marco HK Ho, MD, FHKAM (Paediatrics)2; Janette Kwok, PhD, FRCPA7; CB Chow, MD, FHKAM (Paediatrics)1; Paul KH Tam, FRCS, FRCPCH8,9; Godfrey CF Chan, MD, FRCPCH,2; WH Leung, MD, PhD2; YL Lau, MD, FRCPCH2; Patrick Ip, MPH, FHKAM (Paediatrics)2; Mike YW Kwan, MSc (Applied Epidemiology) CUHK, FHKAM (Paediatrics)1
1 Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong
2 Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong
3 Centre for Safe Medication Practice and Research, Department of Pharmacology and Pharmacy, The University of Hong Kong, Hong Kong
4 Research Department of Practice and Policy, UCL School of Pharmacy, University College London, United Kingdom
5 Department of Microbiology, Carol Yu Centre for Infection, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong
6 Dermatology Division, Department of Medicine, Queen Mary Hospital, Hong Kong
7 Division of Transplantation and Immunogenetics, Department of Pathology, Queen Mary Hospital, Hong Kong
8 Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Hong Kong
9 Dr Li Dak-Sum Research Centre, The University of Hong Kong–Karolinska Institutet Collaboration in Regenerative Medicine, The University of Hong Kong, Hong Kong
Co-first authors
 
Corresponding author: Dr Mike YW Kwan (kwanyw1@ha.org.hk)
 
 Full paper in PDF
 
 
Case report
In July 2020, a 17-year-old Pakistani boy presented with pain in his right foot unrelated to trauma or insect bite, after returning from Pakistan. The following day he tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. He had no previous medial history but was obese with a body mass index of 32.4 kg/m2. He denied any camping, water-trekking, or outdoor barefoot walking while in Pakistan. Physical examination revealed multiple purplish, flat, dry lesions <5 mm in diameter on his right toes and dorsum of the foot. They were tender on palpation but there was no surrounding erythema (Fig). No lesions were evident on the left foot or elsewhere and he had no symptoms or signs suggestive of any systemic autoimmune disorder. Pain associated with the lesions subsided by day 4 of illness. Some lesions spontaneously resolved but some became raised and crusted after day 3 (Fig). Topical fusidic acid for 1 week was prescribed to treat any potential bacterial infection. Of note, he developed a fever up to 39°C on day 8 and a productive cough. Vital signs remained stable with no respiratory distress or need for oxygen therapy. Chest X-ray did not show pneumonic changes and fever subsided within 24 hours. White blood cell count and differential were normal and C-reactive protein was 28 mg/L. Alanine aminotransferase was initially elevated at 131 U/L but showed a downward trend on rechecking. Clotting profile and D-dimer were normal. On day 12, SARS-COV-2 immunoglobulin G was detected and the patient was discharged from the hospital. His toe lesions resolved completely a few days later.
 

Figure. Clinical photos of COVID toes. (a) Reticular erythema and chilblain-like lesions over the dorsum of the foot and toes: multiple violaceous-erythematous macules and papules evident over the toes. (b) Some lesions were tender and crusted on day 5 of presentation. (Written consent for publication obtained)
 
Discussion
Rash is an uncommon symptom in coronavirus disease 2019 (COVID-19) infection.1 It has been described in Italy where 20% of COVID-19 patients developed cutaneous signs, including erythematous rash and widespread urticarial or vesicular lesions, at disease onset or following hospitalisation. The lesions usually subsided after a few days and there was no correlation with disease severity.2 Cutaneous manifestations included pseudo-chilblain (pernio-like), vesicular eruptions, urticarial lesions, maculopapular eruptions, and livedo or necrosis.2 3
 
Classic chilblains (or pernios) are inflammatory skin lesions that occur on the dorsal surface of the fingers and toes. They form painful and itchy erythematous and oedematous nodules that may ulcerate. They are triggered by cold and usually recur yearly during winter.3 Since March 2020, cases of acral lesions resembling chilblains have been reported across Europe, coinciding with the beginning of the COVID-19 outbreak. These lesions have differed to classic ones, showing an equal sex distribution, absence of obvious triggering factors, and involvement of the feet and distal third of the legs.3 They have been seen more commonly in previously healthy children or adolescents aged >10 years, almost always (74%-100%) on the feet but occasionally on the hands and fingers. The lesions were multiple and varied in size from a few millimetres to centimetres and were described as erythematous, violaceous, swollen, or purpuric. Itchiness and mild pain were frequently reported but required only symptomatic treatment. Lesions started to regress within 12 days to 8 weeks with complete resolution. The appearance of chilblain-like lesions was not thought to be associated with a poor disease outcome.2 3 A major limitation of these reports is that only 11% of cases hospitalised tested positive for SARS-CoV-2 by polymerase chain reaction (PCR), with the remainder untested or testing negative. Some authors have attributed this to the low sensitivity of tests or low viral load in children.3 The pathophysiological relationship between COVID-19 infection and chilblain-like lesions remains poorly understood, but has been hypothesised to be related to type 1 interferonopathies.3
 
Our patient is one of the few reported cases of laboratory-confirmed SARS-CoV-2 infection with chilblain-like lesions. To date, our patient is the only child in Hong Kong to present with SARS-CoV-2 infection as well as so-called “COVID toe”.1 Currently, there are insufficient data to determine a clear relationship between these dermatological symptoms and COVID-19. Rash is a common manifestation of many diseases and may not be associated COVID-19 infection. A recent case series of 17 adolescents in Italy who developed chilblain-like lesions during the first wave of COVID-19 screened negative on SARS-CoV-2 PCR of nasopharyngeal swabs, negative for SARS-CoV-2 immunoglobulin M and immunoglobulin G, and had no viral genome in biopsy specimens. However, this report was limited by its small sample size and did not compare data with an age- and gender-standardised background incidence of chilblains in the population.4 Most patients with dermatological manifestations were not confirmed to be infected with SARS-CoV-2. Another systematic review also concluded that some, but not all paediatric cases, who developed chilblain-like lesions during the COVID-19 pandemic had positive SARS-CoV-2 PCR, serology or viral particles confirmed in electron microscopy.5 Larger-scale epidemiological study is needed to confirm an association between these chilblain-like lesions and COVID-19 infection. Reported manifestations and histological findings were too heterogeneous to ascertain the pathophysiology. Nevertheless, physicians should remain vigilant since dermatological manifestations may be the first or only symptom in patients with COVID-19 infection,2 3 enabling a timely diagnosis of COVID-19 infection to reduce transmission. Physicians should also consider the possibility of coagulopathies and interferonopathies.
 
Author contributions
Concept or design: MYW Kwan, P Ip.
Acquisition of data: C Wan, SH Lau, SCS Ho, JS Rosa Duque.
Analysis or interpretation of data: C Wan, SH Lau, SCS Ho, JS Rosa Duque.
Drafting of the manuscript: JSC Wong, TS Wong, GT Chua.
Critical revision of the manuscript for important intellectual content: ICK Wong, KKW To, WWY Tso, CS Wong, MHK Ho, J Kwok, CB Chow, PKH Tam, GCF Chan, WH Leung, YL Lau.
 
All authors approved the final version of the manuscript and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
 
Funding/support
This study is supported by the Collaborative Research Fund (CRF) 2020/21 and One-off CRF Coronavirus and Novel Infectious Diseases Research Exercises (Ref: C7149-20G). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
 
Ethics approval
The patient was treated in accordance with the Declaration of Helsinki, provided informed consent for the treatment/procedures, and provided consent for publication.
 
References
1. Chua GT, Wong JS, Lam I, et al. Clinical characteristics and transmission of COVID-19 in children and youths during 3 waves of outbreaks in Hong Kong. JAMA Network Open 2021;4:e218824. Crossref
2. Andina D, Belloni-Fortina A, Bodemer C, et al. Skin manifestations of COVID-19 in children: Part 2. Clin Exp Dermatol 2021;46:451-61. Crossref
3. Andina D, Belloni-Fortina A, Bodemer C, et al. Skin manifestations of COVID-19 in children: Part 1. Clin Exp Dermatol 2021;46:444-50. Crossref
4. Discepolo V, Catzola A, Pierri L, et al. Bilateral chilblain-like lesions of the toes characterized by microvascular remodeling in adolescents during the COVID-19 pandemic. JAMA Network Open 2021;4:e2111369. Crossref
5. Koschitzky M, Oyola RR, Lee-Wong M, Abittan B, Silverberg N. Pediatric COVID toes and fingers. Clin Dermatol 2021;39:84-91. Crossref

Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: a case report

Hong Kong Med J 2022 Feb;28(1):76–8  |  Epub 14 Feb 2022
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
CASE REPORT
Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: a case report
Gilbert T Chua, MB, BS, FHKAM (Paediatrics)1 †; Joshua SC Wong, MB, BS, FHKAM (Paediatrics)2 † Jaime Chung, MB, BS2; Ivan Lam, FHKCP, FHKAM (Paediatrics)2; Joyce Kwong, FHKAM (Pathology)3; Kate Leung, FRCPA, FHKAM (Pathology)3; CY Law, PhD, FHKAM (Pathology)4; CW Lam, PhD, FRCP5; Janette Kwok, PhD, FRCPA6; Patrick WK Chu, MPhil6; Elaine YL Au, FRCPA, FHKCPath7; Crystal K Lam, MB, BS7; Daniel Mak, MRCPCH, FHKAM (Paediatrics)2; NC Fong, FRCPCH2; Daniel Leung, PhD (Candidate)1; Wilfred HS Wong, PhD1; Marco HK Ho, MDM, FRCP1; Sabrina SL Tsao, MB, BS, FACC1; Christina S Wong, MRCP, FHKAM (Medicine)8; Jason C Yam, MB, BS, FCOphthHK,9; Winnie WY Tso, FHKAM (Paediatrics)1; Kelvin KW To, MD, FRCPath10; Paul KH Tam, FRCS, FRCPCH11,12Godfrey CF Chan, MD, FRCPCH1; WH Leung, MB, BS, PhD1; KY Yuen, MD, FRCPath10; Vas Novelli, FRCP, FRCPCH13,14; Nigel Klein, PhD13,14; Michael Levin, PhD, FRCPCH15; Elizabeth Whitaker, MRCPCH, PhD16; YL Lau, MD (Hon), FRCPCH1; Patrick Ip, MPH, FHKAM (Paediatrics)1; Mike YW Kwan, MRCPCH, MSc (Applied Epidemiology CUHK)2
1 Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong
2 Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong
3 Haematology Laboratory, Department of Pathology, Princess Margaret Hospital, Hong Kong
4 Division of Chemical Pathology, Department of Pathology, Queen Mary Hospital, Hong Kong
5 Department of Pathology, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong
6 Division of Transplantation and Immunogenetics, Department of Pathology, Queen Mary Hospital, Hong Kong
7 Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, Hong Kong
8 Division of Dermatology, Department of Medicine, Queen Mary Hospital, Hong Kong
9 Department of Ophthalmology and Visual Sciences, Chinese University of Hong Kong, Hong Kong
10 Department of Microbiology, Carol Yu Centre for Infection, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong
11 Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Hong Kong
12 Dr Li Dak-Sum Research Centre, The University of Hong Kong–Karolinska Institutet Collaboration in Regenerative Medicine, The University of Hong Kong, Hong Kong
13 Department of Paediatric Infectious Diseases, Great Ormond Street Hospital for Children, London, United Kingdom
14 Institute of Child Health, University College London, London, United Kingdom
15 Section of Paediatrics, Imperial College London, London, United Kingdom
16 Paediatric Infectious Diseases Department, Imperial College Healthcare NHS Trust, London, United Kingdom
Co-first authors
 
Corresponding author: Dr Mike YW Kwan (kwanyw1@ha.org.hk)
 
 Full paper in PDF
 
Case report
A 10-year-old ethnic-Russian boy was confirmed to have severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) during the second wave of the coronavirus disease 2019 (COVID-19) outbreak in Hong Kong.1 He had a past medical history of coarctation of the aorta with corrective surgery performed at age 2 months. He returned from Russia on 6 June 2020 and his first deep throat saliva specimen saved on arrival at Hong Kong International Airport tested negative for SARS-CoV-2. Four days later, he developed fever, malaise, and headache. On 13 June 2021, he was admitted to our Paediatric Infectious Disease Unit and a new deep throat saliva specimen was positive for SARS-CoV-2. He did not require oxygen during his hospital stay. He was discharged from the hospital after being tested positive for SARS-CoV-2 anti-nucleoprotein immunoglobulin G antibodies 17 days after admission. This complied with the discharge criteria set by the Department of Health, the Government of Hong Kong Special Administrative Region.1
 
On 16 July, 16 days after being discharged, he returned to our Paediatric Infectious Disease Unit with a 2-day history of high fever and right cervical tender lymphadenopathy. Repeat nasal pharyngeal swab for SARS-CoV-2 polymerase chain reaction was negative. He was presumed to have bacterial lymphadenitis and was prescribed intravenous antibiotics but symptoms progressed. Ultrasound of the neck showed evidence of lymphadenitis but no signs of abscess formation. His fever and lymphadenitis persisted for 5 days and he also developed bilateral non-purulent conjunctivitis with peri-limbic sparing, erythematous and cracked lips, strawberry tongue and blanchable erythema over the trunk (Fig). Serial blood tests showed mild thrombocytopenia (trough 110 × 109/L), and raised erythrocyte sedimentation rate (peak 60 mm/Hr), C-reactive protein (peak 102 mg/L; range, <5.0), lactate dehydrogenase (270 U/L; range, <270), ferritin (1568 pmol/L; range, 31-279), highly sensitive troponin I (peak 643 ng/L; range, <21), N-terminal prohormone of brain natriuretic peptide (peak 3213 ng/L; range, <112), and interleukin-6 (IL-6) (peak 480.9 pg/mL; range, <4). Electrocardiogram and echocardiogram were unremarkable. His clinical presentation was compatible with Kawasaki-like disease. Since he had been infected with COVID-19 approximately 4 weeks previously, he was suspected to have PIMS-TS (paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2).2 Other differential diagnoses were excluded but included streptococcal and staphylococcal infection, Epstein–Barr virus infection and infection-related myocarditis. Owing to the rarity of PIMS-TS in East Asia, the clinical team discussed the case with experts from the United Kingdom who concurred with the diagnosis. He was treated with two doses of intravenous immunoglobulin (IVIG) at 2 g/kg/dose as his fever resurged 1 day after the first dose. Fever and other symptoms subsequently subsided after the second dose of IVIG, and serial echocardiograms did not reveal any coronary lesions. Whole exome sequencing performed to look for the possibility of an underlying monogenic immune dysregulation syndrome because of the rarity of this condition was unremarkable.
 

Figure. Clinical photographs showing (a) bilateral conjunctival injection with peri-limbic sparing and strawberry tongue, and (b) blanchable erythema over trunk. (Written consent for publication obtained from the patient’s parents)
 
Discussion
Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 is one of the most severe complications of COVID-19 infection in children. It was initially described in several case series in Europe and North America among children who presented with Kawasaki-like illness and were confirmed or known to have been in contact with another SARS-CoV-2–infected individual.2 3 Kawasaki disease (KD) is most prevalent among East Asians but rare in other parts of the world.3 On the contrary, paediatric cross-sectional clinical studies from East Asia have reported that PIMS-TS is rare among East Asians.1 We present the first, and so far, the only case of PIMS-TS in China. The case was an ethnic Russian boy who showed features of KD approximately 4 weeks after confirmation of SARS-CoV-2 infection. There is no consensus on the treatment regimen at present. In our patient, IVIG alone, instead of steroid or immunomodulators, was effective in treating the condition.
 
The pathophysiology of PIMS-TS remains uncertain. Studies have shown significant clinical and laboratory differences between PIMS-TS and KD, despite some similarities in clinical presentation. Patients with PIMS-TS are generally older than those with KD (median age, 8.3-9 years vs 2.7 years).2 3 They also have a higher white blood cell and neutrophil count and C-reactive protein, and a greater degree of lymphopenia and anaemia and tendency to develop thrombocytopenia in contrast to thrombocytosis in KD. In addition, fibrinogen and troponin levels are more elevated in PIMS-TS.2 These factors are associated with an increased risk of intensive care admission among children with PIMS-TS.2 These findings imply that PIMS-TS is a different entity to KD, with a greater degree of inflammation and myocardial injury. Studies have shown that certain cytokines, such as IL-6, appear to be particularly elevated in patients with PIMS-TS and may be involved in myocardial depression.2 Studies have also suggested that life-threatening COVID-19 pneumonia may be associated with monogenic inborn errors of immunity related to type 1 interferonopathies or type 1 interferon neutralising antibodies.4 Certain human leukocyte antigens, which are prevalent in East Asians but not Caucasians, have been associated with KD.4 However, no genes have been identified to cause PIMS-TS. Future studies will continue to explore the genetic factors related to PIMS-TS and the possible associated leukocyte antigen that explains the ethnic differences in PIMS-TS prevalence.
 
The treatment for PIMS-TS is similar to that for KD. A recent observational study demonstrated that patients who received IVIG and methylprednisolone together were less likely to require second-line biological agents, and were at lower risk of secondary acute left ventricular dysfunction and need for haemodynamic support with a shorter length of stay in the intensive care unit.5 Interleukin-1 and IL-6 receptor monoclonal antibodies have been used as second-line biological agents and have been shown to achieve remission when first-line therapies fail.2 5 Short-term outcomes of PIMS-TS are generally good. Immediate cardiac complications include coronary abnormalities, transient valvular regurgitation and myocardial dysfunction.2 The majority of patients recover without sequelae, but mortality has been reported.2 Data on the long-term outcomes of PIMS-TS are lacking.
 
The PIMS-TS remains a rare disease among East Asian patients.1 Nevertheless, frontline paediatricians in East Asia should remain vigilant when looking after ethnic non-East Asian children with COVID-19 infection in case they develop PIMS-TS after their initial recovery. Paediatricians should advise parents about the symptoms and signs of PIMS-TS so that timely medical consultation can be sought.
 
Author contributions
Concept or design: GT Chua, JSC Wong, P Ip, MYW Kwan.
Acquisition of data: J Chung, I Lam, J Kwong, K Leung, CY Law, CW Lam, J Kwok, PWK Chu, EYL Au, CK Lam, MYW Kwan.
Analysis or interpretation of data: D Mak, NC Fong, D Leung, WHS Wong, MHK Ho, SSL Tsao, CS Wong, JC Yam, WWY Tso, KKW To, PKH Tam, GCF Chan, WH Leung, KY Yuen, V Novelli, N Klein, M Levin, E Whitaker, YL Lau.
Drafting of the manuscript: GT Chua, JSC Wong, I Lam, J Chung.
Critical revision of the manuscript for important intellectual content: All authors.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Funding/support
This work was supported by the Collaborative Research Fund (CRF) 2020/21 and One-off CRF Coronavirus and Novel Infectious Diseases Research Exercises (Ref: C7149-20G). The funding source was not involved in the study design, collection, analysis or interpretation of data; in the writing of the manuscript; or in the decision to submit the manuscript for publication.
 
Ethics approval
The patient was treated in accordance with the Declaration of Helsinki, and the parents of the patient provided informed consent for the treatment and procedures.
 
References
1. Chua GT, Wong JS, Lam I, et al. Clinical characteristics and transmission of COVID-19 in children and youths during 3 waves of outbreaks in Hong Kong. JAMA Netw Open 2021;4:e218824. Crossref
2. Whittaker E, Bamford A, Kenny J, et al. Clinical characteristics of 58 children with a pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2. JAMA 2020;324:259-69. Crossref
3. To KK, Chua GT, Kwok KL, et al. False-positive SARS-CoV-2 serology in 3 children with Kawasaki disease. Diagn Microbiol Infect Dis 2020;98:115141. Crossref
4. Sancho-Shimizu V, Brodin P, Cobat A, et al. SARS-CoV-2-r elated MIS-C: A key to the viral and genetic causes of Kawasaki disease? J Exp Med 2021;218:e20210446.
5. Ouldali N, Toubiana J, Antona D, et al. Association of intravenous immunoglobulins plus methylprednisolone vs immunoglobulins alone with course of fever in multisystem inflammatory syndrome in children. JAMA 2021;325:855-64.Crossref
 

Thoracoscopic repair of congenital oesophageal atresia in a newborn: a case report

Hong Kong Med J 2022 Feb;28(1):73–5  |  Epub 6 Dec 2021
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
CASE REPORT
Thoracoscopic repair of congenital oesophageal atresia in a newborn: a case report
Michelle ON Yu, FCSHK, FHKAM (Surgery)1; Patrick HY Chung, FCSHK, FHKAM (Surgery)1; Mabel Wong, FHKAM (Paediatrics)2; Anne Kwan, FHKAM (Anaesthesiology)3; Yee-Eot Chee, FHKAM (Anaesthesiology)3; Kenneth KY Wong, FCSHK, FHKAM (Surgery)1
1 Department of Surgery, Queen Mary Hospital, Hong Kong
2 Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Hong Kong
3 Department of Anaesthesiology, Queen Mary Hospital, Hong Kong
 
Corresponding author: Dr Patrick HY Chung (chungphy@hku.hk)
 
 Full paper in PDF
 
 
Case report
In July 2021, a 1.5-kg baby girl presented with polyhydramnios on antenatal ultrasound scan at 27 weeks that had resolved by 35 weeks. Physical examination showed no dysmorphic features. She was born by caesarean section at 35 weeks due to discordant growth in a dichorionic diamniotic twin pregnancy. She was intubated at birth but resistance was noted during orogastric tube insertion. Chest X-ray revealed coiling of the gastric tube at the upper pouch of the oesophagus with the presence of intestinal gas, which is a classic feature of type C oesophageal atresia (Fig 1). Echocardiogram revealed several cardiac anomalies including a ventricular septal defect, a moderate patent ductus arteriosus, and a large atrial septal defect.
 

Figure 1. A 1.5-kg baby girl delivered at 35 weeks gestation. Plain radiograph taken at birth showing the orogastric tube (arrow) coiled at the proximal oesophageal pouch with distal bowel gas
 
Thoracoscopic repair of oesophageal atresia was scheduled for the next day following stabilisation. The patient was positioned in a semi-prone position with the right chest slightly elevated. She was put on conventional ventilation without single lung ventilation, as per our practice. A camera port was inserted at the 5th intercostal space and two further 5-mm working ports were inserted under direct vision. Pneumothorax of 3 to 4 mm Hg was created using CO2. The azygous vein was cauterised and divided with monopolar diathermy and the tracheoesophageal fistula readily identified. This was closed by Weck Hem-o-lok (Teleflex; Wayne [PA], United States) and divided (Fig 2). The proximal oesophageal stump was identified and opened with scissors. End-to-end esophago-oesophagostomy was performed using single-layer interrupted 5/0 polydioxanone sutures (Ethicon; Bridgewater [NJ], United States). A 12-Fr chest drain was inserted at the end of the procedure. The operation was uneventful and completed in 145 minutes.
 

Figure 2. The same 1.5-kg baby girl delivered at 35 weeks gestation. Surgical photograph showing the clipping of the tracheoesophageal fistula (arrow)
 
After surgery, the patient was managed in the neonatal intensive care unit according to our usual protocol. On day 1 after surgery she developed sudden profound desaturation with CO2 retention when the endotracheal tube was secured at 8 cm from the upper lip. Bedside bronchoscopy showed a tracheal pouch (fistula remnant) close to the tip of the endotracheal tube. The tip completely entered the pouch with minimal advancement of the endotracheal tube. The endotracheal tube was then withdrawn to 7.5 cm from the upper lip and no further desaturation was noted.
 
A contrast swallow study on day 18 after surgery showed an intact anastomosis. The patient was extubated successfully on day 19 after surgery. Bolus feeding via a feeding tube was established after extubation, and oromotor training was commenced. She was transferred back to the referring hospital for management of her cardiac disorders. At 6 weeks after surgery, she had good weight gain with full oral feeding and no clinical gastroesophageal reflux.
 
Discussion
The introduction of minimally invasive surgery has undoubtedly revolutionised the treatment of many surgical disorders. The role of minimally invasive surgery in common neonatal procedures such as inguinal hernia repair and pyloromyotomy is well established. However, this operative approach in more complex procedures is still limited by various factors including equipment size, surgical expertise, and perioperative support.
 
Among all the neonatal operations, repair of oesophageal atresia is one of the most challenging. In addition to the need for meticulous surgical skill, adequate support from a dedicated neonatal intensive care unit and expert paediatric anaesthetists are equally important. The first successful thoracoscopic repair of oesophageal atresia was reported in 1999.1 A subsequent international multicentre study published 15 years ago further confirmed that thoracoscopic repair of oesophageal atresia was at least as good as traditional thoracotomy.2 However, this operative approach is still not widely practised.
 
In our unit, we started to perform thoracoscopic surgery in 2007. In our early experience reported in 2012,3 we selected patients with a reasonably large body size for minimally invasive surgery. With the accumulation of neonatal operative experience, we became confident performing these operations on smaller-sized babies. Prior to our case, Son et al4 published their experience in thoracoscopic repair of oesophageal atresia in babies <2 kg, and Rothenberg1 reported a successful experience in a 1.2-kg baby. We believe that thoracoscopic repair of oesophageal atresia in neonates can be performed safely in experienced centres with proper case selection.
 
Traditionally, the Spitz classification has been considered the prognostic indicator. Neonates with oesophageal atresia with birth weight <1.5 kg and major cardiac anomalies (as in our patient) are predicted to have only a 50% survival rate with a traditional open surgical approach. However, advances in surgical skills, neonatal care, and anaesthesia combined with the ability to optimise the surgical approach have resulted in improved surgical outcomes and consequent survival rates.1 4
 
The challenges faced in this operation included the patient’s small size and presence of cardiopulmonary complications before and during surgery. The pneumothorax created during thoracoscopy may compress the lung causing difficulty in ventilation and compromise cardiac function, further increasing the complexity of anaesthesia. We overcame this by limiting the pressure to 3 to 4 mm Hg, resulting in less operative space but better patient tolerance. In our opinion, the reduced working space is not a major hurdle for a surgeon competent in minimally invasive surgery. In a baby with congenital heart disease, an anaesthetist who has experience in neonatal thoracic surgery is essential for optimum intra-operative management.
 
Holcomb et al2 demonstrated in a multi-institutional study that there were no significant differences in reported postoperative complication rates between minimally invasive surgery and open repair. A minimally invasive approach has the additional benefits of smaller wounds and less pain. More specifically, thoracoscopic repair allows for clearer magnification. Although CO2 pneumothorax will compress the right lung during surgery, its effect is significantly less than that of manual compression during open surgery. Long-term studies of musculoskeletal problems also reveal a superior outcome for thoracoscopic surgery.1
 
In conclusion, we report a successful thoracoscopic repair of oesophageal atresia in a high-risk neonate with very low birth weight. To the best of our knowledge, this is the smallest baby with oesophageal atresia in Hong Kong to have this operation. While proper case selection to ensure patient safety remains the top priority, small body size should not preclude a thoracoscopic surgical approach. The combined efforts and advances in surgery, anaesthesia and neonatal care are key to success.
 
Author contributions
All authors contributed to the concept or design of the study, acquisition of the data, analysis or interpretation of the data, drafting of the manuscript, and critical revision of the manuscript for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
As the editor of the journal, KKY Wong was not involved in the peer review process for this article. Other authors have no conflicts of interest to disclose.
 
Funding/support
This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
Ethics approval
The patient was treated in accordance with the Declaration of Helsinki. The patient’s parents provided informed consent for all treatments and procedures and provided consent for publication.
 
References
1. Rothenberg SS. Thoracoscopic repair of esophageal atresia and tracheo-esophageal fistula in neonates: evolution of a technique. J Laparoendosc Adv Surg Tech A 2012;22:195-9. Crossref
2. Holcomb GW 3rd, Rothenberg SS, Bax KM, et al. Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis. Ann Surg 2005;242:422-8. Crossref
3. Huang J, Tao J, Chen K, et al. Thoracoscopic repair of oesophageal atresia: experience of 33 patients from two tertiary referral centres. J Pediatr Surg 2012;47:2224-7. Crossref
4. Son J, Jang Y, Kim W, et al. Thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula: is it a safe procedure in infants weighing less than 2000 g? Surg Endosc 2021;35:1597-601. Crossref

Combined pulmonary fibrosis and emphysema: a commonly missed diagnosis

© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
PICTORIAL MEDICINE
Combined pulmonary fibrosis and emphysema: a commonly missed diagnosis
KO Cheung, MBBS, FRCR; CC Chan, FRCR, FHKAM (Radiology)
Department of Radiology, North District Hospital, Hong Kong
 
Corresponding author: Dr KO Cheung (ronald.mbbs@gmail.com)
 
 Full paper in PDF
 
In September 2019, a 79-year-old man was referred to the medical out-patient clinic for assessment of chronic cough and exertional shortness of breath. He was an ex-smoker for more than 10 years and previously worked as a bird market hawker. He stopped working 6 months previously because of coughing. He had no history of chemical or occupational dust exposure and took no drugs associated with pulmonary fibrosis. He had a known history of previous pulmonary tuberculosis. The patient had no history of fever, rash, polyarthralgia or uveitis. He had no family history of autoimmune disease. Physical examination revealed no rash or joint pain and no ulcers. He had full proximal and distal muscle power and no features of autoimmune disorder.
 
Immunological tests revealed rheumatoid factor, <15.9 IU/mL (normal range <15.9IU/mL); anti-proteinase 3, 5.5 RU/mL (normal range <20 RU/mL); and anti-myeloperoxidase, 3.7 RU/mL (normal range <20 RU/mL). Immunological tests were positive for anti-neutrophil cytoplasmic antibodies, but negative for anti-nuclear antibodies, anti-ds DNA, and anti-extractable nuclear antigen. Echocardiogram was not performed.
 
Lung function testing performed in September 2019 revealed a forced expiratory volume in 1 second (FEV1) of 2.23 L (98% predicted), forced vital capacity (FVC) 3.01 L (95% predicted) and FEV1/FVC ratio 74.1% (above predicted). Results of lung function tests suggested that the shortness of breath was likely due to a combination of restrictive and obstructive lung defects (the former plays a dominant role). The patient subsequently underwent chest radiography and computed tomographic (CT) imaging.
 
Chest radiograph in 2020 (Fig 1a) showed coarsened bilateral lower lobe interstitial markings, raising a concern for a superimposed parenchyma process, such as pulmonary oedema or other chronic process such as fibrosis. Reviewing patient’s previous chest radiograph in 2009 (Fig 1b), there was no bilateral lower lobe interstitial markings. This demonstrated that bilateral lower lobe interstitial markings in the 2020 chest radiograph were recent onset.
 

Figure 1. (a) Chest radiograph 2020. Chest radiograph in 2020 showing decreased upper lung markings, typical of the architectural destruction caused by emphysema. There are coarsened bilateral lower lobe interstitial markings. This raises a concern for a superimposed parenchyma process, such as pulmonary oedema or other chronic process such as fibrosis. The patient subsequently underwent highresolution computed tomography thorax to determine the cause of increased basal interstitial markings. (b) Chest radiograph 2009. Previous chest radiograph in 2009 of the same patient showing preserved lung volume and no bilateral lower lobe interstitial markings. There is mild left apical fibrosis, possibly due to previous tuberculosis. This demonstrated that bilateral lower lobe interstitial markings in the 2020 chest radiograph were of recent onset
 
Computed tomography thorax (Fig 2) in 2020 showed centrilobular and paraseptal emphysematous change at bilateral upper zones. There was septal thickening with reticulations, honeycomb formation and mild traction bronchiectasis at basal regions. On the basis of these findings, a radiological diagnosis of pulmonary fibrosis with emphysema was made.
 

Figure 2. High-resolution computed tomography images of the thorax showing (a) centrilobular and paraseptal emphysematous change at bilateral upper zones; (b) at carina level, there is emphysematous change with subpleural reticulations on the left side; and (c) at basal regions, there is septal thickening with reticulations and honeycomb formation. (d) Coronal and (e) sagittal views showing architectural distortion and mild traction bronchiectasis at bilateral basal regions. The radiological diagnosis was combined pulmonary fibrosis and emphysema
 
Pulmonary function testing on 17 January 2020 revealed severely diminished diffusing capacity for carbon monoxide (DLCO) of 35% (predicted: 19.1 mL/mmHg/min, best: 6.6 mL/mmHg/min) and carbon monoxide diffusion coefficient of 41% (predicted: 4.29 mL/mHg/min/L, best: 1.74 mL/mHg/min/L). Results of testing demonstrated no airflow obstruction or significant post-bronchodilator response. The patient’s DLCO and carbon monoxide diffusion coefficient were low, indicating impaired diffusion due to underlying pulmonary fibrosis. Based on his DLCO <80% predicted and FEV1 >80% predicted, cardiopulmonary exercise testing was proposed to determine any need for lung resection.
 
Radiological and clinical significance of combined pulmonary fibrosis and emphysema
Characteristic radiological findings of combined pulmonary fibrosis and emphysema (CPFE) syndrome include upper-lobe emphysema and lower-lobe interstitial fibrotic changes. The emphysema in CPFE includes bullous, paraseptal, and centrilobular changes and is typically distributed in the upper lobes. Fibrotic changes are not typical in emphysema and should prompt further aetiological investigation. Honeycombing refers to CT-detected clustered thick-wall cystic air spaces (3 to 10 mm in diameter, but occasionally as large as 25 mm) that are usually subpleural, peripheral and basal in distribution. Honeycombing indicates interstitial fibrosis. In our patient, bilateral basal honeycombing on CT confirmed end-stage fibrosis as the cause of increased interstitial markings seen on chest radiography.
 
The coexistence of pulmonary fibrosis and emphysema was first noted in 1990 but was not considered a distinct entity until further characterisation 15 years later. There has been increasing recognition that these two processes may coexist in some patients, and this overlapping disorder has often been termed combined emphysema and fibrosis or CPFE. In general, patients with CPFE have preserved FEV1 and FVC, but the diffusion capacity of the lung for carbon monoxide is severely diminished.1
 
Typically, CPFE is more common in men, current or former smokers.2 Some classic features of CPFE include the following:
  • More rapid lung function decline than in patients with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF) alone. In CPFE, due to coexisting fibrosis and emphysema, the restrictive component (fibrosis) can counterbalance the obstructive component (emphysema), resulting in a near normal FEV1/FVC ratio. Nonetheless an isolated severe impaired DLCO on pulmonary function testing offers an important clue to a diagnosis of CPFE.2

  • Increased risk of primary lung malignancy. Lung cancer significantly affects prognosis as lung function may not support surgery or chemotherapy.3

  • In a retrospective study of 61 patients with CPFE, about half of all patients (47%) had concomitant pulmonary hypertension, with a poor prognosis. Survival was 87.5% after 2 years and 54.6% after 5 years. Median survival was 6.1 years.4

  • A complication of CPFE is acute exacerbation (AE-CPFE) that can be attributed to the emphysematous component (AE-CPFE, COPD type) or fibrotic component of CPFE (AE-CPFE, IPF-type) Treatment depends on the predominant underlying type of exacerbation. The prognosis is worse with the above complications.
  •  
    Management of combined pulmonary fibrosis and emphysema
    The mainstay of treatment for patients with CPFE is supportive care. Smoking cessation is definitely indicated for both components of CPFE. Supplemental oxygen therapy may be beneficial, also COPD treatments such as bronchodilators and inhaled steroids. Case reports of patients with CPFE reveal that lung volume reduction (LVR) may be beneficial in cases of advanced emphysema, even without plethysmographic evidence of severe hyperinflation.5 Treatment with antifibrotic drugs, such as pirfenidone and nintedanib, may be effective in CPFE but further trials are awaited.2 There is evidence that nintedanib can decrease the annual rate of decline in FVC in patients with other (non-usual interstitial pneumonia-like) fibrotic patterns as well as those with IPF. Currently, to the best of our knowledge these are not yet available for CPFE.2 Further investigation is needed into future use of antifibrotic drugs for CPFE. Ultimately, lung transplantation is the only cure.
     
    Author contributions
    All authors contributed to the concept, acquisition and interpretation of data, drafting of the manuscript, and revision for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
     
    Conflicts of interest
    All authors declare no conflicts of interest related to the work in this manuscript.
     
    Funding/support
    This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
     
    Ethics approval
    This study was conducted in accordance with the principles outlined in the Declaration of Helsinki. The patient provided written informed consent for all treatments and procedures, and verbal consent for the publication of this study.
     
    References
    1. Cottin V. Combined pulmonary fibrosis and emphysema: bad and ugly all the same? Eur Respir J 2017;50:1700846. Crossref
    2. Hage R, Gautschi F, Steinack C, Schuurmans MM. Combined pulmonary fibrosis and emphysema (CPFE) clinical features and management. Int J Chron Obstruct Pulmon Dis 2021:16:167-77. Crossref
    3. Kitaguchi Y, Fujimoto K, Hanaoka M, Kawakami S, Honda T, Kubo K. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology 2010;15:265-71. Crossref
    4. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005;26:586-93. Crossref
    5. Straub G, Caviezel C, Frauenfelder T, Bloch KE, Franzen D. Successful lung volume reduction surgery in combined pulmonary emphysema and fibrosis without body-plethysmographic hyperinflation—a case report. J Thorac Dis 2018;10 (Suppl 23):S2830-4. Crossref

    Paediatric acute respiratory distress syndrome and haemophagocytic lymphohistiocytosis complications of scrub typhus: a case report

    © Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
     
    CASE REPORT
    Paediatric acute respiratory distress syndrome and haemophagocytic lymphohistiocytosis complications of scrub typhus: a case report
    Ronald CM Fung, MB, ChB, MRCPCH1; Karen KY Leung, MB, BS, MRCPCH1; CC Au, MB, BS, MRCPCH2; KN Cheong, MB, BS, MRCPCH2; Mike YW Kwan, MRCPCH3; Grace KS Lam, MB, BS, MRCPCH2; KL Hon, MB, BS, MD1
    1 Paediatric Intensive Care Unit, Department of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong
    2 Department of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong
    3 Department of Paediatrics and Adolescent Medicine, Prince Margaret Hospital, Hong Kong
     
    Corresponding author: Dr KL Hon (ehon@hotmail.com)
     
     Full paper in PDF
     
    In June 2020, a previously healthy 7-year-old boy presented with a 1-week history of persistent fever. He had an unremarkable medical history, and had gone hiking a week before the onset of his fever. He was initially treated for a presumed viral illness but his condition worsened over the subsequent 2 days. His fever became high and fluctuating, with a peak of 39°C, and he developed dyspnoea without cough. He had no headache or body aches. Physical examination revealed crepitations with diminished breath sounds over both lung fields and tachypnoea with a respiratory rate of 30 per minute. Cervical lymphadenopathy and hepatomegaly were present. There was no eschar. He had distributive shock with hypotension (80/40 mm Hg) and tachycardia (150 beats per minute) and a norepinephrine infusion was commenced. Within one day, type I respiratory failure became evident with increasing oxygen requirement from room air to FiO2 0.4 and continuous positive airway pressure of 6 cmH2O to maintain oxygen saturation above 90%. The PaO2:FiO2 ratio was 232.5 mm Hg. Plain radiograph of the chest revealed bilateral opacities and peribronchial thickening (Fig). Echocardiography and lung ultrasound confirmed normal heart function and the absence of pleural effusion. By definition, the boy was diagnosed with paediatric acute respiratory distress syndrome (PARDS: an acute lung injury occurring within 7 days of a known clinical insult, with acute hypoxaemia of PaO2:FiO2 ratio ≤300 when the child is on non-invasive ventilation, and new infiltrates consistent with acute pulmonary parenchymal disease on chest radiograph, which cannot be explained by acute left ventricular heart failure or fluid overload), where he had progressive respiratory failure and bilateral diffuse infiltration on chest radiography.1 Further blood tests and bone marrow findings met the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH: a life-threatening clinical syndrome of systemic hyperinflammation and progressive immune-mediated organ damage due to excessive immune activation): anaemia (haemoglobin 8.3 g/dL), thrombocytopenia (45 × 109/L), hypertriglyceridaemia (4.5 mmol/L), high ferritin (4467 pmol/L), hypofibrinogenaemia (0.9 g/L), and elevated soluble CD25 (8569 pg/mL).2 Bone marrow aspiration and trephine biopsy showed haemophagocytosis. There was no evidence of Epstein–Barr virus association on immunohistochemical analysis. Orientia tsutsugamushi antibody titre of 512 increased to 4096 (ie, more than a fourfold increase) after 2 weeks. The child was diagnosed with PARDS and secondary HLH associated with scrub typhus infection and prescribed oral doxycycline 50 mg twice daily (~3.7 mg/kg/day) for the scrub typhus. Intravenous dexamethasone 5 g every 12 hours (10 mg/m2/day) was commenced as treatment of HLH with a starting dose as per the HLH-2004 study protocol. He became afebrile within 1 day of commencing treatment and respiratory distress gradually resolved. He was weaned off continuous positive airway pressure ventilation after 3 days. Platelet count rose to >100 × 109/L after 4 days. Ferritin lowered the day after treatment and was within normal range after 2 weeks. The boy completed a 1-week course of doxycycline and dexamethasone was also tapered off in 1 week.
     

    Figure. Plain radiograph of the chest of a 7-year-old boy presenting with paediatric acute respiratory distress syndrome and haemophagocytic lymphohistiocytosis following hiking. Peribronchial thickening at bilateral perihilar and right paracardiac regions, and atelectasis at right lower, left middle and lower zones
     
    Discussion
    Our patient presented with non-specific symptoms of scrub typhus and developed severe complications including PARDS and HLH without the pathognomonic eschar. Scrub typhus is caused by the bacterium O tsutsugamushi and is spread to humans through the bites of infected chiggers, Leptotrombidium mites, that can be both a vector and a reservoir for O tsutsugamushi.3 It is a notifiable disease in Hong Kong with between 7 and 28 cases reported each year over the past 10 years.4 Symptoms of scrub typhus usually begin within 10 days of being bitten and can range from non-specific signs including fever, headache, body aches and rash, to multiorgan failure and death with a median mortality rate of 6% if left untreated.3 Although eschar is pathognomonic for scrub typhus, it is rare among Southeast Asian patients. Laboratory confirmation of the diagnosis usually requires indirect fluorescent antibody test and is the mainstay of serologic diagnosis. Polymerase chain reaction assay of whole blood sample if available can speed the diagnosis.
     
    Acute respiratory distress syndrome is a serious complication of scrub typhus; it has been reported in 4% to 22% of cases,5 6 and can involve over 50% of children who developed secondary HLH associated with scrub typhus infection.7 The pulmonary manifestations vary from bronchitis and interstitial pneumonitis to acute respiratory distress.5 6 Acute respiratory distress has also manifested in many patients with HLH due to other causes and has been reported as the initial manifestation of HLH. Nahum et al8 reported that 7 of 11 children with HLH and multiple organ failure exhibited PARDS after HLH was diagnosed, highlighting the importance of close monitoring and early intervention for children with PARDS and HLH. The presentation of acute respiratory distress syndrome in children differs from that in adults and a consensus on a formal PARDS definition was reached in 2015 by the Paediatric Acute Lung Injury Consensus Conference.1
     
    In some cases, HLH can cause cytokine release syndrome, a life-threatening disorder of severe excessive inflammation (hyperinflammation) caused by uncontrolled proliferation of activated lymphocytes, macrophages and secretion of inflammatory cytokines.9 Although rare, O tsutsugamushi is a significant cause of HLH, especially in Asia.7 The HLH-2004 protocol, which includes etoposide, dexamethasone and cyclosporine as the initial therapy, is designed for treatment of patients with primary HLH.2 For patients with secondary HLH, treatment of the underlying infection or malignancy may help control the HLH and avoid the need for cyclosporine and etoposide. Single antibiotic therapy with doxycycline, minocycline, chloramphenicol, azithromycin or clarithromycin has been reported to result in rapid defervescence in patients with HLH associated with scrub typhus.10 Thus, an accurate diagnosis of scrub typhus in patients with HLH can help timely targeted antibiotic therapy with subsequent rapid clinical improvement.
     
    This case illustrates an atypical severe manifestation of scrub typhus presenting with non-specific signs and symptoms resulting in complications including PARDS and HLH. Early diagnosis and treatment with doxycycline are crucial to prevent complications. Physicians should be vigilant for scrub typhus as a potential diagnosis in a child who presents with pyrexia of unknown origin and a history of participation in rural outdoor activities.
     
    Author contributions
    Concept or design: RCM Fung, KKY Leung, CC Au, KL Hon.
    Acquisition of data: RCM Fung, KL Hon.
    Analysis or interpretation of data: All authors.
    Drafting of the manuscript: RCM Fung, KKY Leung, KL Hon.
    Critical revision of the manuscript for important intellectual content: All authors.
     
    All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
     
    Conflicts of interest
    As an editor of the journal, KL Hon was not involved in the peer review process. Other authors have disclosed no conflicts of interest.
     
    Funding/support
    This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
     
    Ethics approval
    The patient was treated in accordance with the tenets of the Declaration of Helsinki. The patient’s parents provided written informed consent for all treatments and procedures and consent for publication.
     
    References
    1. Pediatric Acute Lung Injury Consensus Conference Group. Pediatric acute respiratory distress syndrome: consensus recommendations from the Pediatric Acute Lung Injury Consensus Conference. Pediatr Crit Care Med 2015;16:428-39. Crossref
    2. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31. Crossref
    3. Peesapati N, Rohit T, Sunitha S, Pv S. Clinical manifestations and complications of scrub typhus: a hospital-based study from North Andhra. J Assoc Physicians India 2019;67:22-4.
    4. Center for Health Protection, Department of Health, Hong Kong SAR Government. Number of notifiable infectious diseases by month. 2020. Available from: https://www.chp.gov.hk/en/statistics/data/10/26/43/6896.html. Accessed 25 Jun 2020.
    5. Sankuratri S, Kalagara P, Samala KB, Veledandi PK, Crossref
    6. Kumar Bhat N, Dhar M, Mittal G, et al. Scrub typhus in children at a tertiary hospital in north India: clinical profile and complications. Iran J Pediatr 2014;24:387-92.
    7. Naoi T, Morita M, Kawakami T, Fujimoto S. Hemophagocytic lymphohistiocytosis associated with scrub typhus: Systematic review and comparison between pediatric and adult cases. Trop Med Infect Dis 2018;3:19. Crossref
    8. Nahum E, Ben-Ari J, Stain J, Schonfeld T. Hemophagocytic lymphohistiocytic syndrome: Unrecognized cause of multiple organ failure. Pediatr Crit Care Med 2000;1:51-4. Crossref
    9. Hon KL, Leung KK, Oberender F, Leung AK. Paediatrics: how to manage septic shock. Drugs Context 2021;10:2021-1-5. Crossref
    10. Hon KL, Leung AS, Cheung KL, et al. Typical or atypical pneumonia and severe acute respiratory symptoms in PICU. Clin Respir J 2015;9:366-71. Crossref

    Purtscher-like retinopathy in a patient with lupus: a case report

    © Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
     
    CASE REPORT
    Purtscher-like retinopathy in a patient with lupus: a case report
    Cynthia KL Cheng, MB, ChB1; Kenneth KH Lai, MB, ChB1; Andrew KT Kuk, MB, BS, MRCS1; Tracy HT Lai, FCOphthHK, FHKAM (Ophthalmology)2,3; Sarah T Wang, MB, BS, MRCS1; Simon TC Ko, FRCS (Edin), FHKAM (Ophthalmology)1
    1 Department of Ophthalmology, Tung Wah Eastern Hospital, Hong Kong
    2 Department of Ophthalmology, United Christian Hospital, Hong Kong
    3 Department of Ophthalmology, Tseung Kwan O Hospital, Hong Kong
     
    Corresponding author: Dr Cynthia KL Cheng (chengklc@yahoo.com)
     
     Full paper in PDF
     
    Case report
    In January 2020, a 32-year-old Filipino woman was admitted to the medical unit of our hospital with subacute onset of a facial malar rash, digital vasculitis purpura and alopecia. Systemic lupus erythematosus (SLE) had been newly diagnosed according to the Systemic Lupus International Collaborating Clinics criteria. Initial blood tests revealed an elevated serum anti-ds DNA antibody level, positive anti-SM antibody, low C3 and C4 as well as pancytopenia. Fasting glucose and lipid profile were unremarkable. She was first treated with oral hydroxychloroquine 200 mg daily and oral prednisolone 20 mg daily. One week later, she presented to our ophthalmology clinic with acute-onset painless blurring of vision in her right eye upon waking that morning. Physical examination revealed a best-corrected visual acuity of finger counting at 30 cm in her right eye and 6/6 in her left eye with a right relative afferent pupillary defect. Slit-lamp examination was unremarkable with no anterior chamber inflammation. Fundus examination revealed numerous Purtscher flecken, which are polygonal patches of retinal whitening with distinct border and normal retina in between, across the posterior pole. A pseudo cherry-red spot and confluent retinal whitening could be seen in the right eye secondary to proximal occlusion of the retinal arteries. A few flame-shaped retinal haemorrhages were observed inferior and temporal to the optic disc in the right eye. The right optic disc was mildly pale with sharp margins and the retinal vessels were not tortuous, while the left optic disc is pink (Fig 1).
     

    Figure 1. A 32-year-old woman with systemic lupus erythematosus. Fundus photographs of (a, b) the patient’s right eye showing Purtscher flecken (closed arrows), cotton wool spots (open arrow), and a pseudo cherry-red spot (arrowhead); and (c, d) the patient’s left eye showing no unusual findings
     
    Urgent optical coherence tomography of the right eye showed hyper-reflectivity in the retinal nerve fibre layer and areas of retinal thickening. Fundus fluorescein angiography (FFA) of the patient’s right eye showed capillary non-perfusion corresponding to Purtscher flecken, superior branch retinal arterial occlusion, and peripapillary staining (Fig 2). Optical coherence tomography and FFA of the patient’s left eye were normal. Given the typical fundus appearance and FFA findings, our provisional diagnosis was Purtscher-like retinopathy with branch retinal artery occlusion related to SLE. We advised her medical team to escalate her systemic treatment immediately and she was given intravenous methylprednisolone 500 mg daily for 3 days, followed by oral cyclophosphamide 500 mg for 1 day before switching to oral prednisolone 40 mg daily. Oral hydroxychloroquine 200 mg daily was continued. On high-dose steroids, she developed features of psychosis that subsequently resolved. Magnetic resonance imaging of the brain showed no features typical of central nervous system lupus such as ischaemia and vasculitis. We closely monitored her condition and visual acuity had not improved 1 month after systemic treatment was started, remaining at finger counting at 30 cm. Fundus examination of the right eye after treatment revealed macula oedema and pseudo cherry-red spot, with no signs of optic atrophy. Cotton wool spots in the left eye had resolved. The patient chose to seek further medical attention overseas.
     

    Figure 2. Same patient. (a-i) Fundus fluorescein angiography images of the patient’s right eye showing superior branch retinal artery occlusion (white arrow) and areas of capillary non-perfusion (blue arrowhead). (j) optical coherence tomography of the patient’s right eye showing hyper-reflectivity in the retinal nerve fibre layer (arrow) and areas of retinal thickening (arrowhead)
     
    Discussion
    At least one third of patients with SLE have ophthalmological involvement. Retinal and choroidal pathology are sinister ocular complications of SLE that can lead to permanently impaired visual acuity. Purtscher-like retinopathy is a type of vaso-occlusive retinopathy associated with multiple conditions including connective tissue disorders, pancreatic disease, renal disease, and haematological disease.1
     
    We report this rare case of Purtscher-like retinopathy in a patient with SLE which had a devastating visual outcome despite systemic treatment. In an observational case series of 5688 patients with SLE, eight cases of Purtscher-like retinopathy were diagnosed.1 All patients had received treatment but most had optic atrophy and persistent low visual acuity,1 consistent with the outcome for our patient. Poor visual acuity has also been attributed to presentation with a pseudo cherry-red spot. The prognosis of Purtscher-like retinopathy is better in patients with other underlying diseases. For example, Shahlaee et al2 reported a case of postviral Purtscher-like retinopathy presenting with finger count visual acuity in both eyes. No treatment was given but the patient’s final visual acuity improved to 20/20 with bilateral scotoma on visual field assessment. Another case of Purtscher-like retinopathy in a patient with adult-onset Still’s Disease was reported by Yachoui,3 in which initial visual acuity was 20/20 in the right eye and 20/25 in the left. Oral prednisolone 60 mg daily was initiated and the patient’s final visual acuity was 20/25 in both eyes with evidence of established bilateral optic atrophy.
     
    In a retrospective case control study, Gao et al4 reported a 0.66% prevalence of retinal vasculopathy among SLE patients with signs ranging from cotton wool spots, retinal vascular attenuation to retinal haemorrhages. Lupus retinopathy can result in severe complications such as neovascularisation, macula oedema and retinal vessel occlusion resulting in vision loss.4 It is known that patients with high Systemic Lupus Erythematosus Activity Disease Index score are at higher risk of Purtscher-like retinopathy, central nervous system lupus1 and vaso-occlusive retinopathy, reflecting severe systemic microangiopathy.1 4
     
    Because patients with active lupus are at risk of Purtscher-like retinopathy that can lead to severe irreversible visual loss,1 it is important to raise awareness in patients with active lupus and visual symptoms. A high index of suspicion for retinopathy is needed for those with very active disease and prompt referral to an ophthalmologist is warranted in the presence of visual symptoms. Despite escalation of treatment after the onset of visual symptoms, our patient’s vision did not recover. Early aggressive treatment to control SLE disease activity is essential to prevent this blinding complication.
     
    References
    1. Wu C, Dai R, Dong F, Wang Q. Purtscher-like retinopathy in systemic lupus erythematous. Am J Ophthalmol 2014;158:1335-1341.e1. Crossref
    2. Shahlaee A, Sridhar J, Rahimy E, Shieh WS, Ho AC. Paracentral acute middle maculopathy associated with post viral Purtshcer-like retinopathy. Retinal Cases Brief Rep 2019;13:50-3. Crossref
    3. Yachoui R. Purtscher-like retinopathy associated with adult-onset still disease. Retin Cases Brief Rep 2018;12:379-81. Crossref
    4. Gao N, Li MT, Li YH, et al. Retinal vasculopathy in patients with systemic lupus erythematous. Lupus 2017;26:1182-9. Crossref

    Custom-made double inner-branched aortic arch endograft for the treatment of mycotic aortic arch aneurysm: a case report

    © Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
     
    CASE REPORT
    Custom-made double inner-branched aortic arch endograft for the treatment of mycotic aortic arch aneurysm: a case report
    Benien JP Hau, BBiomed, MB, BS; YC Chan, MD, FRCS; Stephen W Cheng, MS, FRCS
    Division of Vascular and Endovascular Surgery, Department of Surgery, Queen Mary Hospital, The University of Hong Kong, Hong Kong
     
    Corresponding author: Dr YC Chan (ycchan88@hkucc.hku.hk)
     
     Full paper in PDF
     
    Case report
    A 76-year-old frail man was admitted to hospital with fever and delirium. His medical history was significant for diabetes mellitus, hypertension, and dyslipidaemia. White blood cell count was 9.3 × 109/L and C-reactive protein 18.20 mg/dL. Blood cultures yielded serogroup D Salmonella enteritidis. Computed tomography (CT) angiogram showed an aortic arch aneurysm of 4.7 cm (Fig 1a). Gallium scintiscan confirmed focal uptake at the proximal aortic arch. He developed progressively worsening chest discomfort for the subsequent 3 weeks, and a new CT angiogram showed rapid increase in the aneurysm size to 5.3 cm (Fig 1b).
     

    Figure 1. A 76-year-old man presenting with fever and delirium. Computed tomography (CT) angiograms revealed (a) an aortic arch aneurysm of 4.7 cm (arrow). New CT angiogram showed (b) rapid enlargement to 5.3 cm after 3 weeks (arrow)
     
    A diagnosis of mycotic aortic arch aneurysm was made, and multidisciplinary consultation concluded that he was too frail to undergo conventional open repair. A custom-made double inner-branched aortic arch endograft (Cook Medical, Bloomington [IN], US) was arranged. During the interim, he received intravenous ceftriaxone. The left subclavian artery was revascularised with a left common carotid artery (CCA) to left subclavian artery bypass as a first-stage procedure. The custom-made endograft was available 3 weeks later (Figure 2a,b) and the endovascular procedure was performed accordingly.
     

    Figure 2. (a, b) Photographs of the custom-made double inner-branched aortic arch endograft (Cook Medical, Bloomington [IN], US) showing side branches at 12:30 and 11:30 (arrows). (c) Intraoperative angiogram and (d) completion angiogram showing successful exclusion of the aneurysm and preservation of all supra-aortic branches. The left subclavian and vertebral arteries are perfused by the left common carotid artery to left subclavian artery bypass graft (arrow)
     
    Briefly, all procedures were performed under general anaesthesia in a well-equipped hybrid operating room by experienced endovascular surgeons. The endografts were flushed with heparinised saline multiple times to completely evacuate trapped gas in the sheath. Access was gained through an open groin cutdown exposing the common femoral artery and vein, and bilateral neck cutdowns to expose the right and left CCA. After full systemic heparinisation and under fluoroscopic guidance, a Terumo wire and pigtail catheter were positioned in the left ventricle and exchanged for a double-curved extended Lunderquist Extra-Stiff Wire (Cook Medical). The main stent graft body was then delivered to the aortic arch. The branched endograft was deployed under fluoroscopy, with controlled systolic hypotension by an inferior vena cava occlusion balloon (Coda 46 mm balloon; Cook Medical) introduced via the right femoral vein. Accurate alignment of the orientation markers with the coronary and supra-aortic vessels was essential. After deployment of the main stent graft, the proximal inner branch was sequentially cannulated in a retrograde fashion through the exposed carotid arteries. A custom-made thoracic stent graft extension was used (Cook Medical) to bridge the proximal inner branch and the innominate artery. A similar procedure was repeated for the distal inner branch via the left carotid artery, where a Fluency self-expanding covered stent (CR Bard, Murray Hill [NJ], US) was used to bridge the distal inner branch and the left CCA, taking care not to cover the left CCA to left subclavian artery bypass. The left subclavian artery origin was finally occluded with an Amplatzer vascular plug (St Jude Medical, Saint Paul [MN], US) to prevent endoleak into the sac. Completion angiogram showed successful exclusion of the aneurysm with patent supra-aortic branches (Fig 2c,d).
     
    The patient recovered well without neurological sequelae. He was discharged on postoperative day 13 with lifelong oral ciprofloxacin prescribed. Computed tomography angiogram at 3 months (Fig 3a,b) and 1 year (Fig 3c,d) after surgery showed successful and durable results.
     

    Figure 3. Computed tomography angiograms taken (a, b) 3 months and (c, d) 1 year after endovascular repair showing successful and durable treatment with shrinkage of the aneurysm
     
    Discussion
    To the best of our knowledge, this is the first reported use of a custom-made double inner-branched thoracic aortic endograft for treatment of Salmonella-related mycotic aortic arch aneurysm. In septic and frail patients, a custom-made endovascular device should not generally be first-line treatment as the manufacturing time may take up to a few months. However, in our patient, urgency was emphasised and availability of the stent-graft expedited.
     
    Conventional open repair has always been the gold standard therapy for mycotic aortic aneurysms, but endovascular stent grafts may be a temporising or permanent option in critically ill patients who will not tolerate open surgery.1 2 Custom-made branched thoracic endovascular aortic repair now adds to the armamentarium of options. The use of custom-made inner-branched thoracic endografts is technically challenging and requires endovascular expertise and experience. Meticulous preoperative planning with analysis using the iNtuition workstation (TeraRecon, San Mateo [CA], US) is paramount, with reference to the characteristics of the proximal and distal landing zones in relation to the diameter, angulation, and length of the supra-aortic arteries. The cervical left CCA to left subclavian artery bypass debranching procedure can be a staged or simultaneous procedure. The operative technique is well described in published literature.3 4
     
    As with most endovascular aortic arch repairs, the most feared complication is of stroke that can occur when atherosclerotic or gaseous emboli are released from the arch or proximal supra-aortic branches during wire and graft manipulation, from clamping of carotid arteries, or due to peri-operative fluctuation of blood pressure. Another major concern after stenting the ascending aorta is the risk of retrograde type A dissection, which can be mitigated by controlled hypotension during the deployment of the stent graft. Care must also be taken to not cover the orifices of the coronary arteries with the stent graft.
     
    Off-the-shelf solutions for single and double inner-branched aortic arch endografts are currently under development and were not available at the time of preparation of this case report. The use of a custom-made thoracic endograft is a major development for these patients who would otherwise require traditional open repair, frozen elephant trunk procedure, or hybrid procedures. Frail elderly patients with co-morbidities or prior sternotomies would be denied surgery as they would not tolerate extracorporeal cardiopulmonary bypass with deep hypothermic circulatory arrest. Endovascular experience and technical support are important as the access vessel is remote to the arch. There is often a fine balance between the procedure and maintenance of cerebral perfusion. Comparing non-custom chimney and custom graft for arch pathology, O’Callaghan et al5 showed that mortality was higher in the non-custom group (7% vs 18%), and a trend favouring better durability of fenestrated grafts for sealing and re-intervention rates was noted. All patients should have regular CT surveillance to monitor durability and exclude aneurysm-related complications. Long-term postoperative antibiotic therapy is also important since endovascular options preclude debridement of infected tissue.
     
    This new technique involving a custom-made double inner-branched aortic arch endograft can be considered in patients with mycotic aneurysms of the thoracic arch, with favourable and durable results.
     
    Author contributions
    All authors contributed to the concept or design of the study, acquisition of the data, analysis or interpretation of the data, drafting of the manuscript, and critical revision of the manuscript for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
     
    Conflicts of interest
    All authors have disclosed no conflicts of interest.
     
    Funding/support
    This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
     
    Ethics approval
    The patient was treated in accordance with the Declaration of Helsinki. The patient provided written informed consent for all procedures.
     
    References
    1. Chan YC, Morales JP, Taylor PR. The management of mycotic aortic aneurysms: is there a role for endoluminal treatment? Acta Chir Belg 2005;105:580-7. Crossref
    2. Taylor PR, Chan YC. Endovascular treatment in the management of mycotic aortic aneurysms. In: Thompson MM, Morgan RA, Matsumura JS, Sapoval M, Loftus IM, editors. Endovascular Intervention for Vascular Disease. Principles and Practice. Boca Raton: Taylor & Francis Group; 2008: 235-42.
    3. Fiorucci B, Tsilimparis N, Rohlffs F, Heidemann F, Debus ES, Kölbel T. How to confirm catheterization of inner branches in aortic endografting: The Universal Flush Test. J Endovasc Ther 2017;24:539-41. Crossref
    4. Tsilimparis N, Detter C, Law Y, et al. Single-center experience with an inner branched arch endograft. J Vasc Surg 2019;69:977-85.e1. Crossref
    5. O’Callaghan A, Mastracci TM, Greenberg RK, Eagleton MJ, Bena J, Kuramochi Y. Outcomes for supra-aortic branch vessel stenting in the treatment of thoracic aortic disease. J Vasc Surg 2014;60:914-20. Crossref

    Amoebic liver abscesses with an unusual source: a case report

    © Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
     
    CASE REPORT
    Amoebic liver abscesses with an unusual source: a case report
    Juanita N Chui, BSc (Adv), MD1; Albert KK Chui, FRACS, MD2
    1 School of Medicine, University of Sydney, Sydney, Australia
    2 Private Practice, 12/F, Emperor Commercial Centre, Central, Hong Kong
     
    Corresponding author: Dr Albert KK Chui (akkchui@netvigator.com)
     
     Full paper in PDF
     
    Case report
    In July 2020, a 50-year-old man presented to a public hospital in Hong Kong with a 3-week history of abdominal pain localising to the right upper quadrant, associated with low-grade fever, chills, and fatigue. He denied any diarrhoea or vomiting. His medical history and family history were unremarkable. He was a non-smoker and consumed alcohol infrequently. He had recently returned from Southern California, United States, where he was bitten by a horse 1 month before the onset of his symptoms. Despite sustaining deep wounds to his left neck (Fig 1), he did not seek medical attention at the time.
     

    Figure 1. A 50-year-old man presented with a 3-week history of abdominal pain localising to the right upper quadrant, associated with low grade fever, chills, and fatigue. The patient was bitten by a horse 1 month before onset of symptoms. Clinical photograph of the bite wounds on the neck after blood cleansing
     
    On admission, a contrast-enhanced computed tomography (CT) scan of the abdomen revealed three liver abscesses. He was treated initially with intravenous antibiotic (amoxicillin/clavulanic acid for 5 days before switching to piperacillin/tazobactam) but his clinical condition showed no improvement. A new CT scan 5 days later showed enlargement of the abscesses. Percutaneous drainage of the abscesses was performed under ultrasound guidance and the patient was transferred to our private hospital for further management.
     
    On arrival, the patient was haemodynamically stable but clinically dehydrated, jaundiced, and delirious. The abdomen was soft and non-tender, with three abdominal drainage tubes in situ. A new CT scan confirmed that the liver abscesses and the drainage tubes were blocked, and they had to be replaced (Fig 2). Drainage fluid afterwards was noted to have the appearance of anchovy sauce and amoebic infection was suspected.
     

    Figure 2. Contrast-enhanced computed tomography scans of the patient’s liver after drainage, showing (a) the segment IV abscess (5.9 × 4.9 × 4.2 cm) with indwelling drainage catheter, (b) the segment VIII abscess (4 × 4.3 × 4.2 cm), and (c) the segment V abscess (3.1 × 3.7 × 2.9 cm)
     
    Blood tests on arrival showed leucocytosis (white cell count 31 × 109/L) with markedly elevated inflammatory markers (C-reactive protein 357 mg/L), and deranged liver function (alkaline phosphatase 231 U/L, alanine aminotransferase 64 U/L, bilirubin 56 μmol/L and albumin 26.5 g/L). Blood cultures were negative. No abnormalities were found on colonoscopy and stool ova, cysts and parasite microscopy were negative. Fluid drained from the abscesses was negative on microscopy and culture, but positive for Entamoeba histolytica DNA on polymerase chain reaction. Initial amoebic serology was negative for E histolytica immunoglobulin G antibodies. Intravenous metronidazole was commenced empirically in addition to piperacillin/ tazobactam. The patient subsequently made a steady recovery. New amoebic serology demonstrated a significant titre of E histolytica immunoglobulin G antibodies 9 days later, indicating recent amoebic infection. Repeated blood tests showed continued normalisation of initial results and follow-up CT scans showed progressive resolution of abscesses. The patient was discharged from the hospital after 2 weeks.
     
    Discussion
    Amoebiasis is a leading parasitic infection in terms of morbidity and mortality worldwide. It is most prevalent in developing countries and in tropical regions. It is caused by the protozoa, E histolytica, transmitted via the faecal-oral route by ingestion of contaminated food or water containing amoebic trophozoites or cysts. Acute infection typically presents as amoebic colitis. The most common symptoms associated with amoebiasis are abdominal pain (98% of cases), fever (74%), and dysentery (30%).1 2 Although extraintestinal complications of invasive infection are rare (<1% of cases), liver abscesses are the most common secondary manifestation, occurring when trophozoites invade the colonic mucosa and penetrate mesenteric venules to enter the portal circulation.3
     
    This case was an unusual presentation of amoebic liver abscess, confirmed by polymerase chain reaction testing of aspirates and serology results that suggested an acute infection. However, the patient presented with no gastrointestinal symptoms typical of amoebic colitis and stool investigations and colonoscopy were normal. The typical period of incubation for amoebiasis in those with liver abscesses has been reported to be 8 to 20 weeks.4 In this case, the patient presented with symptoms of invasive disease 4 weeks following his horse bite. The patient denied recent travel to an endemic area or any sick contacts. Southern California is known to have many migrants from South America where amoeba is prevalent. There was little evidence from the patient’s clinical history or investigations to support a faecal-oral route of transmission. As such, the possibility of liver abscess from a cutaneous source was considered.
     
    The patient was initially treated for pyogenic liver abscess. Drainage of the abscesses and the addition of metronidazole dramatically improved his condition. It is conceivable that the horse bite harboured amoebic trophozoites or otherwise facilitated their invasion from contaminated environmental water, soil, or vegetation. Just as invasive trophozoites are known to reach the liver by hematogenous dissemination to form abscesses, in our patient they may have entered the circulation via the bite wound to invade the liver. The short incubation period was also consistent with direct inoculation. Although cutaneous bacterial infection leading to liver pyogenic abscesses is well reported in the literature, the development of amoebic liver abscesses from a similar source has not previously been described. This may be the first described case of amoebic liver abscesses of cutaneous origin and warrants further study.
     
    Author contributions
    Concept or design: AKK Chui.
    Acquisition of data: AKK Chui.
    Analysis or interpretation of data: AKK Chui.
    Drafting of the manuscript: Both authors.
    Critical revision of the manuscript for important intellectual content: Both authors.
     
    All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
     
    Conflicts of interest
    As an editor of the Journal, AKK Chui was not involved in the peer review process for this article. The other author has disclosed no conflicts of interest.
     
    Funding/support
    This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
     
    Ethics approval
    The patient was treated in accordance with the tenets of the Declaration of Helsinki. Patient consent was obtained.
     
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    2. Donovon AJ, Yellin AE, Ralls PW. Hepatic abscess. World J Surg 1991;15:162-9. Crossref
    3. Swaminathan V, O’Rourke J, Gupta R, Kiire CF. An unusual presentation of an amoebic liver abscess: the story of an unwanted souvenir. BMJ Case Rep 2013;2013:bcr2012006964. Crossref
    4. Li E, Stanley SL Jr. Protozoa. Amebiasis. Gastroenterol Clin North Am 1996;25:471-92. Crossref

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