A 72-year-old man with recurrent hepatitis B virus–related hepatocellular carcinoma was referred for right portal vein embolisation (PVE) prior to right hepatectomy. He had Child-Pugh class A cirrhosis, with calculated indocyanine green–R15 of 8%. Portal embolisation was indicated due to the presence of multiple co-morbidities and marginal future liver remnant volume of 35%.

Preprocedural computed tomography revealed duplication of the portal vein (DPV) [Fig 1]. The anatomy and feasibility of the procedure was discussed with hepatic surgeons. Right PVE was successfully performed with n-butyl cyanoacrylate glue. Left hepatic lobe hypertrophy from 430 cm³ to 560 cm³ was achieved. The patient subsequently underwent an uneventful right hepatectomy.

Portal vein embolisation is a commonly adopted strategy to induce future liver remnant hypertrophy prior to hepatectomy. Knowledge of the portal venous anatomy and its variants is vital for treatment planning. Duplication of the portal vein is a rare congenital anomaly that has been described only in case reports. It is related to the spectrum of vitelline vein regression anomaly with pathogenesis believed to be failed regression of the left cranial part of the vitelline vein (Fig 2a-e).1 A variation of DPV has been reported; some authors describe two portal veins arising separately without extrahepatic communications,2 while some describe an additional portal vein arising anomalously from either the superior mesenteric vein or the splenic vein (Fig 2f-i).3 The latter was evident in our patient (Fig 1).

Another anomaly with double channel portal vein is portal vein fenestration in which there is a small fenestration at the mid portion of the main portal vein.4 The exact pathogenesis and its relationship with portal vein duplication remains unknown.

In the presence of DPV, there was altered flow dynamic with preferential opacification of the right or left portal vein branches depending on different catheter tip positions (Fig 3). There was preferential flow towards the left portal branches at the intrahepatic communication at the hepatic hilum, giving a narrow safety margin for embolisation to prevent non-target embolisation of the left portal vein that could jeopardise the future liver remnant.

Our patient successfully underwent PVE without complication. The degree of hypertrophy was similar to that reported in local cohorts.5 Surgeons discussed whether the anomalous portal vein could be embolised to improve the efficacy of PVE but there was also a risk of jeopardising venous return from small branches of the superior mesenteric vein that may worsen liver function.

During hepatectomy, DPV was confirmed (Fig 4). It did not affect surgical planning and the patient underwent right hepatectomy uneventfully.

Duplication of the portal vein is a rare congenital anomaly. Because of the possible altered flow dynamics, it is important to identify this anomaly on preprocedural imaging and arrange multidisciplinary team discussion to plan PVE and ensure a safe and effective procedure.

Concept or design: All authors.
Acquisition of data: All authors.
Analysis or interpretation of data: OL Chan, YS Lee.
Drafting of the manuscript: OL Chan, YS Lee.
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.

All authors have disclosed no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

This study was approved by the Central Institutional Review Board of Hospital Authority, Hong Kong (Ref No.: CIRB-2023-064-1). Written informed consent was obtained from the patient for publication of this article.

1. Qin Y, Wen H, Liang M, et al. A new classification of congenital abnormalities of UPVS: sonographic appearances, screening strategy and clinical significance. Insights Imaging 2021;12:125. Crossref

2. Dighe M, Vaidya S. Case report. Duplication of the portal vein: a rare congenital anomaly. Br J Radiol 2009;82:e32-4. Crossref

3. Kitagawa S. Anomalous duplication of the portal vein with prepancreatic postduodenal portal vein. J Rural Med 2022;17:259-61. Crossref

4. Balradja I, Har B, Rastogi R, Agarwal S, Gupta S. Portal vein fenestration: a case report of an unusual portal vein developmental anomaly. Korean J Transplant 2022;36:298-301. Crossref

5. Yu KC, Wong SS, Wong YC, et al. Procedure time, efficacy, and safety of portal vein embolisation using a sheathless needle-only technique compared with traditional technique. Hong Kong J Radiol 2022;25:35-44. Crossref

A 67-year-old female was admitted to the neurology department in October 2020 with abnormal behaviour and cognitive impairment. Her memory and numeracy had declined, and symptoms progressed over the preceding week. She had a 30-year history of chronic alcohol abuse with an average daily intake of 500-mL liquor.

Routine biochemistry including electrolytes, liver function, and vitamin B12 were within normal limits. Magnetic resonance imaging of the brain showed areas of hyperintensity of the corpus callosum (splenium, body, and genu), bilateral middle cerebellar peduncles, periventricular white matter, and subcortical white matter of the frontal lobe on T2-weighted and fluid-attenuated inversion recovery images. Diffusion-weighted imaging revealed prominent high-intensity signal lesions involving the splenium, and these corresponding lesions were hypointense on the apparent diffusion coefficient map (Fig). Based on her history, physical examination and magnetic resonance imaging features, the patient was diagnosed with Marchiafava–Bignami disease (MBD). Despite a normal level of serum vitamins, the patient was prescribed vitamin B and neurotrophic treatment. Symptoms improved and she made a good recovery over the next 30 days.

Marchiafava–Bignami disease is a rare neurological syndrome characterised by primary degeneration and necrosis of the corpus callosum associated with chronic alcoholism and malnutrition. The clinical manifestations of MBD are severe and nonspecific and include an altered mental state, impaired walking, deficient memory, and dysarthria. Symptoms and imaging findings may improve following thiamine treatment.1 2 The role of magnetic resonance imaging is essential to confirm the diagnosis. Chronic alcohol abuse plays an important role in its development although MBD has been occasionally diagnosed in patients with no history of alcohol abuse, in particular individuals with poorly controlled diabetes mellitus or following surgery.3 4 The aetiology and pathophysiology of MBD remain unclear. Possible mechanisms include cytotoxic oedema, blood-brain barrier breakdown, demyelination, and necrosis. Early pathological manifestations are mainly intramyelinic or cytotoxic oedema. In the later stages, demyelination and necrosis of the corpus callosum (especially in the genu and the body) may follow5 with necrosis leading to atrophy and cavitation in chronic stages, and a decreased number of oligodendrocytes. Occasionally, similar lesions can involve extracallosal regions, such as the anterior and posterior commissures, subcortical white matter, middle cerebellar peduncle, optic chiasm, putamen, internal capsules, hippocampus, and frontal cortex.2

The recent advances in neuroradiology techniques help understand the pathophysiological processes of MBD. Studies with diffusion-weighted imaging have shown a low apparent diffusion coefficient, which has been interpreted as irreversible cytotoxic oedema, and may precede the development of demyelination and necrosis and predict a poor or partial recovery.2 5 Nonetheless the high apparent diffusion coefficient showing reversible signal changes favoured an underlying vasogenic oedema-related process. In magnetic resonance spectroscopy studies, an increased choline/creatine ratio indicates demyelination during the acute phase of MBD, while a reduced N-acetylaspartate/creatine ratio suggests secondary axonal injury. In addition, decreased cerebral blood flow and cerebral blood volume in magnetic resonance perfusion suggest hypoperfusion. Recognition of the neuroradiological features is crucial to establish a diagnosis.

Concept or design: Both authors.
Acquisition of data: Q Wang.
Analysis or interpretation of data: F Ren.
Drafting of the manuscript: F Ren.
Critical revision of the manuscript for important intellectual content: Q Wang.

Both 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.

Both authors have disclosed no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

This study was approved by the Hospital of Chengdu University of Traditional Chinese Medicine Research Ethics Committee, China. Informed consent for all treatments and procedures, and consent for publication were obtained from the patient.

1. Tsai CY, Huang PK, Huang P. Simultaneous acute Marchiafava–Bignami disease and central pontine myelinolysis: a case report of a challenging diagnosis. Medicine (Baltimore) 2018;97:e9878. Crossref

2. Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava–Bignami disease: a review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry 2014;85:168-73. Crossref

3. Pérez Álvarez AI, Ramón Carbajo C, Morís de la Tassa G, Pascual Gómez J. Marchiafava–Bignami disease triggered by poorly controlled diabetes mellitus [in English, Spanish]. Neurologia 2016;31:498-500. Crossref

4. Bachar M, Fatakhov E, Banerjee C, Todnem N. Rapidly resolving nonalcoholic Marchiafava–Bignami disease in the setting of malnourishment after gastric bypass surgery. J Investig Med High Impact Case Rep 2018;6:2324709618784318. Crossref

5. Ménégon P, Sibon I, Pachai C, Orgogozo JM, Dousset V. Marchiafava–Bignami disease: diffusion-weighted MRI in corpus callosum and cortical lesions. Neurology 2005;65:475-7. Crossref

Orbital tumours encompass a wide range of benign and malignant space occupying lesions that may arise primarily from the orbit or have spread from other sites in the body. They are rare with an incidence of 1 in every 100 000 and may lead to devastating complications of which mechanical compression causing optic neuropathy is the most important.1 Multiple orbital tumours of the same orbit are even rarer with most reported cases being benign homologous tumours such as cavernous haemangiomas or myxofibrosarcomas.2 3 A 58-year-old ethnic Han Chinese male presented in December 2021 with a 1-month history of right eye proptosis. He had no clinical sign of optic neuropathy. Computed tomography of the orbit revealed right axial proptosis and two separate lesions in the right orbit. One lesion appeared infiltrative and measured 2.2 × 1.5 × 1.7 cm³ (anteroposterior × transverse × longitudinal) at the extraconal space with retrobulbar extension between the lamina papyracea and the medial rectus. The other was an encapsulated mass with regular border located in the superolateral intraconal region measuring 1.9 × 1.7 × 2.0 cm³ and abutting the optic nerve. Both lesions enhanced mildly with intravenous contrast (Fig 1). Blood results revealed normal thyroid function and immunoglobulin G4 and white blood cell levels. Based on the distinguishing radiological features of each lesion, we performed a two-stage surgery for theranostic reasons. An incisional biopsy of the medial infiltrative lesion was performed first through an anterior orbitotomy via an upper lid skin crease approach. Frozen section of the medial yellow jelly-like mass revealed atypical lymphoid cells with enlarged vesicular nuclei and amphophilic cytoplasm, highly suspicious of lymphoproliferative malignancy. We then performed a complete excision of the vascular encapsulated intraconal lesion using cryotherapy via a lateral orbitotomy. Formal histopathology reports revealed the first medial infiltrative lesion to be consistent with diffuse large B-cell lymphoma with positive immunostaining for CD20, BCL2, BCL6, MUM1, and CMYC1 (Fig 2). The second intraconal lesion was consistent with cavernous haemangioma (Fig 3).

At 4 months post-surgery there was no clinical sign of optic nerve damage and best-corrected visual acuity was 0.8 in the right eye. Positron emission tomography–computed tomography showed a residual hypermetabolic lesion over the medial aspect of the right orbit with no extra orbital lesion. The patient is receiving chemotherapy under the care of our haematology team.

Benign multiple homogenous lesions in the same orbit have been reported. Although multiple solitary fibrous tumours of the same orbit without malignant degeneration have been reported,4 multiple heterogeneous tumours in the same orbit are extremely rare. Ma et al5 reported a case of concurrent schwannoma and cavernous haemangioma in the same orbit of a 54-year-old female. To the best of our knowledge, concurrent benign and malignant lesions of the same orbit have not been reported in the English literature.

All authors contributed to the concept and design of this study, acquisition of data, analysis of data, drafting the manuscript, and critical 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.

The authors have no conflicts of interest to disclose.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki and provided informed consent for the treatment and consent for publication.

1. Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology 2002;109:243-8. Crossref

2. Deng C, Hu W. Multiple cavernous hemangiomas in the orbit: a case report and review of the literature. Medicine (Baltimore) 2020;99:e20670. Crossref

3. Du B, He X, Wang Y, He W. Multiple recurrent myxofibrosarcoma of the orbit: case report and review of the literature. BMC Ophthalmol 2020;20:264. Crossref

4. Griepentrog GJ, Harris GJ, Zambrano EV. Multiply recurrent solitary fibrous tumor of the orbit without malignant degeneration: a 45-year clinicopathologic case study. JAMA Ophthalmol 2013;131:265-7. Crossref

5. Ma M, Su F, Yang X. Multiple heterogeneous tumors in orbit: a case report. Int J Clin Exp Pathol 2019;12:4137-41.

A 65-year-old man with a 1-month history of left upper and lower limb chorea was admitted to the medical ward of our institution in February 2021. Blood tests revealed new-onset diabetes mellitus with markedly elevated fasting glucose level of 28.5 mmol/L. Urinalysis for ketones was negative. Urgent non-contrast computed tomography (CT) of the brain showed subtle hyperdensity at the right putamen without mass effect or surrounding oedema (Fig 1). The patient was started on subcutaneous insulin, and upon normalisation of blood glucose level, his hemichorea subsided without additional antichorea medications. Follow-up non-contrast magnetic resonance imaging of the brain performed 2 months later revealed T1-weighted hyperintensity in the right putamen, without restricted diffusion (Fig 2). Imaging findings were in keeping with non-ketotic hyperglycaemic hemichorea (NHH). The patient was prescribed a biphasic insulin regimen upon discharge.

An 87-year-old man with a 2-day history of left upper limb chorea was hospitalised in April 2022. He had known diabetes mellitus but was noncompliant with oral hypoglycaemic therapy. Blood tests revealed markedly elevated random glucose level of 30.8 mmol/L. Urgent non-contrast CT of the brain showed asymmetric subtle hyperdensity in bilateral putamen and caudate nuclei, more extensive on the right, but without mass effect or surrounding oedema (Fig 3). Findings were compatible with NHH. The patient resumed metformin, with gliclazide and subcutaneous insulin injection added for optimal control. Upon normalisation of blood glucose level, his hemichorea resolved without antichorea medications.

Unilateral or asymmetric basal ganglia hyperdensity in brain CT of patients with focal neurological symptoms can be alarming, and intracerebral haemorrhage may be suspected. Nonetheless NHH, a rare complication of uncontrolled diabetes, should not be overlooked.

Case reports of NHH have been documented as early as 1960.1 Previously reported cases were frequent in Asian elderly women with uncontrolled diabetes. In most patients, unilateral chorea was observed, although bilateral involvement could be present.2 3 4 Of note, the aetiology of hemichorea is diverse, and other causes include infarct, haemorrhage and neoplasm. Imaging of the brain is therefore crucial.

Non-contrast CT of the brain of NHH typically shows subtle hyperdensity in contralateral putamen and/or caudate nucleus, although bilateral involvement is also seen. There will be no mass effect or perilesional oedema, and this differentiates NHH from haemorrhage or tumour.2 3 4

Similarly, magnetic resonance imaging of the brain typically shows corresponding signal changes in striatal regions contralateral to the symptomatic side. There will be T1-weighted hyperintense signal. Differentials of increased basal ganglia T1-weighted signal are diverse. They include toxin-related causes such as methanol poisoning or hepatic-related causes such as acquired hepatocerebral degeneration, but they commonly show bilateral and symmetrical involvement.5 T2-weighted or fluid-attenuated inversion recovery signals can be variable. Of note, restricted diffusion is not expected in NHH,2 3 4 and this differentiates it from acute ischaemic stroke.

The pathophysiology of NHH is not fully understood. Proposed mechanisms include depleted gamma-aminobutyric acid and disrupted blood-brain barrier at the corpus striatum.2 3 4 Recognising this rare complication of uncontrolled diabetes mellitus enables prompt medical intervention. Neurological symptoms of NHH usually show substantial improvement after normalisation of blood glucose level without additional intervention.2 3 4

All authors contributed to the concept and design of the study, acquisition of data, analysis and interpretation of 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.

All authors have disclosed no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patients were treated in accordance with the Declaration of Helsinki. They provided informed consent for all treatments and procedures, and consent for publication.

1. Bedwell SF. Some observations on hemiballismus. Neurology 1960;10:619-22. Crossref

2. Zheng W, Chen L, Chen JH, et al. Hemichorea associated with non-ketotic hyperglycemia: a case report and literature review. Front Neurol 2020;11:96. Crossref

3. Narayanan S. Hyperglycemia-induced hemiballismus hemichorea: a case report and brief review of the literature. J Emerg Med 2012;43:442-4. Crossref

4. Cherian A, Thomas B, Baheti NN, Chemmanam T, Kesavadas C. Concepts and controversies in nonketotic hyperglycemia-induced hemichorea: further evidence from susceptibility-weighted MR imaging. J Magn Reson Imaging 2009;29:699-703. Crossref

5. Hegde AN, Mohan S, Lath N, Lim CC. Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus. Radiographics 2011;31:5-30. Crossref

An 84-year-old man was admitted with his third episode over 4 months of extended-spectrum beta-lactamase (ESBL)–producing Escherichia coli bloodstream infection (BSI). He had hypertension, diabetes mellitus, benign prostatic hyperplasia, Parkinson’s disease, ischaemic heart disease, and severe aortic stenosis treated with transcutaneous aortic valvular implantation. His most recent glycohaemoglobin level was 6.4% and he was on a controlled diet. He was not on long-term steroids. His two initial infections (presenting only with fever) had each been managed with a 2-week course of intravenous carbapenem and consequent normalisation of inflammatory markers, white blood cell (WBC) count and bacterial clearance on blood culture. Repeated urine culture and liver biochemistry were unremarkable. Transthoracic echocardiogram showed no evidence of vegetation.

The patient was readmitted with a 1-day history of fever with blood culture showing ESBL-producing E coli (refer to the Table for antibiogram) characterised by leukocytosis (WBC count=20×10⁹/L). Systemic review revealed no localising signs or symptoms. We administered 14 days of intravenous meropenem with rapid defervescence and normalisation of WBC. As a deep-seated infection was suspected, whole-body gallium scan was performed and showed intense uptake over the prostate (Fig a). We decided to treat his chronic prostatitis with a prolonged regimen of oral fosfomycin (3 g daily for 1 week, then 3 g every 48 hours for 6 weeks). The patient tolerated fosfomycin without adverse effects (eg, diarrhoea) and remained free of reinfection 3 months after discharge; interval gallium scan showed almost complete resolution of uptake (Fig b).

The primary site of ESBL-producing E coli BSI is predominantly the urinary tract, but may include intra-abdominal infections (eg, pyogenic liver abscess and psoas abscess).1 Typical prostatitis is diagnosed by a 10-time higher bacterial load in expressed prostatic fluid or urine sample collected after prostatic massage than that in a urine sample without prostatic massage.1 Our patient was asymptomatic with a negative urine culture; diagnosis was incidental on gallium scan, confirming its elusiveness. Once diagnosed, chronic prostatitis requires prolonged treatment for 4 to 6 weeks with an appropriate antibiotic.1

Extended-spectrum beta-lactamase–producing E coli arising from prostatitis has significant treatment implications. Few oral antibiotics can adequately penetrate the prostate to be clinically effective. They include fluoroquinolones, trimethoprim/sulfamethoxazole, and fosfomycin.2 3 Fosfomycin has a high clinical success rate and avoids the cardiac and musculoskeletal toxicities traditionally associated with fluoroquinolones.2 In our centre, it has a striking sensitivity of 97%.4

This report highlights three key points. First, chronic bacterial prostatitis should be considered in occult recurrent ESBL-producing Enterobacteriaceae BSI. Second, oral fosfomycin is an excellent choice for ESBL-producing E coli. Third, early stepdown from intravenous to oral antibiotics is effective in real life and validates historical retrospective studies.2 3 Early outpatient management is a pragmatic approach that is especially important within the current context of the coronavirus disease 2019 pandemic where healthcare facilities have been often overwhelmed. Gallium scan or positron emission tomography should be considered for patients with occult infection to determine its origin.5

All authors contributed to the concept or design, acquisition of data, analysis or interpretation of 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.

All authors declared no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki and provided informed consent for the treatment/ procedures, and consent for publication.

1. Zhanel GG, Zhanel MA, Karlowsky JA. Oral fosfomycin for the treatment of acute and chronic bacterial prostatitis caused by multidrug-resistant Escherichia coli. Can J Infect Dis Med Microbiol 2018;2018:1404813. Crossref

2. Tamma PD, Conley AT, Cosgrove SE, et al. Association of 30-day mortality with oral step-down vs continued intravenous therapy in patients hospitalized with Enterobacteriaceae bacteremia. JAMA Intern Med 2019;179:316-23. Crossref

3. Kwan AC, Beahm NP. Fosfomycin for bacterial prostatitis: a review. Int J Antimicrob Agents 2020;56:106106. Crossref

4. Ho PL, Chan J, Lo WU, et al. Prevalence and molecular epidemiology of plasmid-mediated fosfomycin resistance genes among blood and urinary Escherichia coli isolates. J Med Microbiol 2013;62:1707-13. Crossref

5. Lin KH, Chen YS, Hu G, Tsay DG, Peng NJ. Chronic bacterial prostatitis detected by FDG PET/CT in a patient presented with fever of unknown origin. Clin Nucl Med 2010;35:894-5. Crossref