Paget-Schroetter syndrome

ABSTRACT

Hong Kong Med J 2007;13:243-5 | Number 3, June 2007
CASE REPORT
Paget-Schroetter syndrome
Gursel L Oktar, Emin G Ergul
Division of Cardiovascular Surgery, Ankara Oncology Training and Research Hospital, Ankara, Turkey
 
 
Paget-Schroetter syndrome, or 'effort' thrombosis of the axillary-subclavian vein, is an uncommon deep vein thrombosis usually caused by excessive upper limb activity. It may cause post-thrombotic syndrome, leading to significant disability if not treated appropriately. The optimal management for this syndrome is still controversial because the outcomes of different treatment strategies are based on case studies and series in small numbers. We report a case of Paget-Schroetter syndrome in a young male weight-lifter and discuss treatment strategies suggested by the current literature.
 
Key words: Axillary vein; Subclavian vein; Treatment outcome; Venous thrombosis; Weight lifting/injuries
 
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Unusual eye signs in Wegener's granulomatosis

ABSTRACT

Hong Kong Med J 2007;13:241-2 | Number 3, June 2007
CASE REPORT
Unusual eye signs in Wegener's granulomatosis
Alice YK Chan, Edmund K Li, Paul CL Choi, David TL Liu, Philip TH Lam, Dennis SC Lam
Social Hygiene Service, Department of Health, Hong Kong
 
 
Wegener's granulomatosis is a rare necrotising vasculitis not easily diagnosed due to the obscurity of its diverse clinical features. Despite its comparatively low incidence, the unusual ophthalmic manifestations seen in this disease warrant extra caution from attending rheumatologists. In this case, bilateral peripheral ulcerative keratitis preceded any systemic symptoms. Timely recognition of the significance of this ophthalmic complaint and prompt ophthalmological consultation can help achieve early diagnosis and treatment of this potentially fatal rheumatological disease.
 
Key words: Vasculitis; Wegener's granulomatosis
 
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Orbital involvement in Erdheim-Chester disease

ABSTRACT

Hong Kong Med J 2007;13:238-40 | Number 3, June 2007
CASE REPORT
Orbital involvement in Erdheim-Chester disease
Winnie WY Lau, Edwin Chan, Clement WN Chan
Department of Ophthalmology, Queen Mary Hospital, Pokfulam Road, Hong Kong
 
 
Erdheim-Chester disease is a rare, idiopathic, non-Langerhans' cell, histiocytic disorder. To our knowledge this is only the second case of Erdheim-Chester disease reported in the Chinese population. We describe a 45-year-old woman presenting with unilateral proptosis and periorbital xanthelasma. Histopathological examination revealed a xanthogranulomatous lesion expressing CD68, but negative for S100 protein, CD1a, CD3, or CD20. Systemic involvement was evident on bone scanning, and involvement of the thorax and abdominal aorta was seen on computed tomography. Despite treatment with systemic steroids, immunosuppressants, chemotherapy and interferon, progressive deterioration occurred. Our patient's clinical course was consistent with reports in the literature. Unfortunately, our patient developed neutropenic fever and died from septicaemic shock. Although Erdheim-Chester disease is a rare entity, especially in the Chinese population, an unusual presentation with orbital masses and bilateral xanthelasma, associated with systemic features, should raise the suspicion of this serious and potentially fatal disease.
 
Key words: Eyelid diseases; Histiocytosis, non-Langerhans' cell; Orbital diseases; Xanthomatosis
 
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Non-typhoid Salmonella mycotic aneurysm of the aortic arch

ABSTRACT

Hong Kong Med J 2007;13:234-7 | Number 3, June 2007
CASE REPORT
Non-typhoid Salmonella mycotic aneurysm of the aortic arch
Stella PY Wong, Thomas KK Lai, WL Ng, WK Luk
Department of Medicine, Tseung Kwan O Hospital, Tseung Kwan O, Hong Kong
 
 
Salmonella mycotic aneurysm is a rare but potentially fatal condition. Mortality is high without timely intervention. The clinical presentation is protean and early diagnosis requires a high degree of clinical alertness. Prompt surgical intervention and prolonged antimicrobial therapy are keys to successful treatment. We report an 81-year-old man with an atypical presentation of Salmonella mycotic aneurysm in the aortic arch. The case highlights the need to evaluate all patients over 50 years with non-typhoid Salmonella bacteraemia for possible endovascular infections. Contrast-enhanced computed tomography is useful for making an early diagnosis of this disease.
 
Key words: Aortitis; Aortic arch syndromes; Endocarditis, bacterial; Salmonella infections
 
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Two cases of postmaturity-related perinatal mortality in non-local expectant mothers

ABSTRACT

Hong Kong Med J 2007;13:231-3 | Number 3, June 2007
CASE REPORT
Two cases of postmaturity-related perinatal mortality in non-local expectant mothers
Cherrie Yung, Kennis Liu, WL Lau, Helena Lam, WC Leung, Robert Chin
Department of Obstetrics and Gynaecology, Kwong Wah Hospital, Kowloon, Hong Kong
 
 
We present two cases of postmaturity-related perinatal mortality with delivery at 42 weeks 6 days' and 44 weeks' gestation, respectively. No cause beyond postmaturity was found. Neither induction of labour nor foetal monitoring had been performed despite these gestations going post 41 weeks because of a current 'social obstetrics' phenomenon—non-local expectant mothers coming to Hong Kong from mainland China for delivery.
 
Key words: Infant mortality; Pregnancy, prolonged; Socioeconomic factors
 
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A recalcitrant case of cicatricial pemphigoid

ABSTRACT

Hong Kong Med J 2007;13:157-60 | Number 2, April 2007
CASE REPORT
A recalcitrant case of cicatricial pemphigoid
JTHT Yu, LY Chong, KC Lee
Yau Ma Tei Dermatology Clinic, Social Hygiene Service, 143 Battery Street, Hong Kong
 
 
A 57-year-old woman presented with a 1-year history of blisters and erosions on her oral mucosa with bilateral conjunctivitis and symblephara formation. A diagnosis of cicatricial pemphigoid was made based on the clinical features and immunohistological findings. A multidisciplinary team managed her with different topical and systemic immunosuppressive agents but she finally succumbed due to multi-organ failure secondary to sepsis.
 
Key words: Pemphigoid, benign mucous membrane
 
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Acyclovir-induced nephrotoxicity in a patient with acute retinal necrosis

ABSTRACT

Hong Kong Med J 2007;13:155-6 | Number 2, April 2007
CASE REPORT
Acyclovir-induced nephrotoxicity in a patient with acute retinal necrosis
DTL Liu, VYW Lee, PTH Lam, DSC Lam
Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
A 50-year-old woman developed rapidly progressive acute renal failure on the first day after the administration of intravenous acyclovir for acute left retinal necrosis. Intravenous acyclovir was stopped and replaced with intravitreal injections of ganciclovir sodium (2 mg/0.05 mL) and foscarnet (1.2 mg/0.05 mL) 3 times per week for 4 weeks. Acyclovir-induced renal impairment can be reversed if recognised early and treated with careful, timely body fluid replacement. The necrotising retinitis responded well to intravitreal antiviral agents. No complications were seen at the 6 months' follow-up. Constant vigilance is essential for avoiding acute renal failure when treating ophthalmic conditions with intravenous acyclovir. Systematic monitoring of renal function, urine output, and characteristic symptoms like loin pain is warranted.
 
Key words: Acyclovir; Drug toxicity; Retinal necrosis syndrome, acute
 
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A Chinese family with familial paraganglioma syndrome due to succinate dehydrogenase deficiency

ABSTRACT

Hong Kong Med J 2007;13:151-4 | Number 2, April 2007
CASE REPORT
A Chinese family with familial paraganglioma syndrome due to succinate dehydrogenase deficiency
RCW Ma, CW Lam, WB Chan, WY So, SF Tong, CC Chow, CS Cockram
Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
We report the genetic characteristics of a family with familial paraganglioma syndrome. The index patient was diagnosed with carcinoid tumour of the bronchus at the age of 30 years then later diagnosed with bilateral phaeochromocytoma. His sister had bilateral carotid body tumours. Mutational analyses of succinate dehydrogenase B and SDHD on the index patient showed him to be heterozygous for the M1I mutation of the SDHD gene. A genetic analysis revealed that his sister also had succinate dehydrogenase deficiency with the same mutation. Pre-symptomatic testing confirmed the genetic diagnosis, and led to a clinical diagnosis in an otherwise asymptomatic sibling. Comparison with other known cases of M1I mutation suggests that this is a founder mutation in the Chinese population. Genetic analysis of the succinate dehydrogenase genes can provide a specific diagnosis and allow for genetic screening of at-risk individuals.
 
Key words: Paraganglioma; Phaeochromocytoma; Succinate dehydrogenase
 
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Supradiaphragmatic and transdiaphragmatic intrathoracic migration of a ventriculoperitoneal shunt catheter

ABSTRACT

Hong Kong Med J 2007;13:147-9 | Number 2, April 2007
CASE REPORT
Supradiaphragmatic and transdiaphragmatic intrathoracic migration of a ventriculoperitoneal shunt catheter
MH Rahimi Rad, J Mirzaagazadeh, K Ansarin
Department of Respiratory Medicine, Urmia University of Medical Sciences, Urmia, Iran
 
 
A hydrothorax following ventriculoperitoneal shunt catheter insertion is very rare and usually reported in children. Only about 25 cases have been described in the literature and very few have been adults. We report a 51-year-old woman with a massive hydrothorax and respiratory distress following both supradiaphragmatic and transdiaphragmatic migration of a ventriculoperitoneal shunt catheter into the pleural space. To our knowledge this is the first report of the simultaneous occurrence of two types of such migration in one patient.
 
Key words: Arachnoid cysts; Complications; Diaphragm; Hydrothorax; Pleural effusion; Ventriculoperitoneal shunt
 
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Spontaneous basilar artery dissection

ABSTRACT

Hong Kong Med J 2007;13:144-6 | Number 2, April 2007
CASE REPORT
Spontaneous basilar artery dissection
Z Han, TW Leung, W Lam, Y Soo, KS Wong
Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
Dissection of the basilar artery is rare. We report a 51-year-old man who developed acute pontine infarction associated with dissection of the distal basilar artery. There was no trauma or unaccustomed movement of the head and neck prior to the stroke. The dissection was diagnosed non-invasively by magnetic resonance imaging and magnetic resonance angiography. Cervicocerebral artery dissection is a common cause of stroke in young patients particularly when conventional cardiovascular risk factors are absent. Magnetic resonance angiography combined with magnetic resonance imaging is a useful diagnostic tool prior to invasive angiography.
 
Key words: Basilar artery; Brain stem infarctions; Dissection; Magnetic resonance angiography; Magnetic resonance imaging
 
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