Portal Hypertension in a Case of Klippel Trenaunay Syndrome
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Our case was very similar to that described by Jones, et al. [2] but that girl had persistent tachycardia and most of the clinical features of KD with normal echocardiography. Riphagen, et al. [3] reported a case series of 8 children (only 3 tested COVID-19 positive) needing intensive care support with a hyper-inflammatory shock. One child died after a massive cerebral infarction. All of them had features mentioned in RCPCH guidelines with minimal respiratory symptoms [3]. These children and the two infants with Kawasaki disease most likely had a similar pathogenesis with varied consequences, which needs further research to define it.
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India is still in the early stage of this pandemic and has not yet had many children with severe COVID-19. This 4-monthold child presenting as typical Kawasaki disease represents a novel presentation among the very young population with COVID-19.
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Published online: May 22, 2020; PII: S097475592000659
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BHASWATI C ACHARYYA1*, SAUMYABRATA ACHARYYA2 AND DHRITABRATA DAS3 1 Departments of Pediatric Gastroenterology, 2Pediatrics, and 3Pediatric Cardiology, AMRI Hospitals, Kolkata, West Bengal, India. *[email protected] REFERENCES
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1. Dong Y, Mo X, Hu Y, Qi X, Jiang F, Jiang Z, et al. Epidemiology of covid-19 among children in China.
Portal Hypertension in a Case of Klippel Trenaunay Syndrome
We report an 11-year-old girl who presented with swelling of the right gluteal region noticed since birth. This swelling slowly progressed to involve the whole of the right lower limb accompanied by dilated veins over lateral aspect of the ankle. At six years of age, she developed clusters of small vesicles with warty appearance in the affected lower limb which ruptured spontaneously discharging serous fluid. Baseline hemogram, kidney and liver function tests were normal. Skin wedge biopsy performed was consistent with lymphangioma circumscriptum. Ultrasound doppler of gluteal region revealed dilated anechoic tortuous channels showing no flow within suggestive of lymphangioma. Ultrasonography of abdomen was normal. MR angiography of limb revealed extensive soft tissue hypertrophy involving right gluteal, thigh and upper leg
Klippel Trenaunay syndrome (KTS) is a sporadic disorder that belongs to PIK3CA-related overgrowth spectrum of disorders. The diagnostic criteria for KTS comprise of presence of capillary malformation, venous with or without lymphatic malformation and limb overgrowth. Only 63% patients have all three clinical manifestations [1]. Here we describe a case of KTS presenting as mixed venolymphatic malformation with complication in the form of portal hypertension due to dysplastic portomesentric veins.
INDIAN PEDIATRICS
Pediatrics. 2020; 145. Available from: www. aappublications.org/news. Accessed May 10, 2020. Jones VG, Mills M, Suarez D, Hogan CA, Yeh D, Segal JB, et al. COVID-19 and Kawasaki disease: Novel virus and novel case. Hosp Pediatr. 2020. Available from: https:// hosppeds.aappublications.org/content/hosppeds/early/ 2020/04/06/ hpeds.2020-0123
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