Sturge–Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel–Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge–Weber syndrome in combination with Klippel–Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes.
Medium chain triglyceride (MCT)-based diet, total parenteral nutrition (TPN) and repeated paracentesis are considered as supportive management for congenital chylous ascites (CCA). TPN is considered where therapy with oral MCT is poorly tolerated by the patient especially young infant with unstable hemodynamic. Surgery is recommended when medical therapy fails. Herein, we report a 2½-month-old infant with CCA, treated successfully with octreotide intravenous infusion after the initial failure to response to conventional conservative therapy with MCT-enriched formula and paracentesis.
Hemophilic pseudotumors are rare but dangerous complications of Hemophilia. We hereby report a 3-year-old boy with Hemophilia B, presenting with nasal pseudotumor, showing favorable response to radiotherapy after unsuccessful treatment with factor IX replacement therapy. The diagnosis and treatment of this rare condition is also reviewed.
Small pericardial effusion (PE) is not an infrequent manifestation in primary hypothyroidism. But massive PE with or without cardiac tamponade is rare and often associated with severe form of the disease. Here we report an eight-year-old boy who was admitted with massive PE that required repeated pericardiocentesis. Detailed examinations failed to identify the etiology initially. Five months later, child was readmitted with massive PE with impending cardiac tamponade. Primary hypothyroidism was diagnosed based on the clinical and laboratory finding and was thought to be the underlying etiology of previously encountered undiagnosed massive PE. Beside pericardiocentesis, child was treated with thyroid hormone replacement. Condition gradually improved without further recurrence of PE till date. Therefore, irrespective of the presence of clinical signs, primary hypothyroidism should be suspected in every patient presenting with massive PE to prevent recurrence as well as its serious complications like cardiac tamponade.
Myiasis is an infection of live mammalian tissue by the larvae of dipteran flies and commonly found in the tropics and subtropics. It usually infects domestic and wild animals, but sometimes also affects human. Umbilical cord myiasis in the neonatal period associated with sepsis is a rare occurrence with few reported cases in the literature. In this article, authors report a 7-day-old neonate from a rural area presented with neonatal sepsis due to umbilical myiasis caused by fly larval form of blow fly (Chrysomya megacephala).
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