We have assessed the oncological and functional results of limb salvage surgery using a custom-made endoprosthetic replacement in six patients (mean age 17 years) with distal tibial osteosarcoma (stage IIB). A wide margin excision was possible in three, marginal in two and contaminated in one. Skeletal reconstruction was performed using a locally designed and manufactured custom-made distal tibial and ankle replacement prosthesis. Two patients developed local recurrence and one necrosis of the flap and deep infection. In three in whom the prosthesis remained in place the mean functional score according to the rating system of the Musculoskeletal Tumour Society was 24.3/30. In carefully selected patients limb salvage with prosthetic replacement is possible for distal tibial osteosarcomas.
Primary amoebic meningoencephalitis (PAM) caused by free-living amebae Naegleria fowleri is a rare and fatal condition. A fatal case of primary amoebic meningoencephalitis was diagnosed in a 5-month-old infant who presented with the history of decrease breast feeding, fever, vomiting, and abnormal body movements. Trophozoites of Naegleria fowleri were detected in the direct microscopic examination of CSF and infant was put on amphotericin B and ceftazidime. Patient condition deteriorated, and he was discharged against medical advice and subsequently expired. We also reviewed previously reported 8 Indian cases of primary amoebic meningoencephalitis (PAM) and observed that for the last 5 years, none of the patients responded to amphotericin B. Has an era of amphotericin B-resistant Naegleria fowleri been emerged? Management strategy of PAM needs to be reviewed further.
The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies.
IntroductionKawasaki disease (KD) is a form of acute vasculitis involving small and medium sized arteries. Ischemic necrosis of the extremities is a very rare and potentially severe complication of KD. Here we present a case of KD with peripheral gangrene and auto amputation, which is the first such case to be reported from India and the fourteenth case in medical literature [1]. Case ReportA one year old boy was admitted with history of fever for 20 days associated with maculopapular rash, irritability, congestion of the oral cavity and conjunctiva, and peripheral gangrene for the previous one week. The fever was moderate grade, intermittent, associated with irritability. Five days later, the child developed a maculopapular rash which started over the extremities and later became generalised. It was associated with redness of the oral cavity, lips and the eyes which resolved over a period of one week. Gangrene of the toes and the fingers was noticed 10 days after the onset of fever. On examination the child had a height of 75 cms and weight of 8 kgs. He was irritable, febrile (100.6F) with a heart rate of 130/min and BP of 106/68mm hg. All his pulses were well felt with a capillary filling time of less than 3 seconds. Left sided cervical lymphadenopathy was present along with pallor and bilateral pedal and dorsal oedema. There was desquamation of the skin over the upper and lower extremities with gangrene on the right index finger and bilateral great toes. Blackish discoloration was noticed over other toes and fingers (Fig. 1). Systemic examination did not reveal any sign. Child was discharged following 7 days of antibiotics. At follow up the child was afebrile, not irritable and showed autoamputation of the tip of the right index finger (Fig. 2). The other sites of peripheral gangrene had resolved. Investigations revealed a decreased ESR, CRP, Total WBC and platelet count. Treatment was changed over to low dose aspirin for a period of 6 weeks and was gradually stopped. AbstractKawasaki disease (KD) is a form of acute vasculitis involving small and medium sized arteries. Ischemic necrosis of the extremities is a very rare and potentially severe complication of KD. Here we present a case of KD with peripheral gangrene and auto amputation, which is the first such case to be reported from India and the fourteenth case in medical literature. A one year old child with KD and peripheral gangrene was started on aspirin. The digital gangrene resolved on follow up with amputation of the tip of the right index finger. StreszczenieChoroba Kawasaki (KD) to forma ostrego zapalenia naczyń z udziałem małych i średnich tętnic. Martwica kończyn jest bardzo rzadkim i potencjalnie ciężkim powikłaniem KD. Poniżej prezentujemy przypadek KD z obwodową gangreną i autoamputacją, który jest pierwszym takim przypadkiem zgłoszonym w Indii i 14-tym przypadkiem w literaturze medycznej. U rocznego dziecka z KD i zgorzelą obwodową rozpoczęto leczenie aspiryną. Obserwowano zgorzel palców z amputacją końcówki prawego palca wskazującego.
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