A 6 year old girl woke up with pain and increasing swelling over the left hand and difficulty in breathing. On examination, she had swelling of the left forearm and hand, flaccid quadriparesis and was in respiratory failure requiring mechanical ventilation. Two clean puncture wounds were identified on the left thumb. A provisional diagnosis of snake bite with severe envenomation was made and she was given anti snake venom therapy. Over a period of about 4 hours her weakness progressed. She became areflexic, developed internal and external ophthalmoplegia and loss of other brain stem reflexes mimicking brain death. Mechanical ventilation was continued despite features suggestive of brain stem dysfunction. About 36 hours after ventilation she showed a flicker of movement of her fingers and gradually the power improved. She was weaned off the ventilator and extubated after 5 days. External ophthalmoplegia is an established association with cobra envenomation, but, this combination of internal and external ophthalmoplegia can mimic brain death and pose a dilemma to the caregivers regarding continuation of therapy. Case reportA 6 year old girl who was sleeping on the floor woke up at 3 AM with pain and swelling over the left hand. This was initially ignored by the parents. The pain and swelling gradually increased, and she complained of difficulty in breathing about 2 hours later. At this point she was brought to the hospital. There was no history suggestive of a snake bite. Examination revealed a drowsy child with a massively swollen left hand and forearm with shallow breathing. There were two clean puncture marks on the left thumb but no bleeding from them. There was no blistering or necrosis of the swollen limb. The local lymph nodes were enlarged. Examination of the central nervous system showed the child to be drowsy but arousable, not obeying verbal commands and was localizing pain. She had ptosis and grade 2-3/5 power in all four limbs and sluggish deep tendon reflexes with extensor plantars. Her pulse rate was 80/min, BP-100/60 mmHg SpO 2 -60% on 5 liters of oxygen by face mask and temperature 98.4°F. Endotracheal intubation was done and she was ventilated on synchronized intermittent mandatory ventilation (SIMV) mode as she had some spontaneous respiratory efforts. Polyvalent anti snake venom therapy was started and a total of 100 ml was given (Haffkine Institute, Bombay), after a provisional diagnosis of snake bite with severe envenomation was made. Over a period of about 4 hours, the weakness progressed and involved proximal muscles first and then distal muscles. She was comatose, had no motor response to painful stimuli, became areflexic and her plantar reflex was not elicitable. Her pupils

Early identification of risk factors and appropriate intervention may reduce the incidence and improve the outcome in NEC.

TH reduces oxidative stress-induced DNA damage and improves neurodevelopmental outcome.

Split-hand/split-foot malformation (SHFM) is mainly inherited as an autosomal dominant trait with incomplete penetrance and characterized by malformation of the limb involving the central rays of the autopod. It presents with a deep median cleft of the hand and/or foot, aplasia/hypoplasia of the phalanges, metacarpals, and metatarsals. Pathogenic mechanism is a failure to maintain signaling from the median apical ectodermal ridge. Without this signaling, cells of the underlying progress zone stop proliferation and differentiation which in turn results in defects of the central rays. We describe a case of SHFM in 10-year-old boy.

Frank-Ter Haar syndrome is an unusual type of skeletal dysplasia with megalocornea and developmental delay. It is usually transmitted as autosomal recessive disorder. Only a few cases have been reported in the literature and none from India. The authors report a case with other unusual features and a short review of the condition.