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1.
Iranian Journal of Pediatrics. 2010; 20 (1): 118-122
in English | IMEMR | ID: emr-99081

ABSTRACT

Only few reported cases of tectocerebellar dysraphia with occipital encephalocele have been reported in the literature. Three month baby boy, the first child of healthy, consanguineous parents presented with a small swelling over the occipital region since birth. The child also used to have apneic spells without cyanosis and spontaneous recovery. CT scan showed absence of the cerebellar vermis, absence of tectum and the 4[th] ventricle communicating with the occipital menigocoele sac and an occipital bone defect. The excision of the encephalocoele sac was performed, however the child continued to have apneic spells and did not do well. In our child irregular respiration probably was the manifestation of the tectocerebellar dysraphia syndrome complex and associated shunt malfunction followed by seizures decompensated the physiology of the child leading to fatal outcome


Subject(s)
Humans , Male , Infant , Meningocele , Cerebellum/abnormalities , Nevus , Funnel Chest , Dandy-Walker Syndrome
2.
Iranian Journal of Pediatrics. 2009; 19 (4): 439-441
in English | IMEMR | ID: emr-99997

ABSTRACT

Dermoid cyst with dermal sinus in occipital region is extremely rare occurrencl and still rarer in asterion region. We report a case of dermoid cyst with dermal sinus at the asterion probably the second case of world literature and discuss the findings. 16 year-old female patient with mental retardation and inability to speak since childhood presented with history of progressive increasing swelling since childhood over the left side of occipital region. Local examination and investigations were suggestive of extracranial cystic lesion. The lesion was excised completely. When excision of such lesions is contemplated, careful attention must be paid during exploration. Failure to recognize intracranial communication in such lesions may result in fatal consequences. Pre-operative suspicion of such a connection helped us to identify the underlying defect intra-operatively and facilitated safe excision


Subject(s)
Humans , Female , Intellectual Disability , Cutaneous Fistula , Mutism
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