Progressive facial disfigurement and deafness in craniometaphyseal dysplasia.
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Biomedical subjects
Publications and source records attributed to M A Anjay.
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Citrobacter, a Gram-negative enteric bacillus, is a rare cause of septicemia and meningitis, seldom reported beyond the neonatal period. It is characterized by a fulminant clinical course and a high incidence of complications, including brain abscesses. We studied a three-month-old infant with Citrobacter meningitis, who developed acute communicating hydrocephalus and multiple periventricular brain abscesses while on treatment. The patient died, despite intensive antibiotic treatment directed towards the causative organism, C. diversus.
Jugular phlebectasia is a rare cause of cervical swelling in children. It is a fusiform dilatation of any part of the jugular venous system and can involve the external, internal or anterior jugular veins. Previous reports suggest that the entity is often ignored or misdiagnosed. Unilateral internal jugular phlebactasia presenting as an intermittent neck swelling in a ten-year-old girl is reported. The clinical features are analyzed and the appropriate use of noninvasive imaging modalities is highlighted. The literature is also briefly reviewed.
Beckwith-Wiedemann syndrome is the most common overgrowth malformation syndrome. The classical features include macrosomia, macroglossia, omphalocele and ear lobe anomalies. Among the associated adrenal anomalies, foetal cortical cytomegaly, outer cortical haemorrhage and unilateral benign cysts are well described. A term neonate was admitted with typical features of the syndrome. Radiological evaluation revealed a rare association of bilateral benign hamorrhagic adrenal cysts. Serial sonography confirmed hamorrhage into benign cysts and ruled out neoplasms. Only one similar case has been documented in the literature previously.
A girl presented with a dull ache in the neck and mild difficulty in neck movements. She had limited clinical signs and her initial work up failed to reveal the cause. With the help of imaging modalities and CT guided needle biopsy, she was diagnosed to have an eosinophilic granuloma of the fifth cervical vertebra. There were no neurological symptoms. She was successfully managed with immobilization of spine, local irradiation and systemic vinblastine. The literature is briefly reviewed for clinical features, diagnosis and management.