Indian Pediatrics 2002; 39:1166

Seckel Syndrome

A 16 months old female baby born of consanguineous marriage presented with delayed developmental milestones since birth and severe growth failure. Her anthropometric measurements were: Weight: 2.4 kg, Length: 50 cms and Head circumference : 31 cms. She was born normally at term with low birth weight without any postnatal complications. Her postnatal growth was very poor, inspite of proper feeding.

Fig.1. Appearance of a typical "bird head" in Seckel Syndrome

One of her elder sisters, aged about twelve years also has a similar problem. She was hyperactive, playful and had a pleasant appearance. The CT scan of brain revealed agenesis of corpus callosum. The proportionate short stature, microcephaly, small hands, receding forehead, prominent beak like protruded nose, receding chin, giving the appearance of a typical "bird head" (Fig.1) are the features which suggested the diagnosis of Seckel Syndrome. Seckel Syndrome has an autosomal recessive inheritance and also called "bird headed dwarfism".

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