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Images in Clinical Practice

Indian Pediatrics 1999;36:328

Neu-laxova Syndrome


The Neu-Laxova Syndrome is a rare lethal syndrome, inherited as an autosomal recessive trait characterized by severe intrauterine growth retardation, perinatal death, microcephaly, apparent edema and thick scaly skin. A stillborn male baby was a product of a non consanguineous marriage, delivered at 32 weeks gestation vaginally to a multiparous woman, who had polyhydramnios. The baby weighed 1 kg. The baby had microcephaly, slanting forehead, absent eye lids, micrognathia, bilateral cleft lip and cleft palate. Baby also had multiple contractures, hypoplasia of all the digits, swollen feet and hands. The skin was thickened over the entire body with a waxy consistency (Fig. 1). Abnormal devel­opment of the head, along with severe growth retardation, thickness of the skin and polyhydramnios could be important elements for prenatal diagnosis in an at risk pregnancy.

Fig. 1. Stillborn baby with features of Neu-Laxova Syndrome.

Shreedhara A vabratha K.,
Sulochana P.,
 Somegowda*,
 
Departments of Pediatrics and
                                 *Obstetrics and Gynecology,

Vijayanagara Institute of Medical Sciences,
                                                        Bellary
583 101.
                                                                 Karnataka.
India.

 

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