Naeem Raza, Asif Naseer.
Harlequin baby.
Pak Armed Forces Med J Jan ;55(1):86-7.

A multi-parous female, reported with Labour pains at 28th week of gestation. Ante-natal Sonography revealed breech lie of fetus and cleft palate. At Labour room she delivered a female baby with Apgar score 6/10, weight 1.8 Kg, exhibiting dysmorphic features of boggy scalp, eclabium, cleft palate, rudimentary nose and ears, closed eyes, mitten cased hands and feet deformities and cracked, deeply fissured skin covering the whole body. Baby was diagnosed as Harlequin and nursed in humidified incubator at intensive care unit. Temperature of 32-33oc was maintained, topical care by application of white soft paraffin and liquid paraffin was provided, feeding was done using nasogastric tube. Feeding requirement was increased with passing days. On fourth day she developed foul smell so blood sample and skin swab for culture & sensitivity were ordered. In the meantime she was given a warm saline bath to eradicate the foul smell and on the same day suspension Co-amoxiclave was commenced. However she remained afebrile and no growth of organisms after 48 hours of incubation was observed. On 7th day her intake reduced and the condition gradually deteriorated. Despite adequate measures she expired at the age of 10 days. The couple was consanguineous. Baby was the fourth of other normal siblings with no history of ichthyosis, skin disorders, intrauterine or neonatal deaths in the family. No interventional prenatal procedure (e.g., amniocentesis, and fetal skin biopsy) was performed.

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