5-day-old infant operated on to correct rare skull defect in Mumbai hospital

An infant boy, born with a rare skull defect, was successfully operated on - when five days old - to repair his skull and scalp at a Mumbai hospital, doctors said on Thursday.

The child was born Afreen Khan and Sanaulla Khan, hailing from Tamil Nadu, in a private nursing home in Andheri east last December, but their joy was short-lived as the infant was found to suffer from 'aplasia cutis congenita', where the baby's scalp skin and skull bones were not fully developed in the central area of the head, leaving the underlying areas of the brain and its covering visible.

To correct this, he had to undergo a challenging scalp rotation and advancement flaps when he was barely 100-hours old.

The infant was brought to the Bai Jerbai Wadia Hospital in early January and admitted to the neonatal ICU, under the care of Dr. Nilesh Satbhai, the hospital's Consultant Plastic & Reconstructive Surgeon.

The team assessed him in detail, took all emergency resuscitative measures and the plans were made by the neurosurgery and plastic surgery teams for the further treatment.

"This baby boy was born with a very rare deformity 'aplasia cutis congenita' and a large portion of the vital areas of the brain was left exposed. This is a very rare anomaly in which part of the scalp skin along with the skull bones is not developed, and the protective layers of the skull covering the brain are not formed," Dr Satbhai said.

He said that depending on the severity and associated morbidities, the reported case fatality rate of the condition is 20-50 per cent.

"In this child, the skin, soft tissue, and bones over the vertex of the skull were absent, the dura mater covering the brain was exposed and the problem was identified immediately after birth. There is a high chance of infection or meningitis developing in such a situation. Also, the large blood vessels in the brain can get infected, or ruptured, leading to bleeding and complications. We operated on the child for four hours on the 5th day of his life," said Dr. Satbhai.

For this, the medical team elevated 2 large flaps from the remaining scalp of the child's head, carried out scalp rotation and advancement flap surgery to transpose and rotate the flaps across the midline to cover the entire skin defect and protect the dura mater and underlying brain.

The flaps survived and healed well, resulting in complete coverage of the scalp skin defect, the child was strictly monitored, given meticulous post-operative ICU care and was finally discharged after 14 days, with the wounds healing well.

"Surgery in infants is always challenging, as the tissues are thin and delicate. We take pride in delivering successful treatment outcomes in paediatric patients with rare conditions, using advanced medical technologies to provide precise diagnoses and effective treatment," Wadia Hospitals CEO Dr. Minnie Bodhanwala said.

For the overjoyed parents, they recalled how their happiness after child birth turned to horror on seeing the exposed brain of their infant.

"However, the medical team at Wadia Hospital correctly detected the rare condition and treated our baby without delay. We are extremely happy and thank them for successfully treating our son," said Afreen Khan, caring for her son who has emerged from a nightmarish situation.