Bai Jerbai Wadia Hospital For Children in Mumbai gave a fresh lease of life to a 1.5-month-old baby with a rare condition called aplasia cutis congenita leading to a skull defect. The baby whose scalp skin and skull bones weren’t developed underwent scalp rotation and advancement flaps when he was 5 days old. The baby has been discharged from the hospital and is recovering well.
Parents Afreen and Sanaulla Khan, the residents of Tamil Nadu were overjoyed due to the birth of their son. Unfortunately, their baby boy was born with complete absence of skin and skull bones of his scalp in the central area. The child was born in a nursing home at Saki Naka. He was immediately shifted to the neonatal intensive care unit at BJ Wadia Hospital for Children. The skin and skull bones were absent and underlying areas of the brain and its coverings were visible. The child was assessed in detail and all emergency resuscitative measures were taken. He was assessed by the neurosurgery and the plastic surgery teams and the plan for further treatment was made.
Dr Nilesh Satbhai, Consultant Plastic and Reconstructive Surgeon, Bai Jerbai Wadia Hospital For Children said, “This baby boy was born with a very rare deformity ‘aplasia cutis congenita’ and 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. As a result, the protective layers of the skull covering the brain are not formed. Depending on the severity and associated morbidities, the reported case fatality rate of aplasia cutis congenita is 20-50%. 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. The problem was identified immediately after birth. There is a high chance of infection or meningitis developing in this situation. Also, the large blood vessels in the brain can get infected, or ruptured, leading to bleeding and complications. We operated on the child on day 5 of his life.”
Dr Satbhai added, “We elevated 2 large flaps from the remaining scalp of the child’s head. Scalp rotation and advancement flap surgery was done wherein these flaps were transposed and rotated 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. Surgery in infants is always challenging, as the tissues are thin and delicate. The surgery lasted for 4 hours and it was uneventful. The child was strictly monitored and given meticulous post-operative care in the ICU, to ensure a smooth recovery and was discharged after 14 days. The wounds have now healed well on follow-up."
“We take pride in delivering successful treatment outcomes in paediatric patients even when it comes to dealing with rare conditions. Wadia Hospital has been a beacon of hope for many patients in need of excellent treatment. We have expertise in treating various illnesses and provide uninterrupted services to patients. We use advanced medical technologies to provide precise diagnoses and effective treatments as quickly as possible,” said Dr Minnie Bodhanwala, CEO of Wadia Hospitals.
“The happiness after the delivery of the baby turned into a nightmare for us. We were in a rude shock to see the exposed brain of the baby. But, the doctors at Wadia Hospital correctly detected the rare condition and treated the baby without any delay. We are extremely happy and thank the entire team of doctors who treated our son,” concluded the child’s mother Afreen Khan.
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