Doctors at Bai Jerbai Wadia Hospital for Children successfully performed a complex reconstructive surgery on a newborn diagnosed with Cantrell's pentalogy, a rare congenital condition where vital organs develop outside the body

Doctors at Bai Jerbai Wadia Hospital for Children successfully performed a complex reconstructive surgery on a newborn diagnosed with Cantrell's pentalogy, a rare congenital condition where vital organs develop outside the body

Doctors at Bai Jerbai Wadia Hospital for Children successfully performed a complex reconstructive surgery on a newborn diagnosed with Cantrell's pentalogy, a rare congenital condition where vital organs develop outside the body

The baby girl, diagnosed with Cantrell’s pentalogy before birth, underwent complex reconstructive surgery at Bai Jerbai Wadia Hospital for Children and was discharged 20 days after the operation.

Doctors successfully treated the newborn girl with Cantrell’s pentalogy, a rare congenital condition in which defects affect the development of the chest wall, abdominal wall, diaphragm, pericardium and heart.

Estimated to occur in one in 65,000 to 200,000 live births, the condition can be life-threatening, particularly when the heart is partially or completely displaced outside the chest. In this case, a substantial portion of the baby’s heart, along with parts of the liver and bowel, had developed outside the body because of a large defect involving the lower chest and upper abdomen.

The baby was diagnosed during pregnancy, giving doctors time to plan her delivery and treatment. A multidisciplinary team comprising fetal medicine specialists, obstetricians, neonatologists, paediatric surgeons, cardiothoracic surgeons, radiologists, anesthesiologists and intensive care specialists worked together to prepare for the complex procedure.

She was delivered by planned Caesarean section on June 1 and placed on ventilatory support immediately after birth to stabilise her condition and facilitate further evaluation.

Advanced imaging revealed defects involving the sternum, diaphragm and pericardium, in addition to the large chest and abdominal wall opening. The baby also had a ventricular septal defect (VSD), a hole in the wall separating the heart’s lower chambers, and a large patent ductus arteriosus (PDA), a blood vessel that normally closes shortly after birth but remained open.

The combination of abnormalities indicated the complete form of Cantrell’s pentalogy, which presents significant challenges for neonatal and cardiac surgical teams. After reviewing the imaging findings, the team developed and rehearsed a surgical plan before proceeding with reconstruction.

The operation was performed by pediatric surgeons Dr Pradnya Bendre, Dr Gayatri Munghate and Dr Abhishek Mathur, along with Dr Biswa Panda, cardiovascular and thoracic surgeon.

During the procedure, the surgeons carefully separated the exposed heart, liver and bowel loops from the surrounding sac. They used the baby’s own pericardial tissue to reconstruct the protective covering around the heart, supplementing it with synthetic mesh where necessary. The organs were then repositioned within the chest and abdominal cavities, and the diaphragm and abdominal wall were reconstructed.

The procedure also required careful management of the physiological changes that can occur when organs lying outside the body are returned to their normal anatomical positions. Closing a large defect and repositioning the organs can increase pressure within the chest and abdomen, potentially affecting breathing, blood circulation and cardiac function.

The anaesthesia team monitored and managed these challenges throughout the operation. According to the hospital, the baby remained stable and tolerated the reconstruction well.

Following surgery, the newborn was monitored in the neonatal intensive care unit and gradually weaned off ventilatory and circulatory support. She progressed to full feeds and recovered steadily.

She was discharged after 20 days post-operation, feeding well, with normal bowel and bladder function and a healing surgical wound.

Cantrell’s pentalogy was first described by James Cantrell and colleagues in 1958. The syndrome involves a spectrum of developmental defects affecting the abdominal wall, lower sternum, diaphragm, pericardium and heart. The severity varies between patients, and outcomes depend on the extent of the defects and the presence of associated cardiac abnormalities.

In its most severe form, the condition can be associated with ectopia cordis, in which the heart lies partially or entirely outside the chest. The combination of structural abnormalities can make treatment particularly challenging in newborns.

The hospital said the baby’s recovery highlights the role of prenatal diagnosis, detailed imaging, coordinated surgical planning and specialised neonatal care in managing complex congenital conditions.

Speaking about the outcome, Dr Minnie Bodhanwala, chief executive officer of Bai Jerbai Wadia Hospital for Children, said the recovery reflected the teamwork and planning of the multidisciplinary team. She added that the hospital remained committed to providing advanced pediatric care with compassion and equity, and thanked the family for placing its trust in the team.

Early identification of the abnormalities enabled the team to prepare for the baby's immediate needs after delivery and plan a complex reconstruction.

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