Fetal Medicine achieves a new milestone in successful Pulmonary Sequestration Treatment

by Priya Jadhav

Mumbai, June 27, 2019 :Fetal Medicine in India achieved a new milestone with a successful surgery to save a preterm baby who suffered from life-threatening lung abnormality- Pulmonary Sequestration. A 36- year old woman with 27-weeks of pregnancy was admitted at the Fetal Medicine Department of Surya Hospitals after having diagnosed with serious fetal complications. A multidisciplinary team of experts swung into action and performed one of the most complicated procedures to save a newborn who is now responding well to the treatment. Experts are monitoring his health closely for the last six months.

Detailed fetal ultrasound evaluation revealed generalized swelling around the fetus with severe fluid accumulation around the left lung. The condition was severe enough to displace the heart to the right side of the chest. The fetal lung abnormality was diagnosed as extralobar pulmonary sequestration (PS) which is a mass of non-functional lung tissue separate from the normal lung inside the pleural cavity and having a distinctive blood supply. A large mass can often cause severe heart failure, hydrops (fluid accumulation in the fetus), mechanical compression on the heart and major blood vessels and could result in stillbirth.

Talking about the complexity of the case, Dr. Bhupendra S Avasthi, Founder & MD, Surya Hospitals said, “Pulmonary lobectomy is surgical removal of the sequestrated lobe of the lung and it is a treatment of choice. The chance of survival in a preterm baby with a large PS is reported to be as low as 20-25%. Most of them may not even survive to undergo definitive surgery.”

The family was explained about the prognosis for survival and long term outcomes. The worst prognostic marker In-utero in this fetus was a large lung lesion and the presence of hydrops. Urgent drainage of the pleural fluid was considered necessary to relieve the mechanical compression of the heart and attempt to salvage the fetus In-utero for longer period of gestation. 

After a detailed joint consultation with the feto-maternal medicine specialist and neonatology team, a shared decision for pleural fluid aspiration was undertaken and 90ml of pleural fluid was tapped under ultrasound guidance. However, the pleural fluid re-accumulated within 24 hours of the fetal intervention and hence it was planned to deliver the infant by cesarean section before further fetal compromise.

“After a series of discussion over choosing the right procedure, we zeroed down on repeating the procedure of pleural tapping just prior to delivery. We very carefully took this call in order to improve the neonatal resuscitative measures and provide time for the neonatal intubation and inter-costal drain placement”, said Dr.Vandana Bansal, Director, Fetal Medicine Department, Surya Hospitals.

On 2nd December 2018, the patient underwent an elective cesarean delivery after a repeat procedure of pleural tapping. The patient gave birth to a male child weighing 1.675 kg (of which 600 gms was estimated to be fluid related weight) with generalized swelling all over the body. The baby did not cry immediately after birth and was ventilated in the delivery room and immediately transferred to the neonatal intensive care unit (NICU) at Surya Hospital for further management.

According to Dr. Nandkishor Kabra, Director NICU, Surya Hospitals, “The infant required high-frequency ventilation, surfactant and insertion of chest drains within the first 30 minutes of age to expand the lungs. We conducted a CT scan of the chest as required to confirm the abnormality and also to establish the location and blood supply of the mass prior to surgery. After initial stabilization in the NICU, the baby underwent surgery (thoracotomy) on the 8th day of life for resection of the sequestrated lung mass.”

Dr. Jui Mandke, Paediatric Surgeon, Surya Hospitals states, this baby require a timely thoracic operation with removal of the abnormal lobe of the lung. This requires a highly skilled, precise surgery because if the delicate blood vessels of the lobe get avulsed, there is a torrential blood loss, resulting in loss of life in such a precariously placed baby. Equally important is the paediatric anaesthesia team (CAS). 

 A multidisciplinary team comprising of neonatologists, feto-maternal medicine specialists, paediatric surgeon, radiologist, cardiologist, and physiotherapist were involved in the treatment and care of the infant. He needed ventilator support for a period of 7 weeks in NICU. Full oral feeds were achieved by 8 weeks of age. Meticulous monitoring and management of infections, feeding, and nutrition was also undertaken. Follow up ultrasound of the brain, lung, and heart was found to be normal. The baby was finally discharged on 4th Feb 2019 (65th day of life) with a weight of 2 kg. The infant’s growth and development are normal.

The case highlights the enhanced capabilities of Indian experts in dealing with complex and critical diseases. The breakthrough offers hope for better paediatric treatment and care. PS account for 1-5% of all congenital lung malformations. Large sequestrations are mostly symptomatic and present in the fetal or new-born period. Expert antenatal surveillance and delivery in a centre with level 3 NICU, paediatric surgery and specialty services will be extremely essential to save such newborns.