Aydin Idris Martin, an infant with an extremely rare and serious birth defect, was saved at birth when a multidisciplinary team at NYU Langone’s Hassenfeld Children’s Hospital performed a highly specialized procedure.
The procedure was the first step in his journey to surviving congenital obstructive airway syndrome (CHAOS), a condition that inhibits an infant’s ability to breathe at birth. After 169 days in Tisch Hospital’s Neonatal Intensive Care Unit (NICU), followed by more than 5 months in a rehabilitation facility where he overcame further challenges related to CHAOS, Aydin is finally home with his parents Yadi and Jamaal Martin, to celebrate his 1st birthday.
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A prenatal diagnosis of CHAOS
CHAOS is a congenital disorder that occurs when the trachea, also known as the trachea, becomes blocked or not fully formed during fetal development. The condition, whose causes are still unknown, has a 100 percent mortality rate unless surgery is performed at birth.
The Martins were diagnosed in May 2021, 24 weeks after Yadi’s pregnancy. A fetal echocardiogram that showed abnormalities prompted Dr. James C. Nielsen, Medical Director of the Pediatric Congenital Heart Defects Program at Hassenfeld Children’s Hospital, to consult with Ashley S. Roman, MD, Vice Chair of Clinical Affairs for Obstetrics, Co-Director of the Fetal Diagnosis and Treatment Program and Director of the Division in Maternal-Fetal Medicine in the Department of Obstetrics and Gynecology at NYU Grossman School of Medicine. She immediately confirmed the diagnosis of CHAOS.
“The ultrasound showed large, hyperinflated lungs, and I knew immediately what was going on,” says Dr. Novel. Yadi’s unborn child was only the second she had seen with CHAOS in her 20-plus-year career and the 51st case to be publicly reported in the United States since 1989. “Due to the airway blockage, lung fluid cannot escape from the fetal mouth, causing the lungs to fill and swell immensely. The condition would make breathing impossible and deadly for the baby after a traditional birth.”
After four previous miscarriages, this wasn’t the news the couple hoped to hear near the middle of their pregnancy. “That was our miracle baby,” says Yadi. “We have prayed and prayed for him.”
“As soon as we learned of the diagnosis, we assembled a multidisciplinary team to begin the months of preparation it took to ensure everything ran smoothly in the delivery room,” says Jason C. Fisher, MD, pediatric surgeon, director of the Pediatric Surgery for Hassenfeld Children’s Hospital and Director of the Department of Pediatric Surgery at NYU Grossman School of Medicine. “In a case this complex, you can’t handle it alone. They need a medical center that offers all of these subspecialties in one setting, and that’s Hassenfeld Children’s Hospital at NYU Langone.”
Instead of a traditional birth, Aydin required an innovative surgical approach called the Ex Utero Intrapartum Treatment, or EXIT, that would create an airway before separating it from the placenta.
“The most important thing in CHAOS is early diagnosis, detailed evaluation of the fetus, and adequate postnatal intervention to establish an airway,” says pediatric head and neck surgeon Scott M. Rickert, MD, director of the Division of Pediatric Otolaryngology at NYU Grossman School and co-director of the Pediatric Aerodigestive Center at Children’s Hospital Hassenfeld.
Aware of the risks and obstacles, the Martins were determined to become a family of three.
About the EXIT procedure
On August 3, 2021, at the age of 38, Yadi had an EXIT procedure, a high-risk, complex birth for her and the baby.
In the womb, the fetus exchanges oxygen via the umbilical cord. “Once the umbilical cord is cut,” says Dr. Roman, “The baby isn’t getting any more oxygen because he can’t breathe on his own due to the blocked airway.”
During the EXIT procedure, the baby’s head and shoulders are delivered via a partial caesarean section, leaving everything below the breast in the uterus. At this point, the umbilical cord and placenta remain intact, allowing them to continue delivering oxygen while pediatric surgeons work to create an airway.
“It’s like pausing in the middle of labor,” says Dr. Novel. “Once this baby’s head comes out, there is a 5 to 10 minute window to establish an airway, which requires complete coordination and months of practice and planning.”
Due to the complexity of the case, the Martins had a team of more than 25 service providers within NYU Langone. Specialists in maternal-fetal medicine, neonatology, pediatric surgery, pediatric ear, nose and throat and pediatric anesthesiology all worked together to deliver Aydin.
The EXIT delivery team consisted of Dr. Roman, who directed the baby’s surgery and delivery so the airway could be established; dr Rickert, who worked to evaluate Aydin’s airway and perform a tracheostomy, a surgical procedure to create an opening through the neck into the windpipe; and dr Fisher, who performed further interventions for Aydin after the birth.
“One of the biggest challenges I faced before giving birth was not fully understanding how blocked the airways were,” says Dr. Rickert. “During the evaluation, there was no opening to the airways. So we bypassed the blockage by inserting a tracheotomy and successfully performed a quick procedure to then complete the delivery. But just because we had a secure airway and he was breathing securely didn’t mean Aydin was completely free.”
CHAOS syndrome comes with attendant challenges. After Aydin was successfully delivered, the care team discovered more obstacles ahead.
Complex care in the OR
Aydin’s lungs were inflated from the airway obstruction. This put pressure on his heart and affected its ability to function properly. Shortly after the EXIT, neonatologists began resuscitating Aydin, but it was clear his heart was not recovering. dr Fisher made the quick decision to start ECMO, or extracorporeal membrane oxygenation, a treatment that does the work of the heart and lungs and gives them time to heal.
“Once he was stable we made one final discovery – Aydin had another birth defect. An anorectal deformity, meaning his anus didn’t form properly,” explains Dr. Fisher. “We planned for every contingency, and he still threw us a few curveballs.”
Aydin was transferred to the NICU in critical condition – still on ECMO but with an assisted airway established.
Dedicated care in the neonatal intensive care unit
24 hours after birth, the nursing team made the decision to wean Aydin from ECMO support. “Aydin’s success in stopping ECMO therapy was a clear indication that his health was beginning to improve,” says Dr. Fisher.
Over the next 169 days in the NICU, Aydin miraculously grew stronger, overcoming various obstacles and achieving key milestones along the way.
On September 7, 2021, when he was just over a month old, Yadi and Jamaal Aydin were finally able to hold for the first time. It was a big moment for the grateful parents. “We are so lucky to have him alive and in our arms,” the Martins recall.
Aydin has had other procedures, including heart surgery to correct his congenital heart defect, performed by TK Susheel Kumar, MD, pediatric cardiac surgeon in the Pediatric Congenital Heart Program at Children’s Hospital Hassenfeld; and a procedure to correct his anorectal deformity performed by Dr. Fisher, who also directs the newly established Pediatric Complex Colorectal Care Program.
On Yadi’s 39th birthday, January 19, 2022, Aydin was transferred from Hassenfeld Children’s Hospital NICU to Children’s Specialized Hospital in New Brunswick, New Jersey, a rehabilitation facility that would help the Martins bring Aydin home.
A bright future ahead
Aydin continued to improve. He was brought back to Brooklyn for the first time six months later and celebrated his first birthday on August 3rd. The Martins are most looking forward to seeing Aydin grow up like a normal baby now that he is home and discharged from the hospital.
Aydin’s care team is optimistic about his future. “Although Aydin is doing remarkably well, he will need at least one more operation before he can fully breathe on his own,” says Dr. Rickert. “We plan to continue monitoring Aydin’s airway and perform reconstruction over the next few years when he has grown slightly.”
“The team at the Hassenfeld children’s clinic is now family to us,” says Yadi. “We were in the safest place we could be for ourselves – and for Aydin. I am forever grateful.”