Baby Justin was diagnosed with Congenital High Airway Obstruction detected in utero --1 of only 50 cases reported in medical literature in 20 years

NEW HYDE PARK, NY – A Long Island boy born with a totally blocked airway – one of only 50 reported cases in the past 20 years – will be heading home in the coming days after four months in Schneider Children’s Hospital of the North Shore-LIJ Health System.

Accompanied by his parents Derly and Julian Olivares, Justin Olivares was introduced today at a news conference, where the family thanked six of the more than 35 physicians and nurses who assisted in his dramatic September 22 delivery at Long Island Jewish (LIJ) Medical Center.

The baby’s condition, known as Congenital High Airway Obstruction Syndrome (CHAOS), was detected while he was still in his mother’s womb, during a prenatal examination at LIJ. At some point between six and eight weeks of gestation, the baby’s airway (about ½-inch in diameter) failed to develop. Without surgical intervention, the baby would have been unable to breathe once he was detached from his mother’s placenta. Besides cutting off oxygen, CHAOS causes fluid produced in the lungs to accumulate, resulting in the dangerous enlargement of the lung. This leads to abnormal development and function of the lung and diaphragm (breathing muscle).

In addition to the blocked airway, it was discovered in utero that Justin would be born with ventricular septal defect -- a hole in the wall between the heart’s two major pumping chambers. Fortunately, doctors reported today that the hole is beginning to close on its own, so future cardiac surgery may prove unnecessary.

“The extreme challenge of CHAOS called for the close coordination of maternal fetal medicine specialists from LIJ Medical Center and North Shore University Hospital, and teams of physicians and nurses from numerous pediatric sub-specialties at Schneider Children’s Hospital of the North Shore-LIJ Health System, including anesthesiology, otolaryngology, neonatology, medicine and cardiology, said Dennis Davidson, MD, chief of neonatology at the Children’s Hospital.
At the time of Justin’s delivery, the clinical team established an airway while he was still being supported by his mother’s placenta, which was necessary to ensure that he received enough oxygen during the procedure, so that he would not suffer severe brain damage -- or death.

The procedure was Justin’s only option, but was a major risk to his mother. It was decided that the delivery would take place at 36 weeks, before Mrs. Olivares went into natural labor, so physicians could control the complicated process of performing a tracheostomy (insertion of a breathing tube) while Justin was still attached to his mother’s placenta. In Justin’s case, the obstruction was bypassed by fetal surgery, and he was delivered and given oxygen through mechanical ventilation.

Now, after four months in the Neonatal Intensive Care Unit at  Schneider Children’s Hospital, Justin is breathing on his own, has developed normally, and is preparing to go home to live with is parents and eight-year-old brother, Jason, who told his parents that he is very happy to be a big brother to this miracle baby.

Among the physicians and nurses from Justin’s clinical team who participated in today’s news conference besides Dr. Davidson were:
• Natalie Meirowitz, MD, director, maternal-fetal Medicine, LIJ Hospital;
• Nidhi Vohra, MD, director, Maternal-fetal medicine, North Shore University Hospital;
• Preeta Dhanatwari, MD, director, fetal imaging, Schneider Children’s Hospital;
• Lee Smith, MD, director, pediatric otolaryngology, Schneider Children’s Hospital; and
• Alison Civale, RNC, assistant nurse manager, NICU, Schneider Children’s Hospital.

Contact: Michelle Pinto
(516) 465-2649
mpinto@nshs.edu

To see an illustration and a video of part of Justin’s procedure, go to http://accordent.nslijweb.com/olivares_surgery.html.