The NICU Journey: Airway, Heart, and Feeding

The first few weeks in the Neonatal Intensive Care Unit (NICU) can feel like a whirlwind of specialists, monitors, and medical terms. For a baby with CHARGE syndrome, the NICU journey focuses on three critical life-sustaining priorities: breathing, heart function, and feeding ^[1][2]. Understanding this roadmap can help you navigate the early days and prepare for the surgeries your baby may need.

Priority 1: The Airway (Breathing)

The most immediate challenge for many infants with CHARGE is choanal atresia. This is a condition where the back of the nasal passage is blocked by bone or tissue ^[3]. Because newborns naturally breathe mostly through their noses, this blockage is life-threatening.

• Bilateral atresia (both sides blocked) requires urgent intervention, often involving the placement of an oral airway (a small tube in the mouth) or intubation to help the baby breathe until surgery ^[4].
• The Surgery: Surgeons typically use a transnasal endoscopic repair, using tiny cameras and tools through the nose to open the blockage ^[3][5].
• Healing: Many modern surgeons prefer “stentless” techniques or balloon dilation to keep the airway open while it heals, as traditional plastic tubes (stents) can sometimes cause extra scar tissue ^[6][7].

The “CHARGE Airway”

In addition to the nose, the “CHARGE airway” can be complex due to other issues like laryngomalacia (floppy airway) or vocal fold paralysis ^[8][9]. This makes anesthesia more delicate, requiring an experienced surgical team ^[9].

Because airway emergencies are a top safety risk throughout your child’s life, you must actively alert all future medical providers (including dentists, emergency room staff, or outpatient surgeons) about the “CHARGE airway” and always request experienced pediatric anesthesiologists ^[9].

Priority 2: The Heart

Heart defects occur in about 75-80% of children with CHARGE syndrome ^[10]. These are often conotruncal defects, which are issues with the main “outflow” structures of the heart.

• Common Defects: These may include Tetralogy of Fallot (a combination of four heart issues), Atrioventricular Septal Defects (AVSD), or a Patent Ductus Arteriosus (PDA), where a fetal blood vessel fails to close after birth ^[10][11].
• Management: Some heart issues need immediate surgery, while others are monitored or managed with medication. In some cases, a medication called prostaglandin (PGE) is used to keep the PDA open temporarily to maintain blood flow until a more permanent repair can be made ^[12].

Priority 3: Feeding and Swallowing

Feeding difficulties are nearly universal in CHARGE syndrome due to a combination of physical blockages and cranial nerve dysfunction ^[1][13].

• Esophageal Atresia (EA): Some babies are born with a gap in the esophagus (the tube from the mouth to the stomach), often with a Tracheoesophageal Fistula (TEF), an abnormal connection between the esophagus and the windpipe ^[14]. This requires surgical repair very early in life.
• The Swallowing “Switch”: Even without a physical blockage, the nerves that coordinate the complex “switch” between breathing and swallowing (Cranial Nerves IX and X) often don’t work perfectly ^[1]. This can lead to silent aspiration, where milk or saliva accidentally enters the lungs without the baby visibly coughing or choking. This is why doctors often order specialized swallow studies even if feeding seems normal ^[1].
• Feeding Tubes: To ensure safe nutrition and growth, many babies receive a G-tube (gastrostomy tube) placed directly into the stomach ^[15][13]. This is often a bridge that allows the baby to grow safely while they work on their swallowing skills over months or years.

Managing Multiple Surgeries

It is common for babies with CHARGE to undergo several procedures during their NICU stay. To minimize risks, the surgical team may try to “bundle” procedures—such as performing the choanal atresia repair and G-tube placement during the same anesthesia session ^[9].

Because of the airway complexities, it is vital that the anesthesiologist and ENT (Ear, Nose, and Throat surgeon) are comfortable with the unique challenges of CHARGE syndrome ^[9][16].