5.3 Congenital Respiratory Anomalies and Surgery

Congenital Respiratory Anomalies and Surgery

Quick Answer: Congenital diaphragmatic hernia (CDH) puts bowel in the chest, hypoplastic lungs, and a high risk of PPHN. Delivery-room priorities are immediate endotracheal intubation, gastric decompression, and avoiding prolonged bag-mask ventilation that inflates the gut. Repair is delayed until physiology is stable. Tracheoesophageal fistula with esophageal atresia (TEF-EA) presents with polyhydramnios, choking on secretions, and a coiling gastric tube on radiograph; a Replogle (or similar continuous-suction pouch tube) protects the airway until staged or primary repair. Bilateral choanal atresia causes cyanosis when the mouth is closed or during feeds and is relieved by crying or an oral airway. Tracheomalacia, tracheal stenosis, tracheal atresia, congenital pulmonary airway malformation (CPAM), and chylothorax complete the anomaly list. Perioperative nursing is glucose, temperature, anastomosis and chest-tube surveillance, and a PPHN watch after CDH repair.

Congenital anomalies and surgery are listed respiratory leaves on the current Neonatal CCRN Test Plan. This OpenExamPrep section stays at the crib: what you do in the first minutes, what you watch after the repair, and which findings change the call to surgery. It is independent teaching, not an operative atlas and not an endorsement claim.

CDH: bowel in the chest is a physiology problem

CDH occurs in roughly 1 in 2500–5000 live births. Left-sided Bochdalek (posterolateral) defects are the majority, often about 80–85%. Abdominal viscera occupy the thorax during the canalicular and saccular stages of lung growth, so both lungs—especially the ipsilateral lung—are hypoplastic, with too few alveoli and too much pulmonary vascular muscle. That is why CDH is a PPHN disease as much as a surgical hole.

Bedside clues: scaphoid abdomen, barrel chest, bowel sounds in the thorax, shifted heart tones, and immediate respiratory failure in a neonate who may have had a prenatal diagnosis. A right-sided defect can look like an unexplained opaque hemithorax. Quiet-term vital signs are useless as “normal” here: expect a respiratory rate of 80–100, saturations that need an ETT, and a heart rate of 160–180 until the infant is anesthetized and ventilated.

Delivery-room priorities, in order:

1. A team that can intubate immediately—not a “try CPAP first” reflex.
2. Immediate ETT. Prolonged bag-mask fills the stomach and intestine in the chest, worsens mediastinal shift, and drops venous return.
3. Replogle or large-bore NG/OG to suction so the gut deflates.
4. Pre-ductal saturation (right hand) as the brain-and-coronary number; post-ductal saturations track ductal right-to-left flow.
5. Gentle ventilation. Many CDH pathways accept permissive hypercapnia (for example PaCO2 in the 50s–60s if pH is acceptable) rather than chasing a normal adult CO2 with high PIP.
6. Treat hypoxia, acidosis, hypothermia (keep 36.5–37.5 °C), and hypoglycemia (many units intervene below 40–50 mg/dL)—the same PPHN fuels you already know.
7. Delayed repair. The diaphragm is closed after pulmonary vascular resistance has been optimized—often after 24–72 hours, sometimes longer—not as a midnight crash laparotomy in an infant whose oxygenation index is 50. ECMO, when used, is a bridge for reversible hypertension and hypoplasia severe enough to fail medical therapy, not a cosmetic delay.

Postoperative CDH is a pulmonary hypertension crisis watch: pain, suction, hypoxia, and acidosis can slam the right ventricle. The repaired diaphragm does not instantly grow alveoli. Delayed abdominal closure is used when viscera will not fit without wrecking venous return.

Worked example: a prenatally diagnosed left CDH infant is born and a well-meaning helper bags for 90 seconds because the first intubation attempt failed. The abdomen becomes less scaphoid, saturations fall, and the heart tones move further right. That is gut inflation. Alternate airway (video laryngoscope, laryngeal mask as a bridge per current NRP-style failed-intubation thinking) and decompress—do not bag harder.

TEF-EA: secretions with nowhere to go

Esophageal atresia with or without tracheoesophageal fistula occurs in about 1 in 2500–4000 live births. Maternal polyhydramnios is a clue because the fetus cannot swallow amniotic fluid into the gut. After birth: copious frothy saliva, choking, cyanosis with the first feed, and a gastric tube that coils in a blind proximal pouch on the radiograph. The most common anatomy (often called Gross type C) is proximal esophageal atresia with a distal TEF—gas is then seen in the stomach. A gasless abdomen suggests EA without a distal fistula (long-gap risk).

Replogle tube: a double-lumen tube in the pouch on continuous low suction to keep saliva out of the lungs. Head of bed elevated, NPO, IV glucose, and suction the mouth. Avoid bag-mask that forces air through a distal fistula into the stomach. If the infant needs an ETT, be ready for a stomach that distends through the fistula.

VACTERL association (vertebral, anorectal, cardiac, TE fistula, renal, limb) is the systems search: echo, renal ultrasound, spine, anus, limbs. Do not send an infant to the OR without knowing whether a ductal-dependent heart is hiding. A pink infant with EA can still have a closing coarctation on day 3.

Surgery: primary anastomosis when the gap is short; staged repair (fistula ligation, gastrostomy, later esophageal replacement or delayed anastomosis) when the gap is long. Postoperative nursing: neck position to protect the anastomosis (avoid extreme extension), oropharyngeal suction that does not blindly enter the esophagus beyond a marked depth, watch chest tubes for saliva (anastomotic leak), and later tracheomalacia and gastroesophageal reflux, which are common after EA. A transanastomotic feeding tube, if left, is not a toy; if it comes out, do not replace it blindly.

Choanal atresia and tracheal lesions

Newborns are preferential nasal breathers. Bilateral choanal atresia (choanal atresia overall is often cited near 1 in 5000–8000 live births; bilateral is the emergency subset) presents as cyanosis at rest or with feeding that pinks with crying (mouth open). An oral airway, McGovern nipple, or similar mouth-breathing stent is first aid. Failure to pass a 5–6 Fr catheter through the nose is a bedside clue; CT or nasal endoscopy confirms. CHARGE association (coloboma, heart defects, choanal atresia, growth or developmental delay, genital, ear) is the related search. Unilateral atresia can wait and presents as chronic unilateral discharge.

Tracheomalacia is dynamic collapse of the tracheal wall, often worse on expiration and with agitation, feeding, or a collapsing innominate artery. Stridor or biphasic noise, desaturation with crying, and a honking cough are clues. Prone positioning, CPAP, treating reflux, and time are common; aortopexy is uncommon and surgical. After TEF repair, expect some malacia; not every noisy exhale is a recurrent fistula.

Tracheal stenosis (including complete tracheal rings) is a fixed narrow airway. A too-tight ETT, failed extubation, and a fixed biphasic stridor are clues. Do not force a standard-size ETT; leak and tube size become surgical conversations (slide tracheoplasty in severe complete rings).

Tracheal atresia is rare and often fatal without an EXIT-to-airway strategy or immediate surgical airway because there is no trachea to intubate from the mouth. Prenatal polyhydramnios and absent tracheal fluid on imaging may have been clues. This is not a bag-mask problem and it is not choanal atresia.

CPAM and chylothorax

Congenital pulmonary airway malformation (CPAM)—the current name for what many still call CCAM—is a hamartomatous cystic lung mass. Large lesions cause hydrops prenatally or mediastinal shift and distress postnatally. Smaller lesions may be found on prenatal ultrasound and remain quiet. Symptomatic neonatal CPAM can need lobectomy. Infection risk in cystic lesions is why some asymptomatic infants still have elective resection later—follow the surgical service, do not invent a universal week-2 rule. Nursing: respiratory rate, mediastinal shift, and air leak after resection.

Congenital chylothorax is lymphatic leak into the pleura. The infant has an effusion, respiratory distress, and after feeds the fluid becomes milky. Pleural triglycerides often exceed about 110 mg/dL. Management is drainage, NPO then medium-chain triglyceride formula or parenteral nutrition, and sometimes octreotide. Watch lymphocyte loss, coagulation, and malnutrition. Postoperative chyle after CDH or cardiac surgery is the acquired twin of this lesion.

Perioperative nursing that actually prevents a bounce-back

Shared preoperative work for this cluster: neutral thermal environment, glucose, gastric decompression when indicated, airway plan, prostaglandin only if a cardiac VACTERL lesion needs it, and family teaching that repair timing follows physiology.

Shared postoperative work:

• CDH: PPHN crisis prevention, pain control before suction, chest and abdominal inspection, delayed sternal or abdominal closure in some tight repairs.
• TEF-EA: anastomosis leak, recurrent fistula (choking and gas in the gut after feeds when it should not be there), vocal-cord function, transanastomotic tube security if one is left.
• Choanal repair or stents: nasal stent hygiene, oral feeding safety, CHARGE workup results.
• CPAM resection: air leak, bleeding, pain, remaining-lung expansion.
• Chylothorax: drain output in mL/kg, nutrition, infection from lost lymphocytes.

Exam traps: bag-masking CDH; feeding a suspected EA; treating bilateral choanal atresia as “just nasal congestion”; rushing CDH repair before PVR falls; and describing this OpenExamPrep chapter as official surgical technique training.

Independent practice items live at /practice/ccrn-neonatal.