Neonatal Surgical Issues (Part 1) Sue Ann Smith, MD Neonatologist

An anatomic survey Head and Neck lesions Head and Neck lesions Chest lesions Chest lesions Abdomen Abdomen Abdominal wall defects and infection Abdominal wall defects and infection

The Nose Choanal atresia – bilateral atresia Choanal atresia – bilateral atresia Respiratory distress resolves with crying Respiratory distress resolves with crying Treat with oral airway until surgical repair Treat with oral airway until surgical repair CT scan often used in surgical planning CT scan often used in surgical planning ENT surgeons make opening in bony plate and stent open during healing ENT surgeons make opening in bony plate and stent open during healing Nasolacrimal duct cysts – large and bilat Nasolacrimal duct cysts – large and bilat Respiratory distress resolves with crying Respiratory distress resolves with crying Treat with oral airway Treat with oral airway Can usually be seen with otoscope Can usually be seen with otoscope

Robin sequence AKA Pierre Robin syndrome AKA Pierre Robin syndrome Hypoplastic mandible with U-shaped midline cleft palate Hypoplastic mandible with U-shaped midline cleft palate Respiratory and feeding difficulties Respiratory and feeding difficulties Position prone, may require nasopharyngeal tube, oral airway, LMA, or endotracheal tube Position prone, may require nasopharyngeal tube, oral airway, LMA, or endotracheal tube Mandibular distraction is now treatment of choice at OHSU Mandibular distraction is now treatment of choice at OHSU

The Unusual Laryngotracheal clefts Laryngotracheal clefts Laryngeal webs Laryngeal webs Tracheal agenesis – frequently lethal Tracheal agenesis – frequently lethal Neck masses Neck masses Foregut duplication cyst Foregut duplication cyst lymphangioma lymphangioma

Normal Larynx Laryngeal Web

Congenital Chest Lesions Tracheo-esophageal fistula Tracheo-esophageal fistula Diaphragmatic Hernia (briefly) Diaphragmatic Hernia (briefly) Congenital lobar emphysema Congenital lobar emphysema Cystic adenomatoid malformation Cystic adenomatoid malformation Vascular rings Vascular rings

Tracheo-Esophageal Fistula (TEF) Esophageal atresia with TEF is most common (85%). Esophageal atresia with TEF is most common (85%). Diagnosis may be suspected antenatal with absence of stomach bubble and polyhydramnios. (*Caution: also seen with conditions that lead to poor swallowing) Diagnosis may be suspected antenatal with absence of stomach bubble and polyhydramnios. (*Caution: also seen with conditions that lead to poor swallowing) Often associated with other anomalies: VATER and chromosomal Often associated with other anomalies: VATER and chromosomal

Tracheo-Esophageal Fistula (TEF) (cont) Presentation: excessive salivation and intolerance of feedings. Presentation: excessive salivation and intolerance of feedings. Diagnosis: inability to pass catheter into stomach. Diagnosis: inability to pass catheter into stomach. Pre-op Management: avoid mechanical ventilation (if possible), catheter to suction in the esophageal pouch, elevate head of bed. Pre-op Management: avoid mechanical ventilation (if possible), catheter to suction in the esophageal pouch, elevate head of bed.

Operative management Ligation of fistula at trachea. Ligation of fistula at trachea. Mobilization of distal esophageal segment with primary anastamosis to proximal pouch. Mobilization of distal esophageal segment with primary anastamosis to proximal pouch. NG tube left in place to stent open anastamosis while healing. NG tube left in place to stent open anastamosis while healing. Chest tube left in for serous drainage usually. Chest tube left in for serous drainage usually.

Post-operative Management Careful airway management to prevent trauma to the fistula ligation site in the trachea. Careful airway management to prevent trauma to the fistula ligation site in the trachea. Prior to feedings, must make sure that the esophageal anastamosis does not leak. (swallow study) Prior to feedings, must make sure that the esophageal anastamosis does not leak. (swallow study) Often have on going feeding problems. May need dilation procedures periodically Often have on going feeding problems. May need dilation procedures periodically

Other “TEFs” Esophageal atresia without TEF – very rare Esophageal atresia without TEF – very rare H-Type TEF-also rare. H-Type TEF-also rare. Diagnosis usually after the neonatal period with frequent pneumonias or respiratory distress related to feedings Diagnosis usually after the neonatal period with frequent pneumonias or respiratory distress related to feedings

Congenital Diaphragmatic Hernia (CDH) Most commonly on left side Most commonly on left side Incidence 1:2000 to 1:5000 Incidence 1:2000 to 1:5000 Often associated with other malformations Often associated with other malformations Frequently diagnosed prenatally Frequently diagnosed prenatally Avoid bag-mask PPV Avoid bag-mask PPV

Pre-op CDH Delayed surgical repair – usually after 72 hrs of age Delayed surgical repair – usually after 72 hrs of age NG drainage tube to keep bowel decompressed NG drainage tube to keep bowel decompressed Treat aggressively for pulmonary hypoplasia and Persistent Pulmonary Hypertension – including ECMO(?). Treat aggressively for pulmonary hypoplasia and Persistent Pulmonary Hypertension – including ECMO(?). Surfactant therapy is now controversial Surfactant therapy is now controversial

Post-Op CDH “Anatomy is destiny” “Anatomy is destiny” Survival continues to be around 40-50%. Survival continues to be around 40-50%. Feeding difficulties Feeding difficulties

Congenital lobar emphysema Lesions that cause air trapping, with compression of surrounding tissue Lesions that cause air trapping, with compression of surrounding tissue Most common in left upper, right middle and right upper lobes Most common in left upper, right middle and right upper lobes Usually attempt low volume ventilation. Sometimes selective intubation of other bronchus Usually attempt low volume ventilation. Sometimes selective intubation of other bronchus May require surgical resection May require surgical resection

Congenital Cystic Adenomatoid Malformation (CCAM) May be confused with CDH May be confused with CDH Abnormal lung tissue that forms fluid filled cysts. May be large cysts, or many small cysts and solid areas Abnormal lung tissue that forms fluid filled cysts. May be large cysts, or many small cysts and solid areas Space occupying lesion Space occupying lesion May cause shifting of mediastium May cause shifting of mediastium May spontaneously regress in fetus May spontaneously regress in fetus May require surgical removal May require surgical removal

Vascular Rings Uncommon Uncommon Signs include stridor, vomiting and difficulty swallowing. Signs include stridor, vomiting and difficulty swallowing. Barium swallow can be diagnostic, but may need chest MRI. Barium swallow can be diagnostic, but may need chest MRI. Sometimes may need cardiac catheterization Sometimes may need cardiac catheterization

The Abdomen Abdominal wall Abdominal wall defects defects infection infection Bowel Bowel Obstructions Obstructions

Gastroschisis Abdominal wall defect to right of umbilicus with no covering over intestines Abdominal wall defect to right of umbilicus with no covering over intestines Rarely associated with other anomalies Rarely associated with other anomalies Most babies are SGA and born to young mothers (why?) Most babies are SGA and born to young mothers (why?) 10% will have intestinal atresias 10% will have intestinal atresias Rarely will have significant infarction of most of small bowel (i.e. lethal) Rarely will have significant infarction of most of small bowel (i.e. lethal) Most will have “meconium” stained amniotic fluid (really bile) Most will have “meconium” stained amniotic fluid (really bile)

Gastroschisis Pre-op Empty stomach (usually lots of bilious fluid) Empty stomach (usually lots of bilious fluid) NG tube for decompression NG tube for decompression Place in bowel bag or wrap in warm saline soaked gauze and saran wrap Place in bowel bag or wrap in warm saline soaked gauze and saran wrap Support the bowel so as to maintain perfusion Support the bowel so as to maintain perfusion

Gastroschisis (post-op) Primary closure is attempted Primary closure is attempted May require silo with slow return of intestine into small abdominal cavity May require silo with slow return of intestine into small abdominal cavity Maintain perfusion Maintain perfusion Feeding difficulties are main post-op problem Feeding difficulties are main post-op problem At risk for adhesions throughout life At risk for adhesions throughout life

Omphalocele Abdominal wall defect at umbilicus with covering (sac may rupture) Abdominal wall defect at umbilicus with covering (sac may rupture) Frequently associated with other anomalies Frequently associated with other anomalies Giant omphaloceles: respiratory issues with misshaped chest and airway malacias Giant omphaloceles: respiratory issues with misshaped chest and airway malacias

Omphalocele Decompress stomach initially Decompress stomach initially Careful eval for other anomalies Careful eval for other anomalies Intact sac may defer operation for years Intact sac may defer operation for years “paint” membrane with betadine to toughen into a “rind” “paint” membrane with betadine to toughen into a “rind” Ruptured sac – repair similar to gastroschisis Ruptured sac – repair similar to gastroschisis

Omphalitis Presentation – erythema/induration of the periumbilical area with purulent discharge from umbilical stump. Presentation – erythema/induration of the periumbilical area with purulent discharge from umbilical stump. Can spread extensively to abdominal wall or develop necrotizing fasciitis. Can spread extensively to abdominal wall or develop necrotizing fasciitis. Both gram + and gram neg bacteria implicated Both gram + and gram neg bacteria implicated Full sepsis evaluation Full sepsis evaluation Oxacillin/nafcillin and gent Oxacillin/nafcillin and gent

Normal Larynx (upside down) Laryngeal Web (also upside down)