Surgical Emergencies in the Newborn University of North Carolina at Chapel Hill

Surgical Emergencies in the Newborn

• University of North Carolina at Chapel Hill

• Pediatric Surgery Division

• Patty Lange

• Last revised 4/15/06

Emergencies

• Types

• Airway/Respiratory
• Intestinal Obstruction
• Intestinal Perforation

• Signs

• Respiratory distress
• Abdominal distension
• Peritonitis
• Pneumoperitoneum

Airway/Respiratory

• Neck Masses

• Cystic Hygromas
• Tracheal anomalies

• Thoracic masses/pulmonary lesions

• Congenital lobar emphysema
• Overdistension of one or more lobes (nl histological lung)
• Congenital cystic adenomatous malformation
• Multicystic mass of lung tissue, proliferation of bronchial structures at the expense of alveoli
• Pulmonary agenesis
• Absence of lung
• Congenital diaphragmatic hernia
• Tracheoesophageal fistula

Cystic Hygroma

• Multiloculated cystic spaces lined by endothelial cells

• Separated by fine walls containing numerous smooth muscle cells
• Result of maldevelopment of lymphatic spaces

• Incidence about 1 in 12,000 births

• 50-65% appear at birth, 85-90% appear by age 2
• Neck-75%, Axilla 20%; can be seen in mediastinum, retroperitoneum, pelvis, groin
• Nuchal/post cervical CH’s have been associated with chromosomal abnormalities—high mortality rate

Cystic Hygroma

• Complications

• Respiratory—large hygromas can extend into oropharynx and trachea
• Inflammation/Infection
• Hemorrhage

• Treatment

• Dependent on size, location, symptoms/complications
• Some pts require emergent surgery due to airway compromise
• Best treatment is complete excision
• Aspiration typically not effective due to rapid refilling of fluid
• Sclerotherapy—Bleomycin, OK-432 (no longer available in US), doxycycline, fibrin glue

Cystic Hygroma

Cystic Hygroma

Congenital Lobar Emphysema

• Postnatal overdistension of one or more lobes of histologically normal lung

• Probably due to cartilaginous deficiency in the tracheobronchial tree
• Obstruction causing the overdistension may be due to
• 1—chondromalacia of bronchi
• 2—extrinsic pressure on bronchus by anomalous pulmonary vein or abnormally large PDA
• 3—idiopathic

• Location

• LUL 47%, RML 28%, RUL 20%; lower lobes <5%; Bilat rare

Congenital Lobar Emphysema

• Diagnosis

• Usually can be made by plain CXR; Chest CT and V/P scans may be helpful

• Treatment

• May require urgent surgical decompression with lobectomy
• Selective bronchial intubation
• Sometimes see spontaneous resolution—need close observation

Congenital Lobar Emphysema

Congenital Cystic Adenomatous Malformation (CCAM)

• Mass of cysts lined by ciliated cuboidal or columnar pseudostratified epithelium

• Three types

• I—few large cysts >2cm; thick walls, normal alveoli between the cysts; ciliated pseudostratified columnar epithelium
• II—numerous small cysts <1cm, thin muscular coat, large alveolar-like structures between the cysts; ciliated cuboidal to columnar epithelium; assoc w/other congenital anomalies
• III—bulky firm masses of folded ciliated and non-ciliated cuboidal epithelium and thick layer of smooth muscle; often occupy the entire lobe or lobes of lung

• More common on the left side, 2% bilateral

CCAM

• Diagnosis

• CT scan allows differentiation of types
• Some can be diagnosed on prenatal US

• Treatment

• Surgical excision, typically anatomical lobe resection, due to risk of infection, malignant transformation
• Some are performing fetal aspiration

CCAM

Congenital Diaphragmatic Hernia

• Intro

• 1 in 200-5000 live births, females >males
• Etiology unknown
• Large percentage of fetuses are stillborn
• Still high mortality of those that make it to birth

• DX

• Frequently made prenatally
• CXR

• Treatment

• Respiratory support
• ECMO
• Primary closure or patch closure when pt stable

Tracheoesophageal Fistula and Esophageal Atresia