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NEONATAL SURGERY

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CONGENITAL DIAPHRAGMATIC HERNIA

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Disorder characterized by pulmonary hypoplasia due to intrauterine compression of the developing lungs by herniated viscera.

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EPIDEMIOLOGY

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  • Incidence is 1000 per year (1:2000–1:5000 live births); female:male 2:1

  • 7–10% gestations end in fetal demise

  • Defects more common on left side (about 80%)

  • Associated with anomalies (10–35%) including: Central nervous system (CNS) lesions, tracheobronchial abnormalities, omphalocele, cardiovascular (CV) lesions, skeletal and syndromes (trisomy 13, 18, 21, Beckwith–Weidemann, Brachmann–de Lange, and Pallister–Killian, among others)

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ETIOLOGY

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  • Unknown currently, though several pharmacologic and environmental factors have been implicated, including a possible role for vitamin A deficiency and/or retinoid regulated gene defects

  • Embryologic theory: Lack of closure of the posterolateral pleuroperitoneal canals in the 8th week of gestation fails to separate the thoracic and abdominal cavities

  • Portions of the diaphragm and pulmonary parenchyma arise from thoracic mesenchyme and if disrupted may lead to absence of part of hemidiaphragm and pulmonary hypoplasia

  • Most cases are sporadic; familial cases occur (2%)

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DIFFERENTIAL DIAGNOSIS

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  • Cystic adenomatoid malformation, cystic teratoma, pulmonary sequestration, bronchogenic cyst, neurogenic tumors, primary lung sarcoma, diaphragmatic eventration

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PATHOPHYSIOLOGY

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  • Herniation of abdominal contents into thoracic cavity through posterolateral foramen of Bochdalek

  • Diaphragmatic defect may be small or may include entire hemidiaphragm

  • Pulmonary vasculature has increased muscularization of pulmonary arterioles and decreased branching of vessels resulting in pulmonary hypertension

  • Lungs are hypoplastic due to chronic compression, with decreased numbers of bronchial branches on both ipsilateral and contralateral sides

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CLINICAL MANIFESTATIONS

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  • Most patients present with respiratory distress within the first hours of life secondary to severe pulmonary hypoplasia and associated pulmonary hypertension

  • 10–20% may have delayed presentation characterized by less severe pulmonary hypoplasia and pulmonary hypertension, as well as gastrointestinal (GI) symptoms (e.g., vomiting, abdominal pain, constipation)

  • Pneumothorax

  • On exam: Absence of breath sounds; bowel sounds in chest; scaphoid abdomen; increased anterior-posterior diameter of chest; shifted heart sounds

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DIAGNOSTICS

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  • Prenatal ultrasound able to detect defect as early as 11th week, mean gestational age (GA) at diagnosis is 24 weeks; accuracy has been reported to be between 40 and 90%

  • Antenatal diagnosis is associated with more severe defects and a worse prognosis; if diagnosed by ultrasound, fetal MRI should be performed

  • During fetal period, a lung-head ratio (LHR, area of contralateral lung to fetal head circumference by ultrasound) of <1 indicates severe disease

  • Observed-to-expected LHR (O/E LHR) accounts for changes in LHR with GA, and O/E LHR <25% suggests severe CDH

  • Echocardiography and amniocentesis to detect other anomalies

  • Chest x-ray

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MANAGEMENT

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Initial Medical Management
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  • Initial resuscitation includes correction of hypoxia, hypercarbia, acidosis, and hypothermia as they increase pulmonary vascular resistance and worsen pulmonary hypertension

  • Intubate early to avoid inflation of the stomach with bag-mask ventilation

  • Nasogastric decompression to decrease ...

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