Congenital Causes of Respiratory Distress

Fritz J. Baumgartner

This is an important topic since in general these are well-defined, surgically correctable lesions. The surgically correctable causes of respiratory distress in the newborn include bronchogenic cyst, Bochdalek's hernia, sequestration (usually intralobar), congenital lobar emphysema and cystic adenomatoid malformation. Resuscitation of the infant should be done with oxygen and intubation if necessary. Chest x-rays are important since it can be pathopneumonic in certain instances.

Bronchogenic cysts are lined with ciliated respiratory cells and can be found in the hilum, lung or mediastinum. Surgery should be performed for all bronchogenic cysts since they may compress the airway or become infected. Bronchogenic cysts may be found in adulthood as well (Fig. 20.1).

Bochdalek's hernia involves a defect in the posterolateral portion of the diaphragm, usually on the left, and the x-ray can be diagnostic since it shows intestine in the newborn's chest cavity compressing the lung with a shift of the mediastinum towards the right. Management includes intubation and resuscitation and immediate surgical intervention. The herniated bowel is reduced with repair of the diaphragm. The lungs may be hypoplastic from in utero compression of the lungs by bowel. A period of ECMO may be worthwhile in these situations if there is poor oxygenation despite intubation and oxygen therapy.

Sequestration consists either of the more common intralobar variety or the less common extralobar variety. The intralobar variety usually is in the left lower lobe, receives a systemic arterial supply usually directly off the aorta, drains by the pulmonary vein, and usually has a patent bronchus (Fig. 20.2). Lobectomy is the treatment of choice for intralobar sequestration. Extralobar sequestration likewise is primarily in the left lower lobe. There is usually no patent bronchus present. Venous drainage is via the azygous venous system. There are frequently other congenital anomalies present. No lobectomy is necessary since the incidence of pneumonia and pulmonary destruction is much less common because there is no patent bronchus present.

To differentiate intralobar and extralobar sequestration, CT scan may be necessary as may an arteriogram. Congenital lobar emphysema involves an isolated hyperinflation of the lobe without extrinsic obstruction. Symptoms of respiratory distress exist usually in the first week but may extend over several months. It is more common in males than females. The symptoms are tachypnea, dyspnea,

Fig 20.1a. Gross specimen of a large bronchogenic cyst of an adult male. A large amount of infected mucous and clot was retrieved from the cyst. Courtesy Drs. Bill Nelems and Ken Evans, University of British Columbia.

Fig 20.1b. The cut specimen shows the cyst in relationship to the lung parenchyma. Courtesy Drs. Bill Nelems and Ken Evans, University of British Columbia.

Fig 20.2a. Angiogram of the systemic arterial supply of an intralobar left lower lobe sequestration. Courtesy Drs. Bill Nelems and Ken Evans, University of British Columbia.

Fig 20.2b. A left lower lobectomy was performed revealing the large sequestration. Courtesy Drs. Bill Nelems and Ken Evans, University of British Columbia.

cyanosis and cough. Usually the left upper lobe is involved. In 25%, there is cartilaginous dysplasia of the lobe. In older children, bronchoscopy should be done to rule out foreign body. After intubation, the chest should be opened as soon as possible since positive pressure breathing will further hyperinflate the lung and may cause hemodynamic compromise. Cystic adenomatoid malformation involves overgrowth of the terminal bronchioles and lack of mature alveoli resulting in multiple cysts with both solid and cystic components. Respiratory distress may exist at birth. It may be confused radiographically with a Bochdalek's hernia.

Suggested Reading

1. Cullen ML, Klein MD, Philippart AI. Congenital diaphragmatic hernia. Surg Clin North Am 1985; 65:1115-1138.

2. Eigen H, Lemen RJ, Waring WW. Congenital Labor emphysema: Long-term evaluation of surgically and conservatively treated children. Am Rev Resp Dis 1976; 113:823-827.

3. Flye MW, Conley M, Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg 1976; 22:478-482.

4. Haller JA Jr., Golladay ES, Pickard LR et al. Surgical management of lung bud anomalies; lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration. Ann Thorac Surg 1979; 28:33-43.

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