WEEN Chapter 37: Paediatric Surgery CONGENITAL DIAPHRAGMATIC HERNIA Sees Congenital diaphragmatic hernia is a highly lethal or | Ses ecg morbid disease that affects 1 in 2000 live births. _ Anatomically, congenital diaphragmatic hemia results from an embryologic fusion defect, allowing hemiation of intra- abdominal contents into the chest. Fusion of the transverse septum and pleuroperitoneal folds normally ‘occurs during the eighth week of embryonic development (Fig. 37.7). If diaphragmatic formation is incomplete, the | tae os pleuroperitoneal hiatus (foramen of Bochdalek) persists | == (Fig. 37.8), Intestinal nonrotation is common as the bowel herniates into the thorax rather than undergoing its normal fond Capen ‘sequence of rotation and fixation. [Fig 377. Anatomy ofthe | daptvagm Severe defects cause pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. The larger the hernia and the earlier it occurs, the more severe the pulmonary | hypoplasia. | | Clinical Findings | A. Symptoms and Signs | Infants with large diaphragmatic defects are usually | symptomatic in the delivery room, with tachypnea, grunting | respirations, retractions, and cyanosis, and may require urgent intubation. | ‘Smaller defects may not become symptomatic until the infant |_Fig. 37.8. Boshdalek hema is several days or months old. Typically, the abdomen is scaphoid since much of the abdominal viscera is in the hemithorax. The chest on the side of the hemnia may be dull to percussion, but bowel sounds are not usually appreciated. The left side of the diaphragm is affected four or five times as frequently as the right, with a rate of associated anomalies of 20% (chromosomal abnormalities, neural tube defects, and congenital heart disease). When the hema is on the left, the heart sounds may be heard best on the right side of the chest. B. Imaging Studies A_chest_radiograph_may demonstrate the following (Fig. 37.9) * a paucity of gas within the abdomen, + radiopaque hemithorax if the bowel does not contain a significant amount of gas or if the left lobe of the liver occupies the majority of the Trig 378. Chest xray wi hemithorax, ; : Was . Bochdalek hemia. Intestinal loops * loss of normal ipsilateral diaphragmatic contour, that are filled with gas are seen * bowel in the thorax, inside the eft side of the chest. contralateral mediastinal shift, and heart is pushed to the right.Chapter 37: Paediatric Surgery * a coiled nasogastric tube in the hemithorax, Treatment A nasogastric tube should be placed in the stomach to aspirate swallowed air and to prevent distention of the herniated bowel, which would further compress the lungs. Repair of the diaphragmatic defect is NOT a surgical emergency and should be performed once _the infant has stabilized and has demonstrated minimal _to_no pulmonary hypertension A subcostal abdominal incision should be made and the herniated bowel reduced from the pleural space. Some surgeons prefer a thoracic approach, particularly for right-sided defects either open or thoracoscopically. Following reduction of the bowel, placement of a chest tube in the pleural space is optional; if used, it is connected to a water seal and not to vacuum. The diaphragmatic defect should be closed by nonabsorbable sutures. In many instances, a synthetic material is required to close large defects. Respiratory support and treatment of hypoxemia, hypercapnia, and acidosis are required before and often after repair. FORAMEN OF MORGAGNI HERNIA The foramen of Morgagni occurs at the junction of the septum transversum and the anterior thoracic wall. This anterior, central diaphragmatic defect accounts for only 2% of diaphragmatic hemias. It may be parasternal, retrostemal, or bilateral. Unlike Bochdalek hernias, children are typically asymptomatic and the defect is discovered later in life on a chest radiograph taken for reasons unrelated to the hemia. ‘The lateral chest radiograph demonstrating an air-filled mass extending into the anterior mediastinum is pathognomonic. Repair is indicated in the asymptomatic patient due to the risk of bowel obstruction. The viscera are reduced and any associated hemia sac excised. The defect is closed by suturing the posterior rim of diaphragm to the posterior rectus sheath since there is no anterior diaphragm. Rarely, a prosthetic patch closure is required. There is no associated pulmonary hypoplasia or hypertension. EVENTRATION OF THE DIAPHRAGM Diaphragmatic eventration is an abnormally elevated or attenuated portion of the diaphragm (or both). It may be congenital (usually idiopathic, but can be associated with congenital myopathies or intrauterine infections) or acquired (as a result of phrenic nerve injury during forceps delivery or surgery). In the congenital form, there is variable thinning or absence of diaphragmatic muscle. The elevated hemidiaphragm may produce abnormalities of chest wall mechanics with eeHEED Chapter 37: Paediatric Surgery impaired pulmonary function, Respiratory distress and pneumonia are frequent presenting symptoms. The diagnosis is made by chest radiograph. It is confirmed by fluoroscopy or ultrasound which demonstrate paradoxical movement of the diaphragm during spontaneous respiration. Eventrations that are associated with respiratory symptoms should be repaired by plicating the diaphragm using interrupted nonabsorbable sutures. CONGENITAL GASTROINTESTINAL LESIONS ESOPHAGEAL ANOMALIES The trachea and esophagus are derived from the primitive foregut. Initially, they appear as ‘a. common ventral diverticulum at about the nineteenth day of gestation. Beginning several days later, elongation and separation of the diverticulum into the airway and esophagus ‘occurs in a caudal to cephalad direction. Errors in this process result in esophageal atresia, tracheoesophageal fistula, and their variants. Classification 1. With Esophageal Atresia There is a blind proximal pouch and a fistula between the distal end of the esophagus and the distal one-third of the trachea (type C, 85% of cases) (Fig.37.10A). There is a blind proximal esophageal pouch, no tracheoesophageal fistula, and a blind, short distal esophagus (Type A, 10% of cases). This is referred to as pure or long gap atresia (Fig. 37.10B). There are fistulas between both proximal and distal esophageal segments and the trachea (Type D, 2% of cases). There is a fistula between the proximal —__ esophagus and the trachea and a blind distal esophagus without fistula (Type B, 1% of cases). 1 2 al 2. Without Esophageal Atresia + There is an H type tracheoesophageal There is esophageal stenosis consisting of fistula that is usually present in the low cervical region (Type E, 4-5% of cases). a membranous occlusion (often containing Fig. 37.10. A) Esophageal atresia with di cartilage) between the mid and distal third lracheoesophageal fistula (Type C). B ‘ae | of the esophagus (rare). Lesophageal atresia (Type A) : *| + There is a laryngotracheoesophageal cleft of varying length, consisting of communication between these structures (very rare), 19 Of a linear