Oesphegeal Atresia
Dr. Obay Abdulaziz F.I.B.M.S. (Ped.surg.)Esophageal atresia
Definition: It is a congenital anomaly that affecting the esophagus in which the esophagus is deficient in a varying degree with or without a fistulous connection to the trachea. Incidence: 1 in 4000 live birth esophageal atresia affects males slightly more commonly than females (1.26:1).Classification : Esophageal atresia with distal tracheo-esophageal fistula ( 85%) Isolated esophageal atresia ( 7%) Isolated TEF (H-type fistula) (4%) Esophageal atresia with double fistula(1%) Esophageal atresia with proximal fistula(0.8%)
Associated anomalies (55% of the cases)VACTERL association1. Cardiac VSD,TOF,ASD,PDA2.Ano-intestionalAnorectal atresia, duodenal atresia, malrotation, pyloric stenosis.3.Renal hypospadias, cryptorchidism, VUR,renal agenesis & hypoplasia 4.Vertebral & Skeletal radial limb deformity, vertebral anomalies.5.Chromosomol (Down syndrome ).
Clinical presentation Early presentationThe symptom that occurs shortly after birth is excessive salivation. Excessive salivation results from pooling of secretions in the proximal esophageal pouch and posterior pharynx. Feeding frequently results in regurgitation, choking, gagging, or cyanosis. Tachypnea, atelectasis, and respiratory distress result from reflux of gastric contents into the airway or aspiration from the proximal blind pouch. These events cause a chemical pneumonitis. Abdominal distension results from inspired air entering the gastrointestinal tract via the fistula and causes worsening respiratory distress and pulmonary compromise.
late presentation : (in H-type fistula( inhalation of milk (through the fistula) during feeding will cause chocking & even cyanosis & there will be recurrent severe chest infection.
Diagnosis
1-prenatal diagnosisUltrasonography Maternal Polyhydramnios (60-90%) Small or absent stomach bubble Blind – ending proximal esophageal pouch MRI can confirm the diagnosis2-postnatal diagnosis The diagnosis of esophageal atresia is strongly suggested when there is difficulty or inability to pass a nasogastric or orogastric tube. Resistance is typically encountered when the tube is passed to about 11-12 cm. 1. babygram x-ray frequently confirms the diagnosis in which nasogastric tube coiling within the proximal esophageal pouch.2.Contrast study (water soluble) show dilated upper esophageal pouch without visulization of lower esophagus & stomach,but this test not frequently performed.3.Bronchoscopy , to check the exact site of TEF, it is of importance in H- type fistula.Treatment Preoperative preparation; 1. ECHO study to check for CHD & side of aortic arch which is important for deciding the side of thoracotomy (which is opposite to the side of aortic arch). 2. frequent suction of saliva by using suction catheter (Replogle tube). 3. head up position to minimize aspiration of saliva. 4. Broad spectrum AB + H2 blockers + I.V.F. Operative management; by using either; Thoracotomy (RT sided/4th I.C.S) Thoracoscopy Post operative care; 1. Mechanical ventilation (in some cases). 2. Chest tube care 3. Broad spectrum AB. 4. Feeding through NG tube starting on day 5 postoperativelly.
Postoperative complications : 1. anastomotic leak, 2. stricture, 3. recurrent fistula formation 4. gastro-oesophageal reflux.
Congenital diaphragmatic hernia & eventration
Classification1. Bockdalek hernia (90%) 2. Morgagni hernia (2%) 3. Hiatal hernia (sliding, rolling) 4. Eventration of diaphragm
Associated anomalies:
Congenital heart diseases ( hypoplastic left ventricle,TOF,TGA,ASD,VSD). Pulmonary hypoplasia. Pulmonary sequestration. Intestinal malrotation. Chromosomal abnormalities.1.Bochdalek Hernia
Definition: refers to a congenital defect in the posterolateral diaphragm at the “foramen of Bochdalek.” It is a relatively common cause of neonatal respiratory distress with an overall incidence between 1:2000 and 1:5000 live births. Bochdalek hernia accounts for about 90% of congenital diaphragmatic defects & Eighty to ninety percent of it occur on the left side.Clinical presentations:
It present itself in two ways ;early presentation (immediately after birth or within 24 hr).Late presentation after several months or years (in 10%). The newborn baby presented with severe respiratory distress & Cyanosis. On examination: Tachypnea and grunting respiration. cyanosis.Scaphoid abdomen.Increase chest diameter.On auscultation of the chest: decrease breath sound on the affected side + bowel sound + Apex beat of the heart displaced to the right side (dextrocardia).Diagnosis: Prenatal diagnosis: Fetal U/S can detect this condition in as early as 25th wks of gestation ( bowel loop within the chest )Postnatal diagnosis:1.Chest x-ray :Presence of bowel loops in the chest, even an NG tube can be seen coiled in the left chest if the stomach is herniated up. Displaced mediastinum to the opposite side.Compression of the contra lateral lung.2.Barium study : (not routinely performed) presence of bowel loops filled with barium within the chest with shifting of mediastinum to opposite side.
:Treatment
Preoperative resuscitation : Oxygen therapy. Mechanical ventilation /ECMO (necessory in some patients) Nasogastric tube to minimize gastric distention which worsen respiratory distress. Iv-line & I.V.F. Antibiotics Operative management: via abdominal approach repair of the diaphragm done either primarily or by using prosthetic mesh for larger defects. Post operative care and follow up. same as esophageal atresia.2.Morgagni hernia
Definition: Morgagni hernias (retrosternal hernia) result from failure of the sternal and crural portions of the diaphragm to fuse at the site where the superior epigastric artery traverses the diaphragm. it acc0unt for about 2% of all types of diaphragmatic hernias.Clinical presentation:
The majority of children with Morgagni (retrosternal) hernias are asymptomatic. Diagnosis is often not made until adulthood. Children with this lesion may present with recurrent respiratory infections, coughing, vomiting or epigastric pain/discomfort. Intestinal obstruction and bowel ischemia/necrosis may result from incarceration of bowel within the hernia sac.Diagnosis:
CXR ( lateral view )is often demonstrate an air-fluid filled structure located immediately posterior to the sternum which represent the stomach or bowel. Barium study, it is not routinely performed.Treatment:
Morgagni hernias are surgically repaired via a transabdominal approach. Primary closure of small defects is preferred, but larger defects may require prosthetic patch closure.3.Diaphragmatic eventration
Definition: it is refer to a paralysis of one dome of diaphragm that result in paradoxical movement of diaphragm during respiration causing respiratory distress.Types of eventration: 1.congenital due to either defective enervation of diaphragm. traction injury to phrenic N. root during traumatic delivary. Congenital weakness in diaphragmatic muscles2.Acquired Result from iatrogenic phrenic N. injury during cardiac or mediastinal surgery.Secondary to phrenic n. invasion by mediastinal tumor.