الفهرس 
Embryology and Anatomy
Genetic basis of CDHOnly 14 pages are availabe for public view
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Abstract
Congenital diaphragmatic hernia (CDH) is a congenital defect in diaphragm development, which occurs in approximately in 2500- 3500 live births. CDHs occur mainly during the eighth to the tenth weeks of fetal life. They consist of Bochdalek, Central and Morgagni hernias. It can be detected during fetal life when screening ultrasonography demonstrates herniation of the intestine and/or liver into the thorax. The cardinal aspects of CDH pathophysiology are pulmonary hypertension with persistence of a fetal circulatory pattern, along with a varying degree of lung hypoplasia. CDH may manifest itself at any age by mild respiratory distress or it can be even be an unexpected finding during a medical checkup for other reasons. Following birth, a plain chest radiograph is almost always enough to confirm the diagnosis. The typical image is that of bowel loops seen within the lung fields, with deviation of the mediastinum to the contralateral side of the hernia, and decrease or absence of gas in the abdomen. Following a period of preoperative stabilization and optimization; patients with CDH are surgically repaired via open or MIS approach. The utilization of MIS approaches have been suggested to be advantageous over traditional open surgery including less pain and wound complications, avoidance of thoracotomy- related sequlae. The current study was conducted on 20 patients presented to the Pediatric Surgery Unit in Tanta University Hospitals with Bochdalek CDH from 1st day of life to 2 years.The patients age ranged from 2 – 693 days. They included 14 males (70%) and 6 females (30%). The patients’ weight ranged from 2100 – 13000 gm with no family history in any case. We had sixteen patients (80%) presented with neonatal respiratory distress (Early Presenters) and admitted to NICU while four patients (20%) presented with repeated chest infection and dyspnea (Late Presenters) and admitted to pediatric surgical ward. All cases had underwent thoracoscopic repair of the defect by Ibis head silhouette repair. The first stitch is so crucial as it was taken between the posterior muscle edge and the lateral rib cage where it met the anterior muscle edge, to reorient the diaphragmatic defect into a reversed C-shaped line, composed of two portions; one between the posterior diaphragm and the rib cage (laterally) and the other one between the muscle and itself(medially). The lateral limb was closed by rib-anchoring stitches while the medial limb was closed by muscle to muscle stitches. There were 5 cases (25%) of right CDH while 15 cases (75%) of left CDH. Primary repair had been completed in all cases thoracoscopically. There was no conversion to open thoracotomy in any case. The total operative time including reduction and repair ranged from 55– 140 minutes. The weaning from mechanical ventilation ranged from 2nd – 8th post-operative day. The start of oral feeding ranged from 2nd –9th postoperative day. The postoperative hospital stay ranged from 3–30 days. We had minor splenic injury in two cases, diaphragmatic muscle tear in another two cases and lastly one case with minor bleeding and it was easily controlled by diathermy hemostasis.Recurrence occurred in only one case which had recurred after 3 months and redo thoracoscopic repair trial was done but was unsuccessful and converted to open thoracotomy. As regard to mortality; three cases had died, they were all neonatal presenters with mild pulmonary hypertension, one case died on the third day of operation due to heart failure and the other two cases on 20 days & 23 days postoperative due to severe lung hypoplasia, pulmonary hypertension and septicemia