Paediatric Surgery CDH ,DH pediatrition 2024..ppt
- 1. Dr. Menawar Dajenah Consultant of General Surgery Assis. Prof. Faculty of Medicin Ibb University
- 2. 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.
- 3. Esophageal atresia 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 3000 live birth • affects males slightly more commonly than females (1.26:1). • Approximately one third of infants are born with low birth weight, • and 60% to 70% have associated anomalies
- 4. Etiology and Pathophysiology Etiology and Pathophysiology • A wedge of mesoderm called the tracheoesophageal septum separates the developing foregut (esophagus) from the trachea. • Esophageal atresia and tracheoesophageal fistula are caused by a defect in mesodermal differentiation • About 50% of cases are associated with other mesodermal defects (VACTERL association) • Vertebral anomaly • Anal atresia • Cardiac anomaly • Tracheoesophageal fistula • Esophageal atresia • Renal anomaly • Limb malformation
- 5. Classification : 1. Isolated esophageal atresia ( 7% ) Type A 2. Esophageal atresia with proximal fistula(2%) Type B 3. Esophageal atresia with distal tracheo-esophageal fistula (86 %) TypeC - The most commonly 4. Esophageal atresia with tracheoesophageal fistula connected t o the proximal and distal esophageal segments (1%) Type D 5. H type ‑ tracheoesophageal fistula without atresia double fistula(4%) Type E
- 7. Associated anomalies (55% of the cases) VACTERL association 1. Cardiac VSD,TOF,ASD,PDA 2. 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 ).
- 8. Prenatal features • Polyhydramnios • The fetus is unable to swallow amniotic fluid. • Occurs in approx. 57% of pregnancies • Associated with an increased risk of premature birth Clinical presentation
- 9. Early presentation The symptom that occurs shortly after birth is • excessive salivation 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. • chemical pneumonitis. • Gastric distension results from inspired air entering fistula is connected to the distal esophageal and causes worsening respiratory distress and pulmonary compromise Clinical presentation Postnatal features
- 10. Clinical presentation • 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. • small H-type tracheoesophageal fistula may occur as late as adulthood.
- 11. Diagnosis 1-prenatal diagnosis Ultrasonography Maternal Polyhydramnios (60-90%) Small or absent stomach bubble Blind – ending proximal esophageal pouch • MRI can confirm the diagnosis
- 12. Diagnosis 2-postnatal diagnosis Placement of a feeding tube the diagnosis 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. X-ray of the thorax/abdomen frequently confirms the diagnosis in which nasogastric tube coiling within the proximal esophageal pouch (except with an H type fistula) ‑ Large gastric bubble: air in the stomach (gross types A and B present with a gasless abdomen) Contrast study (water soluble) show dilated upper esophageal pouch without visulization of lower esophagus & stomach,but this test not frequently performed. Bronchoscopy , to check the exact site of TEF, it is of importance in H- type fistula.
- 16. 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.
- 17. Surgery Surgical treatment should be performed within the first 24 hours of birth. • The goal is to reconnect the upper esophageal pouch and the lower esophagus. • A long gap between both ends of the esophagus may not allow primary repair. • In this case, a gastrostomy tube is necessary to allow enteral feeding. • Treatment options include promoting elongation of the esophagus (via the Foker technique) and colon interposition • Operative management; by using either; Thoracotomy (RT sided/4th I.C.S) Thoracoscopy
- 18. 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.
- 19. Postoperative complications : 1.anastomotic leak, 2.stricture, 3.recurrent fistula formation 4.gastro-oesophageal reflux.
- 21. Congenital Diaphragmatic Hernia A congenital diaphragmatic hernia is a complex anomaly with a significant risk of death, principally due to lung hypoplasia. that affects 1 in 2000 Iive births. The aim of postnatal ventilation strategies is to ameliorate this – whereas prenatal intervention seeks to reverse this in utero. 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.
- 22. • Anatomically, congenital diaphragmatic hernia results from an embryologic fusion defect, along hernition of intrabdominal contents into the chest. • Fusion of the transverse septum and pleuroperitoneal folds normally occurs during the 8th week of embryonic development • If diaphragmatic formation is incomplete the pleuroperitoneal hiatus (foramen of Bochdalek) persists Intestinal nonrotation is common as the bowel herniates into the thorax rather than undergoing its normal sequence of rotation and fixation.
- 23. Epidemiology 1 in 3,000 live-births (1 in 2,000 at ~20 weeks gestation – “hidden mortality”) M=F Predominantly left-sided (80%). Bilateral (<2%) Isolated (usually) if live-born Late-presenters (~10%) – excellent prognosis
- 24. Classification 1. Bockdalek hernia (90%) 2. Morgagni hernia (2%) 3. Hiatal hernia (sliding, rolling) 4. Eventration of diaphragm
- 25. Associated anomalies: 1. Congenital heart diseases 2. ( hypoplastic left ventricle,TOF,TGA,ASD,VSD). 3. Pulmonary hypoplasia. 4. Pulmonary sequestration. 5. Intestinal malrotation. 6. Chromosomal abnormalities.
- 26. • 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. 1.Bochdalek Hernia 1.Bochdalek Hernia
- 29. 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: achypnea and grunting respiration. cyanosis. caphoid abdomen. ncrease chest diameter. n auscultation of the chest: decrease breath sound on the
- 30. Clinical presentations: Smaller defects may not become symptomatic until the infant 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 hernia may be dull to percussion • bowel sounds are not usually appreciated. • The left side of the diaphragm is affected four or five times as frequently as the right . • rate of associated anomalies of 20% (chromosomal abnormalities, neural tube defects, and congenital heart • disease). When the hernia is on the left, the heart sounds may be heard best on the right side of the chest.
- 32. 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 contralateral lung. 2.Barium study : (not routinely performed) presence of bowel loops filled with barium within the chest with shifting of mediastinum to opposite side.
- 35. 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 • 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
- 36. Treatment Operative management: via abdominal approach repair of the diaphragm done either primarily or by using prosthetic mesh for larger defects. Asubcostal 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 . The diaphragmatic defect should be closed by nonabsorbable sutures. In many instances, a synthetic material is required to close large defects..
- 37. Treatment Post operative care and follow up. • Post operative care; 1. Mechanical ventilation (in some cases). 2. Chest tube care 3. Broad spectrum AB. 4. Respiratory support and treatment of hypoxemia. hypercapnia. and acidosis are required before and often after repair
- 38. 2.Morgagni hernia 2.Morgagni hernia Definition: O 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. O it acount for about 2% of all types of diaphragmatic hernias.
- 39. Clinical presentation: O The majority of children with Morgagni (retrosternal) hernias are asymptomatic. O Diagnosis is often not made until adulthood. O Children with this lesion may present with recurrent respiratory infections, coughing, vomiting or epigastric pain/discomfort. O Intestinal obstruction and bowel ischemia/necrosis may result from incarceration of bowel within the hernia sac.
- 40. Diagnosis: 1. CXR ( lateral view )is often demonstrate an air-fluid filled structure located immediately posterior to the sternum which represent the stomach or bowel. 2. Barium study, it is not routinely performed.
- 42. Treatment: O Surgically repaired via a transabdominal approach is indicated in the asymptomatic patient due to the risk of bowel obstruction. The viscera are reduced and any associated hernia sac excised. O 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. O There is no associated pulmonary hypoplasia or hypertension
- 44. 3.Diaphragmatic eventration 3.Diaphragmatic eventration O Definition: it is refer to a paralysis of one dome of diaphragm that result in paradoxical movement of diaphragm during respiration causing respiratory distress.
- 45. 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 muscles 2.Acquired Result from iatrogenic phrenic N. injury during cardiac or mediastinal surgery. Secondary to phrenic n. invasion by mediastinal tumor.
- 46. Clinical presentation: Eventration in the neonate can be asymptomatic but most present with tachypnea, respiratory distress and pallor, difficulty sucking and tire easily with feedings. Chest physical signs include; 1. ipsilateral dullness to percussion. 2. unilateral or bilateral diminished breath sounds. 3. The point of maximal cardiac impulse is shifted away from the side of the lesion.
- 47. Diagnosis: 1. CXR re vealing an elevated one dome of diaphragm with shifting of mediastinum to the other side, (commonly affecting the RT dome ) 2. Fluroscopy is often confirm the diagnosis which demonstrate the paradoxical movement of diaphragm. 3. C.T scan occasionally needed.
- 49. Treatment: Asymptomatic eventration can be followed up & it may not require surgery. The treatment of symptomatic eventration is surgical, The diaphragm on the affected side is placated via either a transthoracic or transabdominal approach. Plication effectively immobilizes the flaccid diaphragm, reducing the paradoxical movement and mediastinal shift that occurs with respiration.
- 53. Pulmonary sequestration • Pulmonary sequestration is a cystic or solid mass composed of nonfunctioning primitive tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. • It is a type of congenital thoracic malformation. • It may present as a lung infection on physical examination and chest imaging.
- 54. Pulmonary sequestration • Its blood supply is from systemic circulation rather than the pulmonary circulation. • Multiple feeding vessels may be present in 15-20% of cases. • The 2 forms of pulmonary sequestration are 1. intrapulmonary, which is surrounded by normal lung tissue 2. extrapulmonary, which has its own pleural investment.
- 55. Pulmonary sequestration • * Extralobar sequestration is asymptomatic and is usually discovered incidentally on chest X-ray. • * If the diagnosis can be confirmed, e.g., by CT scan, resection is not necessary. • * Diagnosis of intralobar sequestration may be made prenatally and confirmed on postnatal CT scan. • * Alternatively, the diagnosis of intralobar sequestration may be established after repeated infections manifested by cough, fever, and consolidation in the posterior basal segment of the left lower lobe.
- 56. Pulmonary sequestration • Increasingly the diagnosis is being made in the early months of life by US, and color Doppler often can be helpful in delineating the systemic arterial supply. • * Removal of the entire left lower lobe is usually necessary since the diagnosis often is made late after multiple infections. • Occasionally segmental resection of the sequestered part of the lung can be performed using an open, or ideally, a thoracoscopic approach.
- 57. Pulmonary sequestration • If an open approach is used, it is important to open the chest through a low intercostal space (sixth or seventh) to gain access to the vascular attachments to the aorta. These attachments may insert into the aorta below the diaphragm; in these cases, division of the vessels as they traverse the thoracic cavity is essential. .
- 58. Pulmonary sequestration • Prognosis is generally excellent. • However, failure to obtain adequate control of these vessels may result in their retraction into the abdomen and result in uncontrollable hemorrhage. • It is also possible to perform a combined thoracoscopic and open approach, wherein the vessels are clipped and divided thoracoscopically and then the lesion safely removed through a limited thoracotomy.