Congenital atresia of esophagus :
Incidence :Is a relatively common congenital Mal formation occurring in about one in ( 2500 – 3000 ) life births and usually associated with a tracheoesophageal fistula .
• Etiology :
• Remains unknown but the suggested factors are genetic, drugs, infection.• The majority of cases are sporadic non – syndromic . group are associated with chromosomal abnormality .
• Familial / syndromic cases are extremely rare and are less than 1% of total.
• Associated mal formation are present in about 50% of cases , the most frequent are musculo – skeletal , Cvs , G.1T. , genito – urinary.
• It is important to be aware of this abnormality , because its recognition within forty – eight hours of birth and subsequent surgical correction , is the only hope of survival .
• Classification :
• A . Lower segment opens in to the trachea this is the commonest type 85 % of cases.• E T
• B . upper segment opens in to the trachea represent 2 %.
• B . upper segment opens in to the trachea represent 2 %.• C . Both segments opens in to the trachea this represent 3-5 % of cases .
E
T
• D . Both segments end blindly and the mid oesophagus is
• absent. it represent 3-5 % of cases .• E . trachea – esophageal fistula without atresia . The so called H – type
• or N – type, represent 4 % .• Clinical Feature:-
• The new born baby regurgitates all its first and subsequent feeds . saliva pours, almost continuously from its mouth . this is the sign of oesophageal atresia, it dose not occur in any other condition, attacks of coughing, chocking and cyanosis occur on feeding, sign & symptoms of aspiration pneumonitis may be present. it should be suspected in all cases of hydraminos a condition which is present in 50 % of cases of oesophageal atresia.• The diagnosis may be suspected prenatally by the Finding of small or absent fetal stomach bubble on u/s study performed after the (18th) week of gestation.
• A No. ( 10 – 12 ) soft radioopaqe catheter is introduced in to the esophagus through the mouth , should an obstruction encountened about 10 cm. from the lip the diagnosis is practically certain.
• Contrast study is rarely needed .
• The diagnosis of H- type fistula is often delayed & may present with history of recurrent pneumonia.
• Surgical inter vention is urgent .
• Aspiration pneumonia is nearly always present and antibiotic should be given, dehydration should be corrected, suction catheter is placed in the oesophagheal pouch & patient put in incubator I.V Line and nothing by mouth .pre – operative treatment :
• Operative Treatment :-
• 1 . primary repair : Birth weight, presence of pneumonia & associated anomelies play major role in determining survival .
• 2 .initial gastrostomy & delaying primary repair specially for those tiny premature babies or those with pneumonia .
• 1 . Dysphagia.
• 2 . gastoesophageal reflux.• 3 . Stricture.
• 4 . Major anastomotic leak or disruption.
• 5 . Tracheamalacia .
Post operative complications :-
• Congenital Diaphragmatic Hernia :-
• Congenital hernia occur at centain well – recognised point due to failure of fusion of the varions elements which make up the diaphragm which includes the septum transversum , pleuro peritoneal membrane, osephageal mesentry, body wall mesoderm, through these points Herniation of an abdominal viscus through the diaphragm in to chest .• Incidence :-
• I in every (2000 to 5000 ) life births. females infant are affected twice as often as males .• Associated anomalies: cardiac , pulmonary hypoplasia, pulmonary vascular abnormalities and chromosomal abnormalities .
• types
• 1 . postero- lateral diaphragmatic hernia commonly called bochdalek hernia occur in 85 % to 90 % of hernia that seen in neo natal period .• 2 . Retrosternel hernia ( morgagni ) account for 2-6 % usually occur to the right ) .
• 3 . Hiatus hernia
• Clinical Presentation :-
• Respiratory distress may be developed immediately after birth, tachypnea, grunting, chest retraction, pallor & cyanosis, scaphoid abdomen, bowel sounds may be present within the chest. intestinal obstruction or bowel ischemia, volulus might be the presenting features.
• Diagnosis
• - CXR : presence of air and fluid - filled loops of bowel within the chest, mediastinal shift.• -Prenatally by U/S.
• Treatment :-
• - Resuscitation 02 , NG tube.• - IV Fluid, incubator.
• Respiratory stabilization and care by ECMO
• ( extra corporal membrane oxygenation ) may be needed preoperatively and postoperatively .
• - Surgical repair.