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Described in1905 Landsteiner, observation of meconium obstructing the small bowel with pathologic changes in the pancreas that he attributed to a putative enzyme def. cystic fibrosis 20,8% of the CF population in USA bervariasi 30%-40%
PATHOGENESIS
To begin in utero and result in an intraluminal accumulation of a highly viscid and tenacious meconium: the developments of pancreatic exocrine enzyme def and the secretion of hyperviscous mucus by pathologically abn intestinal glandsmeconium accumulation to obstruct the intestine intraluminally complication
CLINICAL FEATURES
Uncomplicated meconium ileus, immediately at birth with the recognition of abd distention, a unique feature of inspissated meconium filling and obstructing the distal small bowel, bilious vomitting and failure to stool.
Complicated mec ileus, in utero or postnatally intestinal perforation and/or necrosis: crescent or speckles of intraabdominal calcification, peritonitis, erythematous or edematous abd wall and/or demostrable abd tenderness
Simple mec ileus Older child or young adult mec ileus equivalent or distal ileal obstruction syndr
A family history of CF is present in 10%33%,amniocentesis with restriction fragment length polymorphism analysisaccurate diagnosis of the fetus afflicted with CF. Coupling this information + serial in utero sonografi intestinal obstr of mec ileus(about 20% of the CF population) with or without a complicating meconium cyst.
Maternal polyhidramnios may be a feature of in utero mec ileus, a finding putatively resulting from the high-grade intestinal obstr.
In simple or uncomplicated m.i : peritonitis (-)
P.E In fact, m.i is only variaty of neonatal intestinal obstr that produces abd distension at birth before the neonate swallows air. Visible peristaltic wave and palpable, doughy bowel loops are often present. Finger pressure over a firm loop of bowel may hold the indentation, the so-called PUTTY SIGN RT: unremarkable, but characteristically on withdrawal of the examining finger a spontaneous expulsion of meconium does not follow.
Mec peritonitis and cyst formation a palpable abd mass,discoloration of the abd wall, and sign peritonitis Hypovolemia NGT bile-stained gastric fluid usually exceeds 20 ml
RADIOLOGIC
Echogenic bowel wall in the third trimester Plain abdomen, supine and erect films:
1. Great disparity in the size of the intestinal loops because of the configuration of different segments of the bowel 2. No or few air-fluid levels on the erect film because swallowed air cannot layer above the thickened inspissated meconium 3. A granuler, soap bubble or ground-glass appereance seen frequenly in the right half of the abd, a finding that requires swallowed air bubbles to intermix within the sticky mec
Laboratory testing
Stool trypsin and chymotrypsin analysis has historically been a popular screening test for mec ileus Trypsin level less than 80 mg/g of stool CF immunoreactive trypsinogen in blood
DIFF DIAG
Neonatal intestinal obstruction: Ileal atresia Hirschsprungs disease Neonatal small left colon Meconium plug syndrome
MANAGEMENT
Nonoperative
Depends on the dissolution of the inspissated intraluminal meconium in an otherwise patent and uncompromised ileocolon. Noblett described : 1. contrast enema distal intestinal obst
2. comp of volvulus,atresia,perforation,peritonitis excluded 3. enema with careful fluroscopic control 4. Intravenous antibiotic 5. Pediatric surgeon 6. full fluid resuscitation 7. Should be prepared for imminent operation
The technical of solubilizing enema treatment guidelines of Noblett fluoroskopic guidance and an initial solution of 50% gastrografin in waterabdominal radiograph should be repeated in 8-12 hoursthe obstr has been relieved
The success of nonoperative treatment is variable
Operative management
1. Enterotomies with irrigation coupled with a limited resection 2. The Miculicz resection and enterestomy 3. The Bishop-koop resection and enterostomy
4. The Santulli enterostomy 5. Tube enterestomy
Santulli described a proximal chimney enterostomy, an operation that in essence is the reverse of the resection coupled with a distal chimney enterostomy
always requires an operation In contrast, operative indications include persisting intestinal obst, an enlarging abdominal mass, and sign of peritonitis, which may include abdominal wall edema and discoloration, tenderness on physical examination, and clinical and lab sign of ongoing sepsis
Meconium
peritonitis meconium accumulating in peritonial cavity A calcified pseudocyst fibrous wall and spared bowel loop operation mandatory asses residual intestinal length
At
Postoperative management
Support of the infants general physiologi
Complication
Gasrointestinal
Pulmonary
Bacterial
sepsis Bronchopnemonia
Inguinoscrotal Disease
Hernia