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    Cystic fibrosis - 20,8% of the CF population in u.s. - bervariasi 30%-40% PATHOGENESIS to begin in utero and result in an intraluminal accumulation of a highly viscid and tenacious meconium. In simple or uncomplicated mec ileus, perforation and / or necrosis: crescent or speckles of intraabdominal calcification, peritonitis,

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    Cystic fibrosis - 20,8% of the CF population in u.s. - bervariasi 30%-40% PATHOGENESIS to begin in utero and result in an intraluminal accumulation of a highly viscid and tenacious meconium. In simple or uncomplicated mec ileus, perforation and / or necrosis: crescent or speckles of intraabdominal calcification, peritonitis,

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    262 views27 pages

    Ileus Meconium

    Uploaded by

    Debby Tamara
    Cystic fibrosis - 20,8% of the CF population in u.s. - bervariasi 30%-40% PATHOGENESIS to begin in utero and result in an intraluminal accumulation of a highly viscid and tenacious meconium. In simple or uncomplicated mec ileus, perforation and / or necrosis: crescent or speckles of intraabdominal calcification, peritonitis,

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    MECONIUM ILEUS

    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%

    . Etiologi unknown genetic

    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

    Contrast enema (barium, gastrografin, or any water-soluble)microcolon or unused colon

    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

    Gastrografin enema meglumine ditriazone(tween 80),40% sodium diatrizoat

    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

    Simple Meconium Ileus


    In the goal of operation is the relief of intraluminal ileocolonic obstr by either the evacuation of the adherent intraluminal meconium or by resection of the portion of bowel filled with inspissated material

    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

    Criteria for Bishop-koop procedure


    1. Limit intaoperative bowel trauma 2. Resect the disparately enlarged ileal loop filled with inspissated thickened meconium 3. Create an appropriately sized end of proximal to side of the distal ileum anastomosis

    4. Acces for inserting catheter into the distal bowel


    5. Permit an eventual enterostomy closure by bedside ligation of the chimney stoma

    Santulli described a proximal chimney enterostomy, an operation that in essence is the reverse of the resection coupled with a distal chimney enterostomy

    Complicated Mec Ileus


    Almost

    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

    Intussuception Rectal prolapse Colonic stricture

    Pulmonary
    Bacterial

    sepsis Bronchopnemonia

    Inguinoscrotal Disease
    Hernia

    and hydrocele Cryptorchidism

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