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 glands→meconium 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 ileusOlder 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 analysis→accurate 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

Hirschsprung”s 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 water→abdominal radiograph should be repeated in 8-12 hours→the 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 operationIn 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

At operation → mandatory asses residual intestinal length

Postoperative management

→Support of the infant´s general physiologi

Complication

Gasrointestinal Intussuception Rectal prolapse Colonic stricture

PulmonaryBacterial sepsisBronchopnemonia

Inguinoscrotal DiseaseHernia and hydroceleCryptorchidism