*Dept. of Paediatric Surgery, B J Wadia Hospital, Parel, Mumbai-400 012.

Isolated Meconium Ileus

Sandeep Hambarde*, Pradnya Bendre*

Abstract

Isolated meconium ileus as such is relatively rare. Two neonates presented with

features of intestinal obstruction. On exploration, features of meconium were

present. Temporary ileostomy with postoperative distal washes and medical

management was done, followed by ileostomy closure at 6 months of life. Workup for

cystic fibrosis and associated anomalies was negative. Both are thriving well at one

year followup.

Introduction

econium ileus is the intestinal Mobstructive variant of cystic fibrosis 1(CF) or mucoviscidosis. Approximately

15% to 20% of infants with cystic fibrosis

also present with intestinal obstruction

related to meconium ileus. incidence of

cystic fibrosis is 1 in 15,000 in African-1,2Americans, and much less in Asians. In

the intestine, absence of deficient chloride

transport leads to the migration of water

out of the intestinal lumen with

inspisation of meconium and subsequent 2,3,9formation of an obstructive state.

Case Report

Case 1

Day one neonate presented with gross

abdominal distension, bilious vomitings and not

passed meconium since birth. Child was septic with

leucocytosis, positive C reactive protein and was

dehydrated.

Abdominal skin was tense, shiny and bowel

loops were palpable. (figure1)

Xray revealed gas filled small bowel loops with distal

paucity. (figure2)

Ultrasound showed features of small bowel

obstruction. After stabilisation, neonate was

Fig. 1 : Clinical picture with skin inflammation and

palpable bowel loops

Fig. 2 : X-ray abdomen with gas filled

small bowel loops

explored. There was presence of grossly dilated

jejunum with collapsed ileum and dilated ileocolic

junction. (Fig. 3)

Bombay Hospital Journal, Vol. 54, No. 1, 2012 113

Ileal enterotomy was performed and bowel was

decompressed. (Fig. 4)

Fig. 3 : Grossly dilated proximal jejunum, collapsed

ileum with doubtful vascularity

Fig. 4 : Ileal enterotomy for decompression

Thick and tenacious meconium was expelled out.

(Fig. 5)

Fig. 5: Thick, tenacious meconium at ileostomy site

Divided ileostomy was created. Distal washes

with gastrograffin and oral acetylcystein were started

on postoperative day three. Sweat chloride and

genetic studies for cystic fibrosis were negative. Child

gained weight adequately and ileostomy closure was

performed at six months of life. Child is thriving well

at one year of followup with no illness requiring

hospital admission.

Case 2

Day twenty neonate presented with gross

abdominal distension, bilious vomitings and not

passed meconium since day ten of life. Child was

septic with leucocytosis, positive C reactive protein

and was dehydrated.

Abdomen was grossly distended and bowel loops

were palpable. X-ray revealed gas filled small bowel

loops with distal paucity. Ulrasound showed features

of small bowel obstruction. After stabilisation,

neonate was explored. There was presence of grossly

dilated jejunum with collapsed ileum and dilated

ileocolic junction. Ileal enterotomy was performed

and bowel was decompressed. Thick and tenacious

meconium was expelled out. Divided ileostomy was

created. Distal washes with gastrograffin and oral

acetylcystein were started on postoperative day five.

Sweat chloride and genetic studies for cystic fibrosis

were negative. Child gained weight adequately and

ileostomy closure was performed at five months of

life. Child is thriving well at one year of followup with

no illness requiring hospital admission.

Discussion

Diagnosis of cystic fibrosis should be

pursued in all children who present to

surgeons with the following surgical

disorders: meconium ileus, meconium

plug syndrome, rectal prolapse, jejunal

ileal atresias, nasal polyps, neonatal

jaundice, portal hypertension, failure to 4,5thrive and pancreatitis. Sweat chloride

testing (positive if > 60 meq/L) is quite

accurate, and most patients should

undergo this test. Only 1.4% of CF patients

will have a normal test, but the test

depends on the ability to collect sufficient

quantities of sweat (at least 100 mg

obtained after maximal stimulation by

pilocarpine iontophoresis) which may be 6difficult in neonates. Genetic testing is

Bombay Hospital Journal, Vol. 54, No. 1, 2012114

increasingly utilised. Commercial testings

typically examine DNA for 25 to 100 of the

most common mutations (95% of all CF 6,7patients).

Characteristically in meconium ileus,

the proximal ileum is greatly dilated and

contains thick, sticky meconium, while

the distal ileum and colon are collapsed

and obstructed by thickly packed round

mucus plugs that resemble rabbit pellets.

Intestinal obstruction in infants with

meconium ileus is generally evident within

24 to 48 hours following birth. With

meconium ileus, three cardinal signs of

intestinal obstruction are generally

evident including generalised abdominal

distension, bilious vomiting, and failure to 8pass meconium within 48 hours.

Characteristic radiologic features include

varying-sized loops of distended intestine,

a relative absence of air-fluid levels, and a

"soap bubble" appearance of portions of

the abdomen (Neuhauser's sign), 8,9particularly the right lower quadrant.

Complicated meconium ileus is suggested

on plain film of the abdomen when areas of

calcification are seen or when there is a

large dense mass with a rim of calcification

evident, suggestive of cystic meconium 10peritonitis.

Noblett described a method for

washing out obstructing plugs in patients

with meconium ileus of an uncomplicated 2,5nature. With this method, soluble

contrast material such as meglumine

diatriazoate (Gastrografin) is used.

Regardless of the contrast material used, it

must be water soluble to help loosen the

inspisated pel lets of meconium.

Gastrografin has a strikingly high

osmolarity (2150 mOsm), this may result

in serious fluid derangments in patients,

and typically agents such as Hypaque

(1450 mOsm) or cystoconray II (404

mOsm) are being increasingly used. If

contrast evacuation failed in simple

meconium ileus, the goal of surgery is to

completely evacuate the obstructing plugs

and meconium from the ileum proximal 1,10and distal to the point of obstruction.

One method that may be used is to irrigate

the intestine and colon clear of plugs of

obstructing meconium via an enterotomy

in the dilated segment of ileum. At times

intestinal resection and primary

anastomosis may be needed provided that

all of the plugs can be washed out of the 3distal intestine and colon. An alternative

method in patients in whom the distal

bowel cannot be cleaned out, or whose

condition is precarious, is the formation of

an ostomy, with a secondary closure once

respiratory and nutritional factors are

stabilised. Historically, a Bishop-Koop 2procedure had been advocated. This

consists of a Roux-en-Y anastomosis

between the end of the dilated proximal

segment and the side of the collapsed

distal segment approximately 4 cm from

the distal open end, which is brought out

as an ileostomy.

Compl icated meconium i l eus

indicates that the infant has intestinal

atresia or perforation with varying 7,8degrees of meconium peritonitis.

Postoperatively, careful attention must be

given to fluid and electrolyte as well as

acid-base balance init ial ly, and

management of pulmonary complications,

such as atelectasis and infection. These

patients also require careful nutritional

management, balancing intravenous and

enteral nutrition. Additional pancreatic

115Bombay Hospital Journal, Vol. 54, No. 1, 2012

4,9enzyme supplementation is provided.

In recent years survival has improved

with current operative mortality being

10% to 20%. The long-term outlook is the

same as for all patients with cystic fibrosis

where the average life expectancy is now in

t h e r a n g e o f 3 2 y e a r s . L u n g

transplantation has recently become a

promising option for selected patients with

end-stage pulmonary disease related to

cystic fibrosis. Isolated meconium ileus

without cystic fibrosis has excellent

outcome.

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