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448 Tirabassi et al.
Complications
The ostomy created for Hirschsprung’s disease is prone to the same prob-
lems as other ostomies in this age group. However, the frequency of com-
plications is less since the ostomy is temporary. In a study of 146 children
with colostomies (mean age 2.1 years, ranging from 1 day to 22 years), 69
patients (47%) had stomal complications. Seventy (48%) of the colostomies
were performed for Hirschsprung’s disease, followed by 46 (32%) for im-
perforate anus. The most common long-term problem involved skin excori-
ation, accounting for 42% of the complications. This was followed by pro-
lapse (25%), stenosis (13%), retraction (7%), colocutaneous fistula (7%),
and peristomal hernia (3%). Major early complications occurred in 16% of
the patients and included postoperative sepsis in 14 cases, evisceration or
herniation of small bowel in 4, and wound infections in 2. The sigmoid
loop colostomy had a significantly lower complication rate when compared
to the transverse colostomy (39 vs. 79% p < 0.01) [60]. In a similar group
of patients, Brenner and Swenson found that reoperation was required in 7
of 48 (15%) colostomies, specifically for evisceration in 3 cases, loop ne-
crosis in 2, and stenosis in 2 [61].
IMPERFORATE ANUS
Background
An imperforate anus is found in 1 of 4000–5000 births and represents a
wide variety of anorectal malformations, from anal stenosis/perineal fistula
(low lesions) to anorectal agenesis (high lesions), with or without a fistula
(rectourethral in males, rectovaginal in females) [8]. In 1710, Littre first
suggested a colostomy in the treatment of imperforate anus [62]. His idea
was not implemented until 1783, when Dubois was the first to attempt this
procedure [63]. Prior to Dubois, the treatment of children with an imperfo-
rate anus was rupture of the obstructing membrane followed by serial dila-
tions until healing was complete [8].
Some families have a predisposition toward anorectal malformations.
There is a male predominance (55–65%), suggesting the possibility of sex-
linked inheritance. Imperforate anus may occur as an isolated malforma-
tion, with other abnormalities, or in association with several syndromes in-
cluding tracheoesophageal fistula/esophageal atresia, Down’s Syndrome,
and Cat’s eye syndrome [64,65]. Commonly associated cardiovascular ab-
normalities (12–22%) are ventricular septal defect and Tetralogy of Fallot.
Duodenal obstruction secondary to annular pancreas, duodenal atresia, or
Ladd’s bands with malrotations may also occur [8]. Urinary tract abnormal-
ities are especially common in babies with high lesions. A thorough evalua-
tion and examination are necessary to determine the presence of urinary
reflux, renal agenesis/dysplasia, and cryptorchism.
Pediatric Intestinal Stomas 449
Management
Imperforate anus covers a spectrum of anorectal malformations. In theory
they may result from the abnormal formation of the urorectal septum or
absence of the dorsal cloacal membrane [8]. There have been numerous
classification systems for imperforate anus. None have gained widespread
acceptance. Patients are typically placed in two groups high (mid/suprale-
vator) lesions requiring colostomy and low (infralevator) lesions not requir-
ing colostomy (Fig. 16). The initial evaluation of a neonate with imperfo-
rate anus focuses on identification of associated anomalies and determining
the necessity of an ostomy. The absence of a midline crease, a “flat bot-
tom,” or the absence of external sphincter contraction are often indicators
of a high lesion. Sacral anomalies are associated with high lesions. Careful
serial examinations of the newborn over the first 24 hr of life can reveal
important clues. The appearance of meconium in the urine and the passage
of flatus from the penis are signs of a high lesion with a colourethral fistula
in males. In females, lack of any externally visible fistula indicates a high
lesion. A small spot of meconium appearing in the perineum indicates a
low lesion with a fistula. Newborns with high defects will need a colos-
tomy. Infants with a perineal fistula or a thin membrane with visible meco-
Figure 16 The level of imperforate anus determines whether a colostomy is
necessary. Most lesions at the level of the levator or above will require diversion.
450 Tirabassi et al.
nium may be treated with dilation or anoplasty and usually do not require
a colostomy.
Ultrasound and plain radiographs help identify the level of the lesion.
At 24–36 hr of age, air on a prone cross-table lateral abdominal film can
provide adequate confirmation of the defect’s level. The distance between
the skin and air within the distal colon is measured. In addition, ultrasound
can be used to measure the distance from the perineum to bowel. In general,
a distance of 1 cm or more by either modality is considered a high lesion
and will require a colostomy. In male newborns, a VCUG or a precolos-
tomy cystoscopy can confirm a colourethral fistula and prove the diagnosis
of a high imperforate anus.
For patients with low defects, primary reconstruction can be done in
the immediate newborn period. Babies are placed in the lithotomy position
at the time of surgery using infant stirrups or some other support device.
The anal sphincter must be clearly identified using electrical stimulation.
In the case of anal stenosis, simple dilation, as first performed by Scultet
in 1660, will usually suffice [66]. For low lesions in the correct position
but without a fistula, a cutback to the anus with placement of skin-to-mu-
cosa sutures provides satisfactory results [67]. In babies with low lesions,
the following technique may be employed. After the anal sphincter is
mapped, a Fogarty catheter is fed into the rectum using a 16-gauge angioca-
theter. Once the balloon is inflated, the Fogarty catheter can be used as
traction to bring down the rectum and a cutback can be performed. Mucosa-
to-skin sutures are then placed circumferentially. (Personal communication
with Richard Courtney, Pediatric Surgical Services, Inc., Springfield, Mas-
sachusetts, USA.)
In newborns with high lesions, a proximal colostomy is created as a
first step. This decision must be made within the first 48 hr of life. The
proximal sigmoid colostomy leaves an adequate length of distal colon for
reconstructive purposes. A loop colostomy may be constructed if a defini-
tive repair is anticipated within the first 8–10 weeks of life. If reconstruc-
tion is not anticipated for several months, an end colostomy with mucous
fistula tends to be a better choice for more long-term diversion [8]. The
approach is an oblique left-lower-quadrant incision through the lateral left
rectus and oblique musculature, avoiding the umbilicus and iliac crest. For
a diverting sigmoid colostomy, the bowel is divided and brought out through
opposite ends of the wound. The functional end is exteriorized laterally and
the distal end medially, leaving sufficient distance between the two stomas
to allow satisfactory appliance placement on the proximal stoma. Both ends
are deliberately exteriorized with a snug fuscial closure in order to prevent
prolapse. The distal loop is irrigated to remove as much meconium as possi-
ble at the time of surgery or shortly thereafter. Urinary tract infection from
fecal contamination can be difficult to control, especially if it is associated
with vesicoureteral reflux. Urine reflux into the colon can produce a hyper-
Pediatric Intestinal Stomas 451
chloremic acidosis. This complication is seen with more proximally placed
ostomies due to prolonged contact of urine with the colonic mucosa.
Reconstruction is then performed when the baby is older, within the
first year of life. The posterior sagittal approach originally described by
deVries and Pena can be used to repair nearly all anorectal malformations
[45,68]. Ostomy closure after reconstruction becomes the third and final
stage.
Complications
Colostomy formation in neonates with anorectal anomalies is associated
with a high incidence of complications (32%). The most frequent mechani-
cal ostomy complication is prolapse, which is observed in 16.3%. These
ostomies are created with dilated obstructed bowel. Once vented, the bowel
shrinks to a normal caliber predisposing to prolapse. Intestinal obstruction
secondary to adhesions, intussusception, and volvulus has been reported in
14.3% of cases. Skin dehiscence has a 6.1% incidence [69].
Following reconstruction, anal stenosis and constipation are by far the
most common complications. A 20-year follow-up study published by
Nixon in 1977 described 14 of 47 cases developing anal stricture [70]. In
the same study, rectal mucosal prolapse was observed almost as frequently,
11 of 47 patients. These children also have a remarkably high rate of uri-
nary tract infections: 31 of 47 patients. Strict attention to technique is ex-
tremely important [71].
FEEDING JEJUNOSTOMY
Background
The need for enteral access exists in many situations in pediatric surgery.
For neurologically impaired children, feeding access is a special concern.
The goal in these children is to provide a route of access that is convenient
for the parent and child, has a low complication rate associated with the
procedure and access maintenance, and provides the patient with a safe and
effective means of nutrition. Nasogastric, nasoduodenal, and nasojejunal
tubes are temporary access devices. The traditional route of long-term en-
teral feedings is via a Stamm gastrostomy. More recently, the percutaneous
endoscopic gastrostomy (PEG) has been widely accepted to fulfill this role.
The Witzel-type jejunal catheter is employed for patients who cannot
tolerate gastric feedings. While the Witzel-type jejunal catheter may be
used long term, it is rarely a stable permanent solution. In children who
cannot tolerate gastric feedings due to primary gastric motility disorders or
prior upper abdominal surgery, a Roux-en-Y feeding jejunostomy is con-
structed for permanent feeding access.
452 Tirabassi et al.
Management
Permanent enteral access via the jejunum using a Roux-en-Y feeding jeju-
nostomy was originally described by Maydl [72]. In Maydl’s procedure,
the Roux limb, which is approximately 25 cm in length, is brought out to
the skin as a permanent stoma. This stoma is then catheterized with a Foley
catheter when feedings are to be started. Feedings usually begin 48 hr after
surgery.
More recently, DeCou et al., Yoshida et al., and Gilchrist et al. have
described modifications to the Maydl and Ballantine Roux-en-Y jejunos-
tomy [73–75]. These modifications involve various methods of securing
a mushroom or balloon catheter at the end of the Roux limb [73–75].
The catheter is brought through the abdominal wall, with the jejunum se-
cured to the anterior abdominal wall (Figs. 17 and 18) Thus, a Roux-
en-Y jejunostomy is created, but instead of a stoma flush with the skin, a
catheter is present. This catheter can be replaced at a later time with a
button catheter to provide a more convenient and more socially acceptable
appearance.
Complications
Yoshida’s series compared the original stoma technique with the modified
catheter jejunostomy [74]. The nine patients with the permanent stoma jeju-
nostomy had the expected complication of stoma leakage. One child had
such significant skin breakdown as to require conversion to a tube jejunos-
tomy. A second child also had conversion to a tube jejunostomy for stomal
prolapse. None of the 22 patients with the modified tube Maydl Roux-
en-Y jejunostomies had any significant complications. Several children had
their tubes changed to button-type tubes without complication.
BUTTON JEJUNOSTOMY
In 1989, Stellato and Gauderer described a technique to access the jejunum
with a button device to create a permanent and continent feeding jejunos-
tomy [76]. For this less complicated procedure, the button is placed through
the antimesenteric border of the jejunum approximately 10–60 cm from the
ligament of Treitz, depending on patient size and age. A button is used
instead of a red rubber catheter because it is more stable and secure [77].
The jejunum is sutured to the undersurface of the abdominal wall and the
button exits to rest on the skin surface, where it can be used when the
postoperative ileus resolves (Fig. 19) Once the wound has healed com-
pletely, changes to the button catheter can be made in the home or office
setting.
Pediatric Intestinal Stomas 453
Figure 17 Ballantine tube jejunostomy. This demonstrates a Roux-en-Y feeding
jejunostomy combined with gastric decompression.
OTHER INDICATIONS
Gastroschisis is characterized by a defect in the abdominal wall lateral to
the umbilicus, usually measuring 2–5 cm in size. Herniation of the abdomi-
nal contents occurs, most commonly involving the large and small intes-
tines and less frequently the stomach and liver. The incidence varies from
454 Tirabassi et al.
Figure 18 The modified Maydl operation. In this procedure the feeding tube is
advanced beyond the Roux-en-Y anastomosis, allowing earlier postoperative
feeding.
Figure 19 Button jejunostomy. Both the balloon and sutures hold a loop of
jejunum firmly against the abdominal wall, leaving little chance for leakage.
Pediatric Intestinal Stomas 455
1 in 1500–1 in 20,000 live births in the United States, with a male-to-
female ratio of about 1:1 [8]. Management consists of protection of the
eviscerated intestines within a sterile bag, thermal maintenance, fluid and
electrolyte replacement, and then surgical repair. In the majority of cases,
complete reduction and closure is accomplished in one operation.
Intestinal atresia complicating gastroschisis has been reported to occur
in 5–23% of cases in recent series [78–80]. This has been attributed to
vascular compromise secondary to compression at the opening in the ab-
dominal wall. The management of this complication has been quite vari-
able, including resection of the affected segment with primary anastomosis
or creation of a proximally diverting ostomy followed by definitive repair
at a later time [78]. The primary anastomosis in gastroschisis is often pro-
hibited by the thick, rubbery nature of the intestinal wall, which is not
encountered in simple cases of intestinal atresia. One group has described
success with replacing the atretic segment into the abdomen, performing
primary abdominal closure, and re-exploring in 1–3 weeks to establish
bowel continuity through resection and primary anastomosis. The intestines
appeared healthier and more robust after this period of recovery, allowing
for a safer repair [80]. However, a proximal decompressive ostomy is rec-
ommended in cases of severe bowel dilatation or perforation.
The original abdominal wall defect is a convenient site for an end
ostomy in uncomplicated cases of atresia with gastroschisis [29]. After con-
trast study of the entire distal bowel, closure can be carried out in a few
weeks.
Cystic fibrosis can present with lower bowel obstruction secondary to
meconium ileus. Historically, enterostomies were created for the purpose
of decompression and antegrade irrigation. The two most common proce-
dures were either the Bishop-Koop or Santulli enterostomy (Fig. 20) Alter-
natively, a T tube was placed in the cecum for irrigations. Today medical
management (enemas and/or irrigation with diatrizoate or N-acetylcysteine)
minimizes surgical interventions. When surgery is needed, the bowel is
evaluated, opened, irrigated, and then closed without an ostomy. In the case
of complicated meconium ileus, obstruction progresses in utero to perfora-
tion, peritonitis, calcification, and pseudocyst formation, resection of the
devitalized bowel and pseudocyst with intestinal diversion may be the nec-
essary and safest approach.
Biliary atresia, a cause of neonatal jaundice, requires surgical inter-
vention. The standard procedure is a Roux-en-Y portoenterostomy (Kasai
procedure). Variations of the Kasai have included exteriorization of the bile
conduit to decrease the risk of ascending cholangitis, quantify bile output,
and facilitate endoscopic instrumentation of the portoenterostomy site if
bile flow stopped [81,82]. However, exteriorization of the bile conduit is
rarely necessary today.
456 Tirabassi et al.
Figure 20 Procedures described by Bishop-Koop (Panel A) and Santulli (Panel
B) once commonly performed for the decompression or distal irrigation of infants
with Meconium Ileus are now rarely performed.
STOMA CARE
Children with stomas have problems with their self-image and self-esteem
[83]. Positive feeling and reactions conveyed by the parent on a regular
basis help to combat this.
The stoma and appliance cover a greater proportion of the infant’s
abdomen compared to that of an adult (Fig. 21) This can impair a good seal
with the appliance if a properly sized device is not selected. Involvement of
an enteral stomal therapist early in the patient’s care is important. A variety
of appliances are suitable for children and infants. Premature neonates can
be maintained with the two-piece system available from Incutech Inc.,
called Preemie Pouch (Fig. 22) With this system, a skin-protective layer is
applied first. The pouch is then affixed to this layer with the aid of stomahe-
sive. Multiple vendors that provide pediatric appliance lines from the major
trademarks, such as ConvaTec and Hollister can be identified on the In-
ternet. The systems available are one-piece, two-piece, or two-piece with a
flange.
Infants and toddlers will tend to play with the appliance, causing leaks
and appliance failure. A one-piece jumper is helpful in keeping the child’s
hand away from the appliance. However, children should not be discour-
aged from taking part in the care of their stoma. A toddler may be able to
help collect the appliance supplies, while a preschooler can learn to help
empty the pouch. By the age of 10–12 years, most children can take over
Pediatric Intestinal Stomas 457
Figure 21 Neonate appliance. Ostomy appliance in place on this 800 gram pre-
mature neonate about 5 weeks post-op from multiple small bowel resections for
perforated Necrotizing enterocolitis. The attachment of the appliance easily occu-
pies
1
/
4
of the abdominal surface area. Two ostomies are within the bag. Note the
spontaneous maturation of all ostomies.
most of their own stoma care. Children should be encouraged to play and
take part in normal activities. Bathing can be performed with the pouch on
or off, based on personal preference. Water will not harm the stoma, but
bubble bath may be irritating. A stoma usually does not dictate a special
diet. However, the family may find that some foods are more likely to
cause diarrhea and gas. Some trial and error will be necessary to determine
the response and function of childhood stomas.
In newborns and infants, it can be a challenge to produce a good
appliance seal. With distal colostomies producing formed stool, some par-
ents may elect not to use an appliance and instead to use topical protective
skin ointments and diapers. With a proximal colostomy or ileostomy, a
properly fitting appliance is important to protect the peristomal skin. Moni-
liasis and skin excoriation are significant problems. Topical antifungal
agents should be employed at the first sign of skin irritation [8].