11 Congenital Pouch Colon: Devendra K. Gupta and Shilpa Sharma
11 Congenital Pouch Colon: Devendra K. Gupta and Shilpa Sharma
11 Congenital Pouch Colon: Devendra K. Gupta and Shilpa Sharma
large series have used two terms, congenital short co- Table11.1 Cases of congenital pouch colon (CPC) treated be-
lon or CPC to describe this condition [11,12]. tween 1993 and 2005 at the All India Institute of Medical Sci-
The anatomy of this malformation was first de- ences, New Delhi
scribed in 1977 by Singh et al. [13] and subsequently Gender Type Number of cases
in detail by Wakhlu et al. [14] and Chadha et al. [11]. Male Colovesical 133
It was in 1984 that Narsimha Rao et al. proposed an
Female Colocloacal 13
anatomical classification of this condition that has
been widely accepted [9]. An important advance- Colovaginal 6
ment in the management of this condition was the Total 152
technique of coloplasty, which was developed in 1976
by Chiba et al. [15]. Subsequently this has been used
with good results [11,16,17].
11.4 Etiology and Embryogenesis
have been seen from Sweden, Japan, and Italy, and Table11.3 Types of CPC. Initially, in India, cases of types I and
were reported merely as curiosities (personal com- II were more commonly seen and accounted for more than 70%
munications). of cases until 1985. Interestingly, during the past two decades, it
As the blood supply is always abnormal to the is types III and IV CPC that have become more common
pouch in these patients, an early vascular insult can- Type I Normal colon is absent and the ileum opens
not be ruled out. It is only the superior mesenteric directly into the colonic pouch.
artery that is prominent and supplies the whole dis- Type II The ileum opens into a short segment of
tal bowel. The inferior mesenteric artery is present in cecum, which then opens into the pouch.
only 50% of cases of distal CPC, and it is also quite
Type III Presence of a significant length of normal colon
insignificant. A genetic predisposition also needs to
between the ileum and the colonic pouch.
be ruled out.
Type IV Presence of near-normal colon with only the
The north Indian belt is also known as the stone
terminal portion of colon (sigmoid and
belt (due to the deficient nutritional factors in the
rectum) converted into a pouch.
diet) and also for the iodine deficiency in the water
there. The land is very fertile and the pesticides are
used liberally in the fields. The population is mainly
vegetarian and consume lot of fresh vegetables in the
diet. In addition, most of the population with ARM
in this region have a low socioeconomic status. All of
these factors suggest environmental factors affecting
or precipitating the anomaly at a window time after
conception when the hindgut is developing and dif-
ferentiating into urinary and intestinal tracts.
11.5 Classification
1. Incomplete CPC: where the length of the normal mesenteric artery on the left side [21]. The inferior
colon is adequate for performing the pull-through, mesenteric artery is present only in incomplete CPC
without the need for doing a coloplasty. The pro- and supplies the lower half of left lateral side of pouch.
cedure would involve excision of the pouch with The pouch lacks haustrations, taeniae, and appendi-
an end colostomy at birth and a definitive pull- ces epiploicae. At times the inferior mesenteric artery
through later. A single-stage pull-through in the may be completely absent.
newborn stage can also be undertaken if the con- The distal communication of the pouch is in most
dition of the baby permits. instances with genitourinary system. In males, the
2. Complete CPC: where there is either absent or communication is most commonly present with the
insufficient normal colon left to permit a pull- bladder and the fistula opens on the posterior wall
through procedure. In this situation, a coloplasty of bladder near the base. Occasionally the fistula
procedure would be required to retain only a may open higher or lower down (Fig.11.6). The
15-cm length of CPC in the form of a tube, to be fistula is usually quite broad and thick-walled, and
brought out as an end colostomy. A pull-through measures up to 1cm in external diameter. In females,
procedure at the time of performing coloplasty colocloacal fistula is the most commonly occurring
should not be preferred in the newborn stage as it fistula, followed by colovaginal and colovestibular
is associated with high morbidity and mortality. fistulae.
In cases of incomplete CPC, the cecum is situated
According to the authors, the CPC should have the in the epigastrium or the left hypochondrium and a
following anatomical criteria: variable length of normal colon is found, which ends
1. There is anorectal agenesis. in a large sac that communicates with the bladder in
2. The total length of the colon is short (Fig.11.2). males and the vagina or vestibule in females. The ce-
3. The colon has a pouch with varying length; saccu- cum, appendix, and the normal part of the colon are
lar or diverticular with a collection of meconium vascularized by the superior mesenteric artery. The
or fecal matter (Fig.11.3). inferior mesenteric artery, if present, supplies part of
4. The blood supply to the pouch is abnormal the colonic pouch.
(Fig.11.4). The pelvic musculature is variable in cases of CPC,
5. The colon wall is thick and muscular with hyper- and when associated with a complete pouch or low
trophied mucosa (Fig.11.5). vertebral anomalies, the pelvic and perineal muscles
6. The fistula with the genitourinary tract is large, are poorly developed.
muscular, and long. It is closely adherent with the
bladder wall.
7. There is no transitional zone between the pouch 11.6 Histopathological Examination
and the normal bowel. The pattern changes sud-
denly and sharply. The pouch wall consists of a normal number of gan-
glion cells, although a few authors have found reduced
Associated genitourinary malformations (cloacal and very small ganglion cells [7,9,13,22,23]. Nerve
anomalies, double vagina, exstrophy) are common in bundle hypertrophy has also been reported, but is not
girls; however, other (nongenitourinary) associated the regular feature [22]. Congestion of the mucosa
congenital anomalies are less common. and focal hemorrhages are seen commonly [22,23]. In
The anatomical features vary according to the a detailed review of these cases, the authors found the
length of the colon that exhibits pouching. In com- following histological features in patients with CPC:
plete pouching of the colon, there is a large, dilated, 1. In most cases, the muscle coat did not have the
thick-walled pouch occupying most of the left side of normal differentiation of the inner circular and
abdomen. The cecum, if present almost always opens the outer longitudinal muscles. The muscles were
into the sac from the right side. It may be associated also arranged in a decussating pattern. The circu-
with an absent, rudimentary, or double appendix. The lar muscle was incomplete in 50% of cases. The
ileum opens into the cecum or the pouch from right wall of the blood vessels was normal (Fig.11.7 A).
side and there is associated malrotation. The pouch 2. The ganglion cells were mature and present in all
has a poorly developed mesentery and is supplied by cases, with the presence of normal or occasionally
the superior mesenteric artery on the superior and hypertrophic nerve bundles. However, giant gan-
right side and an arcuate extension of the superior glia were seen in 10% of cases (Fig.11.7 B).
11 Congenital Pouch Colon 215
Fig. 11.3 Incomplete CPC with dilated lower end of colon and Fig. 11.4 CPC showing abnormal blood vessels
anorectal malformation. The descending colon is normal and
can easily be used for performing a pull-through procedure
Fig. 11.5 A, B Excised thick-walled CPC with window colos- Fig. 11.6 Diagrammatic representation of colovesical fistula
tomy sites in the bladder in congenital CPC
216 Devendra K. Gupta and Shilpa Sharma
An plain erect x-ray of the abdomen in anteroposte- A large number of associated anomalies are found
rior and lateral views in addition to an invertogram with CPC. The genitourinary system is most com-
forms the mainstay of diagnosis. A large loop of monly involved, followed by the gastrointestinal sys-
bowel with single air fluid level occupying more than tem and others. Table11.4 summarizes the associated
half of the total width of abdomen and displacing the anomalies found with congenital CPC [2127].
small bowel to one side (usually right) is the classi-
cal picture (Fig.11.8). The pouch is proximal to the
pubococcygeal line in the invertogram. The majority 11.10 Management
of the patients can be diagnosed by an erect x-ray in
addition to the conventional invertogram that is usu- Preoperative resuscitation is essential with a wide
ally performed for ARM investigations. bore nasogastric tube to decompress the abdominal
The diagnosis may be missed when there is an in- distention, correction of dehydration and electrolyte
complete pouch. A false diagnosis can be made when imbalance, maintenance of body temperature, anti-
there is significant dilatation of sigmoid colon or lo- biotic coverage, vitamin K injection, and a urethral
calized pneumoperitoneum following perforation in catheter to measure urine output as well as to decom-
patients with ARM presenting late, or in female ba- press the bladder.
bies with rectouterine fistula where the massive dila-
tation of uterus with meconium and gas may mimic
CPC [14]. 11.10.1 Aims of Surgical Management
In cases of perforation of the pouch, the pneu-
moperitoneum may mask the diagnosis of CPC. An The aim of surgery is to utilize the available length
early perforation in cases of high ARM is suggestive of colon for absorption and storage capacity as well
of pouch colon, especially if the baby comes from an as for propelling fecal matter onward with a conti-
area where CPC is commonly seen. nent anal opening. In incomplete CPC, an adequate
A detailed work up of the baby at the time of de- length of normal colon is present so the pouch can
finitive surgery should include ultrasound of the be excised and colonic function is preserved. In com-
abdomen, intravenous urogram, and voiding cysto- plete pouching, these objectives can be achieved only
urethrography and echocardiography to evaluate for by tubularizing the pouch in the form of coloplasty
associated anomalies. Spiral computed tomography (Fig.11.9). However, more complications are antici-
with three-dimensional reconstruction of the pelvic pated with the preservation and use of CPC as a tube.
218 Devendra K. Gupta and Shilpa Sharma
At present, single-stage surgery for CPC is not advo- Fig.11.10 Algorithm for the management of congenital CPC.
cated as there is an unacceptably high mortality asso- APPT Abdominoperineal pull-through, PSARP posterior sagit-
ciated with it. Although there are certain advantages tal anorectoplasty. *Preferred approach
of single-stage surgery, they are not sufficient to war-
rant a major surgical undertaking in a neonate with
associated anomalies and complications. Excision of the pouch with an end colostomy is
the procedure of choice. Window colostomy is sim-
ple surgery, can be performed with minimum anes-
11.10.3 Staged Surgery thesia time in a sick neonate, and provides adequate
decompression and a time period to allow for weight
There are two or more steps in the staged procedure gain and fitness for the second stage. However, it has
depending upon the choice of procedure, which in certain disadvantages, as will be described later. The
turn depends upon the condition of the baby at pre- mortality following window colostomy is reported to
sentation, the technical skill of the surgeon, and the be in the range of 1520% [21]. However, the mor-
availability of facilities for major neonatal surgery tality following coloplasty with end colostomy in the
and postoperative care (Fig.11.10). Proximal diver- newborn period is higher, hence this is better done at
sion may take place in the form of: a later date.
1. End colostomy after division of the fistula and ex-
cision of the pouch in incomplete CPC (preferred
approach). 11.10.4 Definitive Procedure
2. End colostomy after division of the fistula and
coloplasty in complete CPC. In incomplete CPC with a colostomy already per-
3. Window colostomy, in which an opening is made formed, the operative steps include dismantling the
on the anterior surface of the colonic pouch with- colostomy and an abdomino-posterior sagittal ano-
out attempting to ligate the fistulous connection. rectoplasty (PSARP) for creation of a new anus. A
4. Proximal ileostomy in complete CPC proximal diversion with an ileostomy may be consid-
5. Transverse colostomy in incomplete CPC ered in selected cases to protect the neoanus.
11 Congenital Pouch Colon 219
In complete CPC without the previous coloplasty, pouch completely by division of the inferior mesen-
the operation involves ligation of fistula, coloplasty, teric artery (if present) and incising the pouch on the
and abdomino-PSARP. A proximal ileostomy is pre- antimesenteric border, thus preserving the vascular-
ferred and would need to be closed in next stage. ity. The tube is fashioned over a red rubber catheter to
Appendectomy should be performed at the time of obtain a uniform diameter (Fig.11.9). Variable results
pull-through to prevent misdiagnosis in the event of have been reported by different authors [12,14,15,28
appendicitis occurring at a later date. 30]. The authors institutional experience is presented
The coloplasty is performed after mobilizing the in Table11.5.
Complications related to colostomy include anemia, The prognosis depends upon the weight of the child,
excoriation of skin, diarrhea, poor weight gain, pro- age at presentation, presence of sepsis and perfora-
lapse, and stenosis. tion, associated congenital anomalies, and most im-
portantly on the length of colon that has pouching.
The prognosis is better in cases of incomplete CPC as
11.11.3 Coloplasty cases of complete CPC suffer from recurrent watery
diarrhea due to the short length of the large bowel.
Complications related to coloplasty include suture Window colostomy performed in the pouch also does
line leak (has become negligible since the introduc- not allow complete evacuation of the contents and is
tion of the proximal ileostomy) and wound dehis- frequently associated with massive prolapse, bleeding,
cence (minor wound dehiscence occurs in 45% of and recurrent urinary tract infection.
patients, but full-thickness major dehiscence is un-
common and is usually associated with leak from the
coloplasty). Mortality following coloplasty has been 11.14 Overall Results
reduced to less than 5% since being performed as a
staged procedure. In the authors experience, as the anatomy and the
histology of the CPC is abnormal, even the tube made
from the dilated pouch does not work well; it does not
11.11.4 Pull-Through contribute effectively to colonic motility. Rather, the
postoperative complications like mucosal prolapse,
Complications related to pull-through are mucosal incontinence, mucus discharge, skin excoriation, and
prolapse (easily managed by excision), anal stenosis colonic ectasia are more common than in those with
due to noncompliance with dilatation, and colonic ARM. Window colostomy is associated with serious
dilatation. The latter sometimes occurs following complications and is thus not favored. Wherever fea-
coloplasty in long-term follow-up [31]. This may be sible, an excision of the pouch in toto with an end co-
due to the fact that the colonic pouch is abnormal his- lostomy (using normal colon) is the preferred proce-
tologically and has a tendency to dilate. The utiliza- dure. An attempt should be made to excise the pouch
tion of a shorter segment of the pouch for tubulariza- even in cases with colonic perforation.
tion is recommended. The overall mortality of CPC was previously as
high as 3040%, but has now come down to 1020%
as a result of the growing awareness of this condition
11.11.5 Short Colon Length and improvements in surgical management and neo-
natal care. Prognosis depends on the aforementioned
Complications related to short length of colon are re- factors, with the most important factor being the ex-
current, watery diarrhea and poor weight gain. tent of the malformation. Excision of the pouch and
11 Congenital Pouch Colon 221
end enterostomy has been associated with maximal 16. Wakhlu AK, Pandey A, Wakhlu A, et al (1996) Coloplasty
survival (92.3%) in good-risk patients [32]. Babies for congenital short colon. J Pediatr Surg 31:344348
with incomplete CPC fair well with normal conti- 17. Chadha R, Bagga, D, Malhotra CJ, et al (1994) The embry-
nence, physical, motor, and behavioral development. ology and management of congenital CPC associated with
Cases of complete CPC suffer from increased fre- anorectal agenesis. J Pediatr Surg 29:439446
quency of stools for the initial 36months, although 18. Dickenson SJ (1987) Agenesis of the descending colon
the frequency decreases with the growth of the child with imperforate anus. Correlation with modern concepts
and dietary modifications. of the origin of intestinal atresia. Am J Surg 113:279281
19. Wakhlu AK, Wakhlu A, Pandey A, et al (1996) Congenital
short colon. World J Surg 20:107114
20. Holikson MA, Schapiro MB, Garfinkal DJ, Shermeta DW
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