Background: Congenital pouch colon (CPC) is a rare entity in patients of anorectal malformations (ARM) requiring special consideration as to the management. This study is aimed at evaluating the presentation, management, and the outcome of initial surgery in patients with CPC. Materials & Methods: This retrospective study was conducted in the department of Pediatric Surgery of our institute during May 2007- May 2010. The Information about the demography, clinical features, investigations, management, and the outcome of initial surgery was retrieved and analyzed. Results: There were 21 patients of CPC managed during the study period. Sixteen (76%) were males and five (24%) females (M:F 3.2:1). Mean age of presentation was 4.8 days with a range of 12 hours to 45 days. In 18 (85.7%) patients, CPC was found with high ARMs, whereas, in 3 (14.3%) patients it was associated with low ARMs. Imperforate anus with moderate to massive abdominal distension was the presentation in 16 (76%) patients. Abdominal radiographs helped in preoperative diagnosis in 8 patients. Two patients had pneumoperitoneum on abdominal radiographs. At operation, type I CPC was found in 9 (42.8%) patients, type II in 5 (23.8%), type III in 2 (9.5%) patients, and type IV CPC in 5 (23.8%) patients. In 11 (52.4%) patients, pouch was emptied and retained with proximal enterostomy. In 7 (33.3%) patients, end enterostomy with pouch excision was done. In two patients, a window colostomy was formed. In one patient, pouch was disconnected from the normal bowel and Hartmann’s pouch with end ileostomy was formed. There were 2 (9.5%) deaths in our series.Conclusion: CPC is a rare malformation. Massive abdominal distension with imperforate anus is the common presentation. Optimum management can reduce the morbidity and mortality

Afzal Sheikh

Bilal Mirza

Sarfraz Ahmad

Journal of Neonatal Surgery

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  J Neonat Surg 2012;1(3):37   EL-MED-Pub Publishers.   http://www.elmedpub.com * Corresponding Author  O R I G I N A L  A R T I C L E  Congenital Pouch Colon: A Preliminary Report from Pakistan  Bilal Mirza,* Sarfraz Ahmad, Afzal Sheikh  Department of Pediatric Surgery, The Children’s Hospital and the Institute of Child Health Lahore, Pakistan.          ABSTRACT  Background: Congenital pouch colon (CPC) is a rare entity in patients of anorectal malfor-mations (ARM) requiring special consideration as to the management. This study is aimed at evaluating the presentation, management, and the outcome of initial surgery in patients with CPC.  Materials & Methods: This retrospective study was conducted in the department of Pediatric Surgery of our institute during May 2007- May 2010. The Information about the demogra-phy, clinical features, investigations, management, and the outcome of initial surgery was retrieved and analyzed.  Results: There were 21 patients of CPC managed during the study period. Sixteen (76%) were males and five (24%) females (M:F 3.2:1). Mean age of presentation was 4.8 days with a range of 12 hours to 45 days. In 18 (85.7%) patients, CPC was found with high ARMs, whereas, in 3 (14.3%) patients it was associated with low ARMs. Imperforate anus with moderate to massive abdominal distension was the presentation in 16 (76%) patients. Abdominal radiographs helped in preoperative diagnosis in 8 patients. Two patients had pneumoperitoneum on abdominal radiographs. At operation, type I CPC was found in 9 (42.8%) patients, type II in 5 (23.8%), type III in 2 (9.5%) patients, and type IV CPC in 5 (23.8%) patients. In 11 (52.4%) patients, pouch was emptied and retained with proximal enterostomy. In 7 (33.3%) patients, end enterostomy with pouch excision was done. In two patients, a window colostomy was formed. In one patient, pouch was disconnected from the normal bowel and Hartmann’s pouch with end ileostomy was formed. There were 2 (9.5%) deaths in our series.   Conclusion: CPC is a rare malformation. Massive abdominal distension with imperforate anus is the common presentation. Optimum management can reduce the morbidity and mortality. Key words: Imperforate anus, congenital pouch colon, urogenital anomalies   INTRODUCTION Congenital pouch colon is a variant of ARM, characterized by replacement of a variable length of normal colon by a pouch like struc-ture associated with short length of the total colon along with fistulous communication with the genitourinary system. Interestingly, the en-tity has a special predilection for the northern areas of India and most of the literature regard-ing its embryology, etiology, anatomy, and management has been reported from the same region [1]. Sporadic cases or case series have been documented from rest of the world [2].  In Pakistan, a term “football colon” is attributed to complete congenital pouch colon. The condi-tion is equally prevalent in Pakistan; however,   Congenital Pouch Colon: A Preliminary Report from Pakistan      Journal of Neonatal Surgery Vol. 1(3); 2012  no published literature on case series could be retrieved in spite of extensive search. Herein, we present our experience of managing the in-dex entity in a tertiary care centre of Pakistan. The objective of the study was to identify vari-ous presentations, management provided, and the outcome of these patients in our hospital. MATERIALS AND METHODS This is a retrospective review of patients of CPC, managed in the department of Pediatric Surgery, The Children’s Hospital and the Insti-tute of Child Health Lahore, Pakistan; during May 2007 through May 2010. The medical rec-ord of these patients was reviewed for demo-graphic information, clinical features, investiga-tions performed, operative notes, postoperative events, and the outcome of initial surgery. For descriptive purpose, CPC was classified into 4 types as per Rao et al classification (Table 1) [4]. Table 1: Classification of Pouch colon as per Rao et al [4]. Type  Description Type I  Type II  Type III  Type IV Ileum directly opens into the pouch colon- no normal colon available Cecum is present and rest of colon is in pouch configuration Considerable length of normal colon is present proximal to pouch colon. Only sigmoid colon and rectum are in the shape of pouch colon, rest of colon is nor-mal.  RESULTS Demography: Of the 312 cases of ARM, 21 (6.73%) had associated pouch colon, male: fe-male ratio being 3.2:1 (16 males and 5 fe-males). The age of initial presentation ranged between 12 hours and 45 days with a mean of 4.8 days (SD ±9.8). The mean weight at presen-tation was 2.3 kg (SD±0.43) with a range of 1.8kg to 3.8kg. Presentation: Nine patients presented with im-perforate anus and abdominal distension. Table 2 describes clinical presentation of the study group. Of 21 patients, 18 (85.7%) were associ-ated with high variety ARM and 3 (14.3%) with low ARM. Investigations: In 8 (38%) patients abdominal radiograph (erect and invertogram) delineated typical findings consistent with the diagnosis of CPC (Fig.1). Air vesicogram and pneumoperi-toneum was noted on radiographs in two pa-tients each. Ultrasound was diagnostic of CPC in 5 patients only (23.8%). Operation: All the 21 patients underwent pre-liminary operations. Eight (38%) patients un-derwent exploratory laparotomy through a right supra-umbilical transverse incision. They all belonged to high ARM and were diagnosed pre-operatively. In rest of the patients, preoperative diagnosis could not be made.  In 13 (62%) patients, CPC was diagnosed dur-ing colostomy formation. In 3 of these 13 pa-tients, cecum was found in the left iliac region while constructing colostomy for ARM (Fig. 2,3); In another patient with colostomy for cloaca, type IV CPC was found on re-exploration for massive mucosal prolapse. In 9 patients, ileum directly opened into the pouch colon (Type I CPC). Various types of CPC encountered in the study population are tabulated here (Table 3).   Table 2: Showing clinical presentation with respect to frequency, proportions and gender. Clinical presentation with respect to frequency and gender Male  Female Presentation  Frequency  Presentation  Frequency Imperforate anus with abdominal disten-sion Imperforate anus with meconuria and abdominal distension 9  7 Cloaca and abdominal distension Vestibular fistula + intractable constipation + abdominal distension Vestibular fistula + perineal rashes + abdominal distension Anteriorly placed anus + abdominal distension + intractable constipation  2 1   1   1  Congenital Pouch Colon: A Preliminary Report from Pakistan    Journal of Neonatal Surgery Vol. 1(3); 2012   Figure 1: (A) Abdominal radiograph (invertogram) showing a large loop of bowel filled with meconium and air, occupying more than half of the abdominal cavity. (B) Erect radiograph of the same patient.  Figure 2: Cecum and appendix lying at abnormal position, observed during colostomy formation in a patient of ARM that later proved to have type IV CPC. In two patients with type I CPC, there was per-foration of the pouches at lower end of CPC (Fig.4,5). In all the male patients there was a fistula with the posterior wall of urinary bladder (Fig. 6), whereas in female patients the fistula was with the cloaca in 2, and with vestibule in 2, and in one patient the pouch was continuous with an-teriorly placed anus. Table 3: Types of CPC as per Rao et al with type of ARM. Type of CPC Frequency Type of ARM Type I Type II Type III Type IV 9 5 2 5 High High High  Low /high, 3/2   Figure 3: Pouch colon in another patient in whom cecum and appendix were observed in left iliac re-gion.  Figure 4: Operative view of Type I CPC; the ileum is directly opening into the CPC from right side.  Figure 5: Operative view of a Type I CPC which was perforated at one side. In 4 of 9 patients with type I CPC, the pouch was emptied and retained with proximal ileos-tomy. In 3/9 patients with type I CPC the pouch was excised with end ileostomy. In 1 critical patient with type I pouch colon, the pouch was exteriorized as window colostomy. In one patient with type I CPC, the pouch was dis-connected from the normal bowel and Hart-mann’s pouch with end ileostomy was formed.  Congenital Pouch Colon: A Preliminary Report from Pakistan      Journal of Neonatal Surgery Vol. 1(3); 2012  The surgical treatment in the rest of study group is tabulated as under (Table 4).  Figure 6: Operative view of a long and wide fistula with the posterior wall of urinary bladder. Table 4: Summary of surgical procedures. Type I CPC  Initial Procedure Frequency  Proximal ileostomy (pouch retained) Pouch excision and end ileostomy Window colostomy End ileostomy with Hartmann pouch 4 3 1 1 Type II CPC Initial Procedure  Frequency  Proximal ileostomy (pouch retained) Pouch excision and end ileostomy Window colostomy 3 1 1 Type III CPC Initial Procedure  Frequency Pouch excision and end colostomy  2 Type IV CPC Initial Procedure Frequency Proximal colostomy (pouch retained) Pouch excision and end colostomy 4 1  Associated Anomalies: Associated anomalies were mainly from genitourinary and gastroin-testinal tract. Malrotation was observed in 5 patients, hydroureter in 2, and left renal agene-sis in 1 patient. Table 5 describes the associat-ed anomalies encountered in our series. Complications: Postoperative complications en-countered during initial surgery were wound infection in 4, mild wound dehiscence in 2, and enterostomy prolapse in 3 patients. The mortal-ity in our series was 9.5% (2 patients); both the patients were critically sick since the presenta-tion and one patient had associated esophageal atresia and tracheoesophageal fistula. Table 5: Showing frequency of associated anomalies Associated anomalies Frequency   Malrotation Hydroureter Vesicoureteric reflux Meckel’s diverticulum Esophageal atresia and TEF Duplex appendix Renal agenesis Undescended testes Lumbar hernia Scoliosis  Bicornuate uterus Vaginal septum 5 2 2 1 1 1 1 1 1 1 1 1   DISCUSSION  The incidence of CPC in our series was 6.73% of all the cases of ARM. The reported incidence of CPC in other series from India lies between 5-18% of all the cases of ARM [1-4]. The inci-dence of CPC in the western world is less than 1%. The exact incidence of this anomaly in Pa-kistan cannot be commented on as to scarce published data from this country. Similarly the sex distribution (M:F = 3.2:1)in our study was also in agreement with the reported male to fe-male ratio (3-7:1) [1-7]. The presentation may be early or delayed de-pending upon gender, size of fistula and the type of the CPC. In males, massive abdominal distension with imperforate anus during first or second day of life is the usual early presenta-tion, however in case of a wide fistula the pa-tient may be decompressed and present with mild abdominal distension and muconuria. The presentation in females is usually delayed to their early infancy with intractable constipa-tion, recurrent attacks of enterocolitis and per-sistent abdominal distension. The delayed presentation in females correlates with the type of ARM; the patients with associated cloaca usually present early as compared to vestibular fistula or other low ARM, where they are delib-erately deferred to some later age for definitive surgery [1,5-8]. Two out of 5 female subjects with CPC in our series had presented early with cloaca. Of the other 3 patients, 2 presented  Congenital Pouch Colon: A Preliminary Report from Pakistan    Journal of Neonatal Surgery Vol. 1(3); 2012    with intractable constipation and one patient with perineal rashes due to recurrent attacks of enterocolitis and soiling of fecal matter. All the male patients presented early.  Preoperative diagnosis of CPC can be easily made with the help of radiographs (erect and invertogram). A large loop of bowel filled with air and meconium, occupying more than half of the abdominal cavity, is usually diagnostic of CPC in a patient with ARM. Sometimes addi-tionally air vesicogram may be evident on radi-ographs [9]. In our series abdominal radio-graphs were performed in 10 patients that showed typical features of CPC in 8 and pneumoperitonium in 2 patients. In male pa-tients with visible meconuria, and in female patients with common channel, radiographs were not performed on account of the clinical features depicting their type of ARM and straightforward decision of colostomy.  In the index study, type I CPC is frequent varie-ty which corroborates with the previously pub-lished series [4,10-12]. The recent studies how-ever documented type IV CPC being more prev-alent than Type I or II varieties. This can be at-tributed to an increased awareness of the entity as to its morphology, pathology, and manage-ment in the evolving years [1,2,8]. The fistula with the genitourinary tract is char-acteristic of the index entity. Fistulous commu-nication in males occurs with the urinary tract most often with the urinary bladder [1,9,13] as in our series, and in females the site of fistula varies from perineum to the vestibule to the cloacal malformation [5-7].  A number of genitourinary and gastrointestinal anomalies have been associated with CPC. Most common anomalies are hydrouretro-nephrosis, vesicouretric reflux, renal dyspla-sia/agenesis, bicornuate uterus, hypospadias, followed by malrotation, and appendicular du-plex or agenesis, and prune belly syndrome [1,14]. In our series, 11 patients had associated anomalies, the commonest being malrotation. In one female patient of type IV CPC (anteriorly placed anus) there was associated scoliosis and lumber hernia.  CPC can be managed as a primary one stage procedure or as staged procedure. Single stage procedure ideally consists of repair of fistula, excision of the pouch, and primary abdomino-perineal-PSARP/pull through [1,12]. Multi-staged procedures are variable and range from merely window colostomy to complete excision of CPC, repair of the recto-vesical fistula, and end enterostomy in the first stage followed by pull through of the normal bowel with/without covering enterostomy as a second stage proce-dure. Initially single stage procedures were not considered safe but recent reports emerged with good results. Pouch colon patch over the pulled through segment is also advocated [1,12, 16-19]. Window colostomy is indicated in criti-cal patients, but it is often associated with high morbidity and mortality. The problems with window colostomy are massive mucosal pro-lapse, urinary tract infections, incomplete emp-tying of the CPC resulting in persistent ab-dominal distension etc. [1]. The outcome in CPC has improved with mortality reduced from >40% to less than 10% [1,5]. To summarize, CPC is rare malformation in pa-tients of ARM though the incidence in the Asian subcontinent is higher than in the western world. Imperforate anus with massive ab-dominal distension is a common presentation of this anomaly. An erect plain abdominal radi-ograph is diagnostic and should be done in all patients of ARM with abdominal distension. If one encounters cecum and appendix in the left lower quadrant while colostomy formation for ARM, it is mandatory to look for CPC.   REFERENCES 1. Gupta DK, Sharma S. Congenital pouch colon - Then and now. J Indian Assoc Pediatr Surg. 2007; 12: 5-12. 2. Bhat NA. Congenital pouch colon syndrome: A report of 17 cases. Ann Saudi Med. 2007; 27: 79-83. 3. Gupta DK. Congenital pouch colon: Present lacunae. J Indian Assoc Pediatr Surg. 2007; 12: 1-2. 4. Narsimha Rao KL, Yadav K, Mitra SK, Pathak IG. Congenital short colon with imperforate anus (pouch colon syndrome). Ann Pediatr Surg. 1984; 1: 59. 5. Sarin YK, Nagdeve NG, Sengar M. Congenital pouch colon in female subjects. J Indian Assoc Pediatr Surg. 2007; 12: 17-21.  Congenital Pouch Colon: A Preliminary Report from Pakistan      Journal of Neonatal Surgery Vol. 1(3); 2012  6. Chadha R, Gupta S, Mahajan JK, Bagga D, Kumar A. Congenital pouch colon in females. Pediatr Surg Int. 1999; 15: 336-42. 7. Gharpure V. Our experience in congenital pouch colon. J Indian Assoc Pediatr Surg. 2007; 12: 22-4. 8. Pavai A, Pillai SD, Shanthakumari S, Sam CJ, Shylaja M, Sabarivinoth R. Congenital pouch colon: Increasing association with low anorectal anomalies. J Indian Assoc Pediatr Surg. 2009; 14: 218-20. 9. Mirza B, Ijaz L, Sheikh A. Congenital pouch colon. Ann Saudi Med. 2011; 31: 546-7. 10. Wakhlu AK, Wakhlu A, Pandey A, Agarwal R, Tandon RK, Kureel SN. Congenital short colon. World J Surg. 1996; 20: 107-14. 11. Budhiraja S, Pandit SK, Rattan KN. A report of 27 cases of congenital short colon with an imperforated anus: So called 'pouch colon syndrome'. Trop Doctor. 1997; 27: 217-20. 12. Gangopadhyay AN, Shilpa S, Mohan TV, Gopal SC. Single-stage management of all pouch colon (anorectal malformation) in newborns. J Pediatr Surg. 2005; 40: 1151-5. 13. Mirza B, Ijaz L, Zaman M, Sheikh A. Congenital pouch colon with ileovesical and colovesical fistulae: A new variant. J Indian Assoc Pediatr Surg. 2011; 16: 167-8. 14. Baba AA, Hussain SA, Shera AH, Patnaik R. Prune belly syndrome with pouch colon and absent dermatome. Afr J Paediatr Surg. 2010; 7: 25-7. 15. Ratan SK, Rattan KN. "Pouch colon patch graft": An alternative treatment for congenital short colon. Pediatr Surg Int. 2004; 20: 801-3. 16. Gupta DK. Anorectal malformations-Wingspread to Krickenbeck. J Indian Assoc Ped Surg. 2005; 10: 79. 17. Luzzatto C, Zanardao V, Guglielmi M. Imperforate anus with congenital short colon: Combined abdominal and posterior sagittal approach. Pediatr Surg Int. 1990; 5: 375-6. 18. Sharma AK, Harjai MM. Simplified colorrhaphy and posterior sagittal anorectoplasty for the management of congenital short colon with imperforate anus. Br J Surg. 1997; 84: 389. 19. Puri A, Chadha R, Choudhury SR, Garg A. Congenital pouch colon: Follow-up and functional results after definitive surgery. J Pediatr Surg. 2006; 41: 1413-9.     Address for correspondence Muhammad Bilal Mirza,  428 Nishter Block, Allama Iqbal Town Lahore, Pakistan.  E mail: blmirza@yahoo.com  © Mirza et al, 2012 Submitted on: 15-04-2012 Accepted on:   30-05-2012 Published on:  01-07-2012 Conflict of interest: The first author is a Joint Editor of the journal. But he did not take part in the evaluation or decision making of this manuscript. The manuscript has been independently handled by two other editors.   Source of Support: Nil     This work is licensed under a Creative Commons Attribution 3.0 Unported License Available at http://www.jneonatalsurg.com  How to cite: Mirza B, Ahmad S, Sheikh A. Congenital pouch colon: a preliminary report from Pakistan. J Neonat Surg 2012; 1: 37.    

Congenital Pouch Colon: A Preliminary Report from Pakistan

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Congenital Pouch Colon: A Preliminary Report from Pakistan

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