eMedicine Specialties > Pediatrics: Surgery > General Surgery

Cloacal Malformations: Treatment

Author: Marc A Levitt, MD, Assistant Professor of Surgery and Pediatrics, University of Cincinnati; Associate Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Coauthor(s): Alberto Pena, MD, Consulting Surgeon, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Contributor Information and Disclosures

Updated: Jul 10, 2007

Treatment

Medical Therapy

Many patients require antimicrobial prophylaxis for urinary tract infections for associated vesicoureteral reflux.

Surgical Therapy

The repair of persistent cloaca represents a serious technical challenge and should be performed in specialized centers by pediatric surgeons dedicated to the care of these patients.

  • Cloacae represent a wide spectrum of defects. Repairing the defects involves dissection of the fused rectum, vagina, and urethra, which have formed a common channel (see Image 9). This dissection allows their separation and proper placement on the perineum. The length of the common channel can range from 1-10 cm.

  • The rectum and vagina share a common wall, and the vagina and urinary tract likewise have a common wall (see Image 8).

  • The goals of surgical treatment are to achieve bowel control, urinary control, and normal sexual function. Although all 3 goals are sometimes achieved, occasionally, none, 1, or 2 of the goals are achieved. Prognostic factors include quality of the sacrum, quality of the muscles, and length of the common channel.

  • Two groups of patients have been arbitrarily defined. Repair in patients with a common channel shorter than 3 cm is reproducible and feasible for most pediatric surgeons. In patients with a common channel longer than 3 cm, the repair should be performed at a specialized center by a surgeon experienced in managing urologic anomalies who is able to perform complex vaginal reconstructions. If the common channel is shorter than 3 cm, the posterior sagittal approach can be used to repair the defect without an abdominal approach. First, the rectum is mobilized. The vagina and urinary tract are then mobilized together (total urogenital mobilization), with the goal that both of these structures reach the perineum.

  • For patients with a common channel longer than 3 cm, a laparotomy is usually required. Often, the vagina and urinary tract must be separated to gain length, and the urethra must then be reconstructed. The surgeon must be prepared to open the bladder and to reimplant the ureters, if necessary. Complex vaginal mobilizations are often required, and vaginal replacement with small intestine or colon is frequently necessary. The complexities involved with these types of cloacal repair are beyond the scope of this article.

  • Total urogenital mobilization is a technique devised by the senior author; it allows mobilization of the urethra and vagina as one structure. This is possible in patients with the more benign types of cloacae. If total urogenital mobilization does adequately lengthen the vagina, the vagina and urethra must be separated, which is a technically challenging maneuver. Vaginourethral fistulae are more likely after this plane is dissected, which is why total urogenital mobilization significantly improves the patient's functional outcome.

  • The pull-through of the rectum is similar to that performed in other anorectal malformations. As with repair of imperforate anus, the rectum is placed within the limits of the sphincter mechanism, which is determined with an electrical stimulator.

  • The main surgical challenge involves the repair of the vagina, the urethra, and the associated urologic defects.

  • A large vagina can be an advantage during the definitive repair because the surgeon can more easily mobilize it and has more alternatives for vaginal repair. If the vagina required decompression in the newborn period, the pull-through may be tethered to the abdominal wall during a vaginostomy and must be taken down at the time of the definitive repair.

  • Approximately 50% of patients have various degrees of vaginal or uterine septation. These can be totally or partially repaired during the main operation or deferred for definitive repair until puberty. The precise gynecologic anatomy must be ascertained during either the main repair or the colostomy closure (if a laparotomy was not required during the main repair). Approximately one third of the author's patients have obstructed Müllerian structures, which can lead to severe problems due to retrograde menstruation.

  • Future problems, such as amenorrhea in patients with atretic uteri, hydrometrocolpos, and retrograde menses, can be predicted in the newborn period. Presentations of pelvic pain or amenorrhea in teenagers should prompt the suspicion of anomalous gynecologic structures (see Image 3 and Images 12-14). An ongoing collaborative relationship with a gynecologist is vital to address these issues, including fertility and safety of childbirth options.

Intraoperative Details

See Surgical therapy.

Postoperative Details

  • The Foley catheter remains in place for approximately 14 days (occasionally longer). The catheter should remain in place if the perineum appears swollen and the urethral orifice is not visible.

  • In patients with complex persistent cloaca, a suprapubic cystostomy tube may be kept in place for longer-term urinary diversion.

  • If the colostomy is untouched during the operation and no laparotomy was necessary, oral feedings may be started immediately postoperatively. If a laparotomy was necessary, the patient may require a period of fasting and nasogastric decompression.

  • Anal calibration is performed 2 weeks after the operation, followed by a program of anal dilatations. The anus must be dilated twice daily, and the size of the dilator is increased every week. The final size to be reached depends on the age of the patient.

  • Once the desired size is reached, the colostomy can be closed. Cystoscopy and vaginoscopy should be performed before colostomy closure to ensure that no urethrovaginal fistula is present, which would necessitate a repeat procedure.

  • Dilatations are continued afterward according to a prescribed protocol. They are a vital part of the postoperative management to avoid a stricture at the anoplasty site.

Follow-up

For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Bladder Control Problems.

Complications

If the persistent cloaca is not correctly identified, an obstructive uropathy is frequently overlooked. The patient may then receive only a colostomy and may subsequently experience sepsis, acidosis, and, sometimes, death. The other implication of failure to identify a persistent cloaca involves repairing only the rectal component of the anomaly, leaving the patient with a persistent urogenital sinus.

The main operative complication of concern is the development of urethrovaginal fistulae, which requires a reoperation. Therefore,  endoscopy must be performed prior to colostomy closure to ensure that no fistula has developed.

More on Cloacal Malformations

Overview: Cloacal Malformations
Workup: Cloacal Malformations
Treatment: Cloacal Malformations
Follow-up: Cloacal Malformations
Multimedia: Cloacal Malformations
References
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References

  1. Hendren WH, Oesch IL, Tschaeppeler H, Bettex MC. Repair of cloacal malformation using combined posterior sagittal and abdominal perineal approaches. Z Kinderchir. Apr 1987;42(2):115-9. [Medline].

  2. Rich MA, Brock WA, Pena A. Spectrum of genitourinary malformations in patients with imperforate anus. Pediatric Surg Intl. 1988;(3):110-113.

  3. Hendren WH. Cloaca, the most severe degree of imperforate anus: experience with 195 cases. Ann Surg. Sep 1998;228(3):331-46. [Medline].

  4. Hendren WH. Cloacal malformations: experience with 105 cases. J Pediatr Surg. Jul 1992;27(7):890-901. [Medline].

  5. Hendren WH. Further experience in reconstructive surgery for cloacal anomalies. J Pediatr Surg. Dec 1982;17(6):695-717. [Medline].

  6. Hendren WH. Management of cloacal malformations. Semin Pediatr Surg. Nov 1997;6(4):217-27. [Medline].

  7. Hendren WH. Pediatric rectal and perineal problems. Pediatr Clin North Am. Dec 1998;45(6):1353-72. [Medline].

  8. Hendren WH. Repair of cloacal anomalies: current techniques. J Pediatr Surg. Dec 1986;21(12):1159-76. [Medline].

  9. Hendren WH. Urogenital sinus and cloacal malformations. Semin Pediatr Surg. Feb 1996;5(1):72-9. [Medline].

  10. Hendren WH. Urological aspects of cloacal malformations. J Urol. Nov 1988;140(5 Pt 2):1207-13. [Medline].

  11. Hendren WH, Atala A. Use of bowel for vaginal reconstruction. J Urol. Aug 1994;152(2 Pt 2):752-5; discussion 756-7. [Medline].

  12. Hendren WH, Hendren RB. Bladder augmentation: experience with 129 children and young adults. J Urol. Aug 1990;144(2 Pt 2):445-53; discussion 460. [Medline].

  13. Jaramillo D, Lebowitz RL, Hendren WH. The cloacal malformation: radiologic findings and imaging recommendations. Radiology. Nov 1990;177(2):441-8. [Medline].

  14. Levitt MA, Pena A. Outcomes from the correction of anorectal malformations. Curr Opin Pediatr. Jun 2005;17(3):394-401. [Medline].

  15. Levitt MA, Pena A. Pitfalls in the management of newborn cloacas. Pediatr Surg Int. Apr 2005;21(4):264-9. [Medline].

  16. Levitt MA, Pena A. Pitfalls in the management of newborn cloacas. Pediatr Surg Int. Apr 2005;21(4):264-9. [Medline].

  17. Levitt MA, Stein DM, Pena A. Gynecologic concerns in the treatment of teenagers with cloaca. J Pediatr Surg. Feb 1998;33(2):188-93. [Medline].

  18. Lund DP, Hendren WH. Cloacal exstrophy: a 25-year experience with 50 cases. J Pediatr Surg. Jan 2001;36(1):68-75. [Medline].

  19. Lund DP, Hendren WH. Cloacal exstrophy: experience with 20 cases. J Pediatr Surg. Oct 1993;28(10):1360-8; discussion 1368-9. [Medline].

  20. Pena A. Anorectal malformations. Semin Pediatr Surg. Feb 1995;4(1):35-47. [Medline].

  21. Pena A. Management of anorectal malformations during the newborn period. World J Surg. May-Jun 1993;17(3):385-92. [Medline].

  22. Pena A. Posterior sagittal approach for the correction of anorectal malformations. Adv Surg. 1986;19:69-100. [Medline].

  23. Pena A. Surgical treatment of female anorectal malformations. Birth Defects Orig Artic Ser. 1988;24(4):403-23. [Medline].

  24. Pena A. The surgical management of persistent cloaca: results in 54 patients treated with a posterior sagittal approach. J Pediatr Surg. Jun 1989;24(6):590-8. [Medline].

  25. Pena A. Total urogenital mobilization--an easier way to repair cloacas. J Pediatr Surg. Feb 1997;32(2):263-7; discussion 267-8. [Medline].

  26. Pena A, Grasshoff S, Levitt MA. Reoperations in anorectal malformations. J Pediatr Surg. Feb 2007;42(2):318-25. [Medline].

  27. Pena A, Hong A. Advances in the management of anorectal malformations. Am J Surg. Nov 2000;180(5):370-6. [Medline].

  28. Pena A, Levitt M. Cloaca. In: Coran, Spitz eds. Operative Pediatric Surgery. 6th. Hodder Arnold; 2006.

  29. Pena A, Levitt MA, Hong A, Midulla P. Surgical management of cloacal malformations: a review of 339 patients. J Pediatr Surg. Mar 2004;39(3):470-9; discussion 470-9. [Medline].

  30. Pena A, Levitt MA, Hong A, Midulla P. Surgical management of cloacal malformations: a review of 339 patients. J Pediatr Surg. Mar 2004;39(3):470-9; discussion 470-9. [Medline].

  31. Pena A, Levitt MA. Neurogenic bladder and anorectal malformations. In: Esposito C, Guys JM, Gough D, Savanelli A, eds. Pediatric Neurogenic Bladder Dysfunction. Berlin: Springer; 2006:85-88.

  32. Pena A, Levitt MA. Anorectal malformations. In: Grosfeld JL, O'Neill JA, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. 6th ed. Philadelphia: Mosby Elsevier; 2006:1566-89.

  33. Pena A, Migotto-Krieger M, Levitt MA. Colostomy in anorectal malformations: a procedure with serious but preventable complications. Journal of Pediatric Surgery. Apr 2006;41(4):748-756. [Medline].

  34. Soffer SZ, Rosen NG, Hong AR, Alexianu M, Pena A. Cloacal exstrophy: a unified management plan. J Pediatr Surg. Jun 2000;35(6):932-7. [Medline].

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Further Reading

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Keywords

cloacal malformations, anorectal malformations, cloaca, cloacal anomalies, persistent cloaca, confluence of the rectum, confluence of the urethra, confluence of the vagina, urogenital sinus, posterior sagittal approach, posterior sagittal anorectovaginourethroplasty, PSARVUP, urinary continence, fecal continence, rectovaginal fistula, hydrocolpos, imperforate anus, vaginal scarring, cloacal exstrophy, midline intestinal plate, omphalocele, vesicostomy, genitourinary defects, rectovesical fistula, rectovestibular fistula, rectobulbar fistula, rectoperineal fistula, tethered cord, tethered spinal cord, presacral mass, dermoids, teratomas, anterior meningoceles, Currarino triad, hemisacrum, Mitrofanoff procedure, acidosis, spina bifida, spinal hemivertebrae

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Contributor Information and Disclosures

Author

Marc A Levitt, MD, Assistant Professor of Surgery and Pediatrics, University of Cincinnati; Associate Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Marc A Levitt, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Alberto Pena, MD, Consulting Surgeon, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Aviva L Katz, MD, Assistant Professor of Surgery, University of Pittsburgh School of Medicine; Consulting Staff, Division of General and Thoracic Surgery, Children's Hospital of Pittsburgh
Aviva L Katz, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association of Women Surgeons, Physicians for Social Responsibility, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Gail E Besner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Women's Association, American Pediatric Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, and Society of University Surgeons
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Pediatric Oncology Group, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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