Malformation Anorectal Rice

Figure 38-1 Lateral invertogram in a patient with anorectal agenesis and a rectourethral fistula. Air shadow is above the pubococcygeal line (P.C.). Air is noted anteriorly in bladder. I is the line of the ischial ossification site (the level of translevator lesion), whereas M.C. is the mucocutaneous line, the level of low (infralevator) lesions. PIT indicates the anal dimple. (From deVries, P.A.: The surgery of anorectal anomalies: Its evolution with evaluations of procedures. Curr. Probl. Surg., 21:1, 1984.)

Incidence Wangenstein Rice
Figure 38-2 A, Perineal appearance of anorectal anomalies in boys and girls. B, The lateral view of the same defects.
Boys Rectum

Figure 38-3 A, Appearance of a boy with an infralevator (low) lesion and anocutaneous fistula with small amount of meconium. B, Fistulous tract filled with meconium that extends anteriorly along the scrotal raphe. C, Perineum of a male infant with imperforate anus and a rectourethral fistula. No cutaneous fistula site is observed.

Neonate Anal Atresia

Figure 38-6 Y- V cutback anoplasty for anal atresia with anocutaneous fistula. After Y Incision (A), skin flaps are carefully raised, and the sphincter Is Identified and preserved (B). The rectal pouch Is Incised (C), and the edges of the rectum are sutured (full-thickness) to the skin edges (D and E).

Figure 38-6 Y- V cutback anoplasty for anal atresia with anocutaneous fistula. After Y Incision (A), skin flaps are carefully raised, and the sphincter Is Identified and preserved (B). The rectal pouch Is Incised (C), and the edges of the rectum are sutured (full-thickness) to the skin edges (D and E).

Prone Anus

Figure 38-7 Correction of imperforate anus with rectofourchette fistula. The anal site is selected with the aid of an electrical stimulator (A). Traction sutures are placed. The fistula is carefully dissected free with tenotomy scissors (B). The traction sutures are used to guide the opening to a transplanted anal location within the sphincter complex. Interrupted 4-0 absorbable sutures are used (C). The fistulous site is closed with interrupted 4-0 suture. Note the preservation of the perineal body (D).

Perineal AnoplastyPosterior Sagittal Anorectoplasty

Figure 38-8 Posterior sagittal anorectoplasty. In the prone position, an incision is made in the midline from the lower sacrum to the selected anal site (A). The levator and sphincter muscles are divided posteriorly in the midline. The rectal pouch is identified (B). The pouch is opened, and the rectourethral fistula is identified within the rectal lumen (C). Submucosal resection frees the bowel from the fistula, which is closed with interrupted sutures (D). The bowel is tapered to a No. 12 Hegar size (E). The muscle complex is reconstituted starting at the deepest portion of the puborectalis muscle and the deep external sphincter (F). Levators and superficial external sphincters are then reapproximated with interrupted sutures (G). The tapered anoplasty is sutured to the skin with interrupted 4-0 absorbable suture (H).

Perineal AnoplastyAnoplasty Anoplasty

Summary

The management of patients with variants of imperforate anus is difficult and carries a significant degree of physician responsibility, often requiring long-term follow-up into adulthood. A concise understanding of the anatomy and the surgical techniques is essential. These are procedures that should not be attempted by the "occasional surgeon" who rarely deals with neonatal anomalies. The first operation performed well by an experienced pediatric surgeon most often allows the child the best chance for successful bowel control.

References

1. Santulli, T.V., Schullinger, J.N., Kiesewetter, W.B., et al.: Imperforate anus: A survey from the members of the Surgical Section of the American Academy of Pediatrics. J. Pediatr. Surg., 6:484, 1971.

2. Holschneider, A.M., Pfrommer, W., and Gerresheim, B.: Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur. J. Pediatr. Surg., 4:303, 1994.

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7. Smith, E.D.: The bath water needs changing, but don't throw out the baby: An overview of anorectal anomalies. J. Pediatr. Surg., 22:335, 1988.

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9. Templeton, J.M., and O'Neill, J.A., Jr.: Anorectal malformations. In Ravitch, M.M., Welch, K., Randolph, J.G., et al. (eds.): Pediatric Surgery. Chicago, Year Book Medical Publishers, 1985, p. 1022.

10. Swenson, O., and Donnellan, W.L.: Preservation of the puborectalis sling in imperforate anus repair. Surg. Clin. North Am. 47:173, 1967.

11. Hassink, E., Rieu, P., Hamel, B., et al.: Additional congenital defects in anorectal malformations. Eur. J. Pediatr. 155:477, 1996.

12. Peña, A.: Posterior sagittal approach for the correction of anorectal malformations. Adv. Surg. 19:69, 1986.

13. Parrott, T.S.: Urologic implications of anorectal malformations. Urol. Clin. North Am. 12:13, 1985.

14. Rich, M.A., Brock, W.A., and Peña, A.: Spectrum of genitourinary malformations in patients with imperforate anus. Pediatr. Surg. Int. 3:110, 1988.

15. Zivkovic, S.M., Krstie, Z.D., and Vukanic, D.V.: Vestibular fistula: The operative dilemma—Cutback, fistula transplantation or posterior sagittal anorectoplasty? Pediatr. Surg. Int., 6:111, 1991.

16. Long, F.L., Hunter, J.V., Mahboubi, S., et al.: Tethered cord and associated vertebral anomalies in children and infants with imperforate anus: Evaluation with MR imaging and plain radiography. Radiology, 200:377, 1996.

17. Carson, J.A., Barnes, P.D., Tunell, W.P., et al.: Imperforate anus: The neurologic implications of sacral abnormalities. J. Pediatr. Surg., 19:838, 1984.

18. Tsakayannis, D.E., and Schamberger, R.C.: Association of imperforate anus with occult spinal dysraphism. J. Pediatr. Surg., 30:1010, 1995.

19. Levitt, M.A., Patel, M., Rodriguez, G., et al.: The tethered spinal cord in patients with anorectal malformations. J. Pediatr. Surg., 32:462, 1997.

20. Rivosecchi, M., Lucchetti, M.C., Zaccara, A., et al.: Spinal dysraphism detected by magnetic resonance imaging in patients with anorectal anomalies: Incidence and clinical significance. J. Pediatr. Surg., 30:488, 1995.

21. Davidoff, A.M., Thompson, C.V., Grimm, J.K., et al.: Occult spinal dysraphism in patients with anal agenesis. J. Pediatr. Surg., 26:1001, 1991.

22. Parrott, T.S., and Woodard, J.R.: Importance of cystourethrography in neonates with imperforate anus. Urology, 13:607, 1979.

23. Hasse, W.: Associated malformations with anal and rectal atresia. Prog. Pediatr. Surg. 9:99, 1976.

24. Khoury, M.J., Cordero, J.R., Greenberg, F., et al.: A population study of the VACTERL association. Pediatrics, 71:815, 1983.

25. Mollard, P., Marechal, J.M., and Jaubert de Beaujen, M.: Surgical treatment of high imperforate anus with definition of the puborectalis sling by an anterior perineal approach. J. Pediatr. Surg., 13:499, 1978.

26. deVries, P.A., and Peña, A.: Posterior sagittal anorectaoplasty. J. Pediatr. Surg., 17:638, 1982.

27. Wangensteen, O.H., and Rice, C.O.: Imperforate anus: A method of determining the surgical approach. Ann. Surg., 92:77, 1930.

28. Narasimharao, K.L., Prasad, G.R., Katariya, S., et al.: Prone cross-table lateral view: An alternative to the invertogram in imperforate anus. AJR, 140:221, 1983.

29. Potts, W.J., Riker, W.L., and DeBoer, A.: Imperforate anus with rectovesical, -urethral, -vaginal and perineal fistula. Ann. Surg., 140:381, 1954.

30. Okada, A., Kamata, S, Imura, K., et al.: Anterior sagittal anorectoplasty for rectovestibular and anovestibular fistula. J. Pediatr. Surg., 27:85, 1992.

31. Kiesewetter, W.B.: Imperforate anus II. The rationale and technique of the sacra-abdomino-perineal operation. J. Pediatr. Surg., 2:106, 1967.

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