Current Status M a i n t a i n i n g a c o n t i n u i n g flow of appropriate material for this section is impossible, at least currently. Nevertheless, from time to time, w e do receive unsolicited review articles that furnish concise, up-to-date i n f o r m a t i o n on specific subjects, and they w i l l appear in this section. Subjects covered, obviously, will depend upon our contributors. JRH
Rectal Prolapse in Children MARVIN L. CORMAN, M.D.
From the Department o] Colon and Rectal Surgery, Sansum Medical Clinic, Santa Barbara, Cali]ornia
Corman ML. Rectal prolapse in children. Dis Colon Rectum 1985;28:
535-539. Rectal prolapse in children is an uncommon clinical entity in western countries. Treatment is generally supportive with surgery reserved for the intractable case. If operative intervention is advised, it should consist of one of several limited procedures that can be safely performed with low morbidity: anal encirclement, presacral packing, sclerosing injection, or linear rectal cauterization. [Rectal prolapse; Cystic fibrosis; Malnutrition; Diarrhea; Rectopexy]
Predisposing F a c t o r s Rectal prolapse is an u n c o m m o n disease of western children, occurring most frequently in infants with cystic fibrosis. T h e condition can be associated with any illness that causes diarrhea (e.g., amebiasis, giardiasis, worms), constipation, frequent cough (especially w h o o p i n g cough) or malnutrition 8-13 (Table 1). T h i s last factor is a major cause of prolapse in infancy and childhood in underdeveloped countries. In fact, most of the reports emanate from Africa and India. Disappearance of the ischiorectal fat and lack of rectal support are believed to be the causative factors in addition to the diarrhea. Lockhart-Mummery a8 stated that "rectal prolapse is a comparatively c o m m o n affliction a m o n g children, especially in that class which attends hospitals." T h e implication of this statement is that in the "better classes," children are more well-nourished and more quickly attended to if they become ill. Soriano et al. m reported ten cases of rectal prolapse associated with Trichuris as the only intestinal parasite. Stern et al. ~9reported that rectal prolapse occurred in 112 of 605 cystic fibrosis patients (18.5 percent). In one third of their patients, the prolapse preceded the diagnosis of cystic fibrosis. Kulczycki and Shwachman a2 reported an incidence of 22.6 percent of 386 children afflicted with cystic fibrosis.
NOT LONG AGO, I was asked to participate on a panel at the annual meeting of the American College of Surgeons, discussing the subject of rectal prolapse. My area of responsibility was to be limited to the manifestation in children. I accepted this assignment not without trepidation, because my personal experience with the condition in the pediatric population was quite limited. T h e few patients I encountered were treated with reassurance and the beneficent passage of time. Only twice did I find it necessary to intercede, and then with only a minor surgical procedure. Since my presentation at the College meeting was assuredly not to be based on m y voluminous series, I initially perused the standard textbooks of pediatrics 1,2 and of colon and rectal surgery, 3-6 my own included. 7 However, they paid little attention to the condition in children. I then embarked on a Medline search of the literature and learned that the subject had not been reviewed. I have since made my verbal presentation, and now I have the temerity to present this paper in the hope that readers of the Journal m i g h t find this review of value in the care of their patients.
Etiology
Received for publication November 1, 1984. Address reprint requests to Dr. Corman: Sansum MedicalClinic, 317 West Pueblo Street, Santa Barbara, California 93105.
T h e etiology of the condition in infancy may be related to the loose attachment of the mucosa to the underlying
535
5~6
M.L. CORMAN
TABLE l. Associated Conditions and Predisposing Factors for the Development of Rectal Prolapse in Children Diarrhea (amebiasis, giardiasis, ulcerative colitis, trichuriasis) Constipation Straining at urination (e.g., phimosis) Vomiting Cough (e.g., pertussis) Malnutrition Cystic fibrosis Polyp or tumor 14,~5 Ehlers-Danlos syndrome 16 Myelomeningocele Spina bifida Hirschsprung's diseasO 7
muscularis. In this age group, the rectal mucosa may be normally redundant.Z Other anatomic factors in the child tend to predispose to the development of prolapse: vertical course of the rectum, flat sacrum and coccyx, low rectal position in relation to other pelvic organs, and lack of levator support. Rectal prolapse is most common in children under the age of three years, with the most frequent incidence in the first year of life. In this age group, it is the mucosa that tends to prolapse, not the full thickness of the bowel. Most studies report an approximately equal sex incidence.9,~s, 2~
Dis. C~
TABLE2. Medical Treatment of Rectal Prolapse in Children Manual replacement Support perineum during defecation Defecate in recumbent position Tape buttocks Paregoric for diarrhea Stool softeners for constipation
tocks to prevent the prolapse from recurring spontaneously. Stool softeners are advisable for constipation and paregoric for diarrhea (Table 2). Surgical treatment has been advised for patients who are malnourished and for those who do not respond to medical management. Recommended procedures include excision of the mucosal prolapse, 2anal encirclement with silastic t or with catgut, 9 the use of a sclerosing solution (e.g., 30 percent saline 2~ st or 70 percent alcohol), 22packing of the presacral space with gauze or Gelfoam| 1,8,18 linear cauterization of the a n o r e c t u m y transsacral rectopexy (with obliteration of the pouch of Douglas and puborectalis plication), 24 transcoccygeal rectopexy and puborectalis plication, 25perineal proctosigmoidectomy, 26 and transanal rectopexy with delayed suture removal 27 (Table 3).
Symptoms
Results
Symptoms include protrusion, bleeding, passage of mucus, diarrhea, constipation, abdominal pain, and those complaints that may be due to the associated condition. Signs include the protrusion itself, lax sphincter tone and contractility, and usually malnutrition.
T h e results of a number of series are summarized in Table 4. Kay and Zachary21 employed a sclerosing solution of 30 percent saline in 51 children. They noted 100 percent success in up to three treatments. However, two
Treatment
TABLE4. Results of Surgery for Rectal Prolapse in Children
Treatment usually consists of medical management, with normal growth of the child producing cure in the vast majority of patients. However, children with severe malnutrition and those with lack of accessibility to quality medical care and nutritional support are less likely to have the prolapse condition resolve spontaneously. Medical management consists of manual replacement, often with sedation; the knee-chest position may be helpful. Supporting the perineum during defecation is also beneficial as well as having the child defecate in the recumbent position. It may be necessary to tape the butTABLE 3. Surgical Treatment of Rectal Prolapse in Children Anal encirclement Packing Sclerosing Linear cauterization Excision Resection Rectopexy
Investigator Kay and Zachary 2~ (England) Malyshev and Gulin 2z (USSR)
Number of Children
Method
Success Rate (Percent)
51
Sclerosing solution (30 percent saline)
100 (up to three treatmerits)
353
Sclerosing solution (70 percent alcohol)
96
Narasanagi (India) 9
30
Anal encirclement (#1 CCG)
97
Nwako (Nigeria) s
100
Presacral packing (Gelfoam|
100
Hight et al. ~ (Wayne State)
73
Linear cauterization
97
Chino and Thomas 24 (North Carolina)
4
Transsacral rectopexy; puborectalis plication; obliteration of pouch of Douglas
100
Ashcraft et al. z5 (Kansas City)
4
Transcoccygeal rectopexy; puborectalis plication
100
Volume28 Number 7
C U R R E N T STATUS: RECTAL PROLAPSE IN CHILDREN
537
FIG. 1, Injection technique. Sclerosing agent is infiltrated in the perirectal tissue posteriorly and laterally. T h e index finger is inserted into the rectum to confirm the position of the needle tip.
abscesses complicated the procedure. Dutta and Das z~ utilized the same technique in 30 patients (Fig. 1). T h e y reported an 83 percent cure rate with one treatment and 100 percent cure with three treatments. Malyshev and Gulin ~2 reported 353 children from the U.S.S.R. with rectal prolapse treated by perirectal 70 percent alcohol. T h e authors cautioned that one should not employ more than 35 ml. Ninety-six percent were cured. T h e authors advised, however, that this treatment should be limited to those patients w h o do not satisfactorily respond to medical measures. Anal encirclement with silastic is the recommended
FIG. 2. L i n e a r cauterization. Fourquadrant electrocoagulation through the submucosa. (After Hight DW et al. 23)
approach in N e l s o n ' s T e x t b o o k o f Pediatrics. ~ Narasanagi 9 performed anal encirclement using #1 chromic catgut on 30 patients. He reported one failure due to breakage of the suture. Subsequent treatment effectively cured the prolapse. Packing the presacral space with gauze through a posterior approach as well as excision of the prolapsed mucosa for the intractable case have been recommended in R u d o l p h and H o f f m a n ' s textbook of Pediatrics. z N w a k o 8 packed the presacral space with Gelfoam in 100 patients and reported complete cure in every case. H i g h t et al. 23 recommend a surgical approach for
Dis. (2)1, g: Re~t.
538
M.L, CORMAN
july 1985
FIGS. 3A and B. Thomas operation. A. The redundant bowel is delivered through the defect after the levatores are incised. The pouch of Douglas is identified anteriorly, the peritoneum is opened, and the redundant sigmoid is delivered. B. The peritoneum is excised and the floor of the pelvis reconstituted by suturing the seromuscular layer of the proximal bowel to the peritoneum, The levator ani muscle is plicated anterior to the rectum. The bowel in children is not resected. (From Corman ML. 7)
patients who do not respond to medical measures. There were a total of 102 patients in their series, 29 of w h o m were adequately treated in a conservative manner. T h e r e m a i n i n g 73 underwent linear cauterization of the a n t rectum (Fig. 2); all but two patients were successfully treated by this approach. Heald, z7 in a report of his experience with one patient, was able to secure the rectum by means of transanal rectopexy, passing a suture through the full thickness of the rectum and skin overlying the coccyx. T h e suture then was removed at a later date. Perineal proctosigmoidectomy has been utilized in intractable situations with patients who have rectal prolapse secondary to spina bifida. 26 Chino and T h o m a s 2~ recommend transsacral rectopexy with obliteration of the pouch of Douglas and puborectalis plication (Fig. 3), the technique that has been used for adults, except resection is not recommended. Successful repair was noted in four patients. Transcoccygeal rectopexy and puborectalis plication is suggested by Ashcraft et al., 25 with four patients treated and cured by this technique. Opinion As stated at the outset, my limited observation of patients in this age group reveals that medical measures are almost always successful. For the rare infant or child w h o fails to respond, I believe that a minimal surgical
approach is indicated: perirectal injection with a sclerosing agent, presacral packing, an anal encircling operation, or linear rectal cauterization. All have been reported in relatively large series with excellent results and with m i n i m a l morbidity. While more extensive adult-type operative approaches are undoubtedly successful, I cannot justify their application in children when I perceive the availability of a simpler, safer, effective alternative.
References 1. Doershuk CF, Boat TF. In: Behrman RE, Vaughan VC, eds. Nelson's textbook of pediatrics. 12th ed. Philadelphia: WB Saunders, 1983:1097. 2. Santulli TV. In: Rudolph AM, Hoffman JI, eds. Pediatrics. 17th ed. Norwalk: Appleton-Century-Crofts, 1983:990-1. 3. Goligher JC. Surgery of the anus, rectum and colon. 4th ed. London: Balliere Tindall, 1980:227-8. 4. Goldberg SM, Gordon PH, Nivatvongs S. Essentials of anorectal surgery. Philadelphia: JB Lippincott, 1980:266. 5. Gabriel WB. The principles and practice of rectal surgery. 5th ed. London: HK Lewis, 1963:173-5. 6. Bac~ HE" Anus' rectum' sigm~ c~176 diagn~ and treatment" 3rd ed. Philadelphia: JB Lippincott, t949:507-10. 7. Corman ML. Colon and rectal surgery. Philadelphia: JB Lippincott, 1984:157. 8. Nwako F. Rectal prolapse in Nigerian children. Int Surg 1975;60: 284-5. 9. Narasanagi SS. Rectal prolapse in children. J Indian Med Assoc 1973;62:378-80. 10. Soriano LR, del Mundo F, Naguit-Sim L. Rectal prolapse in children with trichuriasis. J Philippine Med Assoc 1966;42: 843-8.
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CURRENT STATUS: RECTAL PROLAPSE IN CHILDREN
11. Traynor LA, Michener WM. Rectal procidentia--a rare complication of ulcerative colitis: report of two cases in children. Cleve Clin Q 1966;33:115-7. 12. Kulczycki LL, Shwachman H. Studies in cystic fibrosis of the pancreas: occurrence of rectal prolapse. N Engl J Med 1958;259: 409-12. 13. Bhandari B, Ameta DK. Etiology of prolapse rectum in children with special reference to amoebiasis. Indian J Pediatr 1977; I4:635-7. 14. Impieri M, Zambarda E. Rectal prolapse in a child with PeutzJeghers syndrome. Acta Gastroenterol Belg 1982;45:429-33. 15. Lamesch AJ. An unusual hamartomatous malformation of the rectosigmoid presenting as an irreducible rectal prolapse and necessitating rectosigmoid resection in a 14-week-old infant. Dis Colon Rectum 1983;26:452-7. 16. Douglas BS, Douglas HM. Rectal prolapse in the Ehlers-Danlos syndrome. Aust Paediatr J 1973;9:109-10. 17. Traisman E, Colon D, Sherman JO, Hageman JR. Rectal prolapse in two neonates with Hirschsprung's disease. Am J Dis Child 1983;137:1126-7. 18. Lockhart-Mummery P. Diseases of the rectum and anus. New York: William Wood, 1914:108.
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19. Stern RC, Izant RJ Jr, Boat TF, Wood RE, Matthews LW, Doershuk CF. Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterology 1982;82:707-10. 20. Dutta BN, Das AK. Treatment of prolapse rectum in children with injections of sclerosing agents. J Indian Med Assoc 1977;69:275-6. 21. Kay NR, Zachary RB. The treatment of rectal prolapse in children with injections of 30 percent saline solutions. J Pediatr Surg 1970;5:334-7. 22. Malyshev YI, Gulin VA. Our experience with the treatment of rectal prolapse in infants and children. Am J Proctol 1973; 24:470-2. 23. Hight DW, Hertzler JH, Philippart AI, Benson CD. Linear cauterization for the treatment of rectal prolapse in infants and children. Surg Gynecol Obstet 1982;154L400-2. 24. Chino ES, Thomas CG Jr. Transsacral approach to repair of rectal prolapse in children. Am Surg 1984;50:70-5. 25. Ashcrafi KW, Amoury RA, Holder TM. Levator repair and posterior suspension for rectal prolapse. J Pediatr Surg 1977; 12:241-5. 26. Nash DF. Bowel management in spina bifida patients. Proc R Soc Med 1972;65:70-1. 27. H,eald CL. A simple, bloodless operation for anorectal prolapse in children. Surg Gynecol Obstet 1926;42:840-1.