rectal prolapse in autistic children

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Rectal Prolapse in Autistic Children By R. Van Heest, S. Jones, and M. Giacomantonio Halifax, Nova Scotia Rectal prolapse in children is not uncommon, but surgery is rarely indicated. In mentally challenged adults and children, rectal prolapse occurs more frequently than in the general population and often requires surgical intervention in the second to third decade of life. The authors describe 3 chil- dren with autism and mental retardation who presented with rectal prolapse at an earlier age than would be anticipated with mental retardation alone. All 3 children required surgi- cal intervention. J Pediatr Surg 39:643-644. © 2004 Elsevier Inc. All rights reserved. INDEX WORDS: Rectal prolapse, autism, mental retardation. R ECTAL PROLAPSE in children is not uncommon and usually presents as a self-limiting disorder in children less than 4 years of age. 1,2 Surgery rarely is indicated. Previous observers have noted that in mentally challenged adults and children rectal prolapse occurs more frequently than in the general population and more often requires surgical intervention. 3,4 Rectal prolapse has been described as a developmental rather than a congenital condition that manifests itself over time. 5 This is indicated by the fact that the average age of patients who are mentally challenged present with complete rec- tal prolapse in the second to third decade of life. 4 In the general population, the disorder presents even later in life, most commonly in elderly women. 6 We have iden- tified a subset of mentally challenged children who not only present with rectal prolapse at an early age (within the first 2 decades of life), but also have a similar Diagnostic and Statistical Manual of Mental Disorders (DSM) IV classification, that of autism. CASE REPORTS Case 1 A 9-year-old boy presented with rectal prolapse of 4 months dura- tion. The patient had Williams’s syndrome (microcephaly, mental moderate mental retardation, and coarctation of the aorta) previously diagnosed. The patient also met the criteria for the DSM IV classifi- cation of autism. Initially, conservative treatments were tried, but the patient was difficult to manage medically because of the fact that as the rectal prolapse increased in size, the patient developed the habit of “rectal digging.” Therefore, a sigmoid resection with rectal suspension without a protecting colostomy was undertaken. The patient did well postoperatively with the exception of one episode of self-inflicted proctitis. There was no evidence of reoccurrence of the prolapse at 11 years. Case 2 This boy presented at 8 years of age with a rectal prolapse. Initially, it was managed with a strict bowel regime. However, by the age of 12 it was apparent that the prolapse was intolerable and that a surgical intervention was warranted. The patient had autism, Tourettes syn- drome, obsessive compulsive disorder, and mild mental retardation diagnosed in early childhood. The patient underwent a sigmoid resec- tion and rectal suspension. On the sixth postoperative day the patient had anastomotic leak and was treated with a diverting colostomy. The colostomy was reversed 6 weeks later with no further complications and no reoccurrence of the rectal prolapse 5 years after the procedure. Case 3 This patient, another 8-year-old boy, presented with a 3-month history of rectal prolapse. Again, this patient had autism, developmen- tal delay, and attention deficit hyperactivity disorder diagnosed in early childhood. On initial presentation, it was apparent that the prolapse was causing the patient significant distress, and the patient underwent a sigmoid resection, rectal suspension, and appendectomy. The patient was not given a protecting colostomy, and the postoperative course was uneventful. To date there has been no evidence of reoccurrence at 2 years. DISCUSSION Rectal prolapse is associated with a number of medical conditions, such as straining associated with constipa- tion, acute or chronic diarrhea, parasitic and neoplastic diseases of the rectum, malnutrition, cystic fibrosis, and Williams syndrome. 7 It is also associated with children who have anatomic abnormalities, either congenital as with myelomeningocele or acquired as after a pull- through for Hirschsprungs disease. The developmentally delayed child is at risk of constipation, resulting from a combination of factors, for example, suppression by higher cortical function. 4 Consequently, this group of patients are at a higher risk of rectal prolapse earlier in From the Department of General Surgery and Division of Pediatric General Surgery, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada. Address reprint requests to Dr Sarah Jones, IWK Health Centre, Division of Pediatric General Surgery, 5850 University Ave, Halifax, NS B3J 3G9 Canada. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3904-0032$30.00/0 doi:10.1016/j.jpedsurg.2003.12.028 643 Journal of Pediatric Surgery, Vol 39, No 4 (April), 2004: pp 643-644

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Page 1: Rectal prolapse in autistic children

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Rectal Prolapse in Autistic ChildrenBy R. Van Heest, S. Jones, and M. Giacomantonio

Halifax, Nova Scotia

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ectal prolapse in children is not uncommon, but surgery isarely indicated. In mentally challenged adults and children,ectal prolapse occurs more frequently than in the generalopulation and often requires surgical intervention in theecond to third decade of life. The authors describe 3 chil-ren with autism and mental retardation who presented with

ectal prolapse at an earlier age than would be anticipated I

ntervention was warranted. The patient had autism, Tourettes syn-

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ournal of Pediatric Surgery, Vol 39, No 4 (April), 2004: pp 643-644

ith mental retardation alone. All 3 children required surgi-al intervention.

Pediatr Surg 39:643-644. © 2004 Elsevier Inc. All rightseserved.

NDEX WORDS: Rectal prolapse, autism, mental retardation.

ECTAL PROLAPSE in children is not uncommonand usually presents as a self-limiting disorder in

hildren less than 4 years of age.1,2 Surgery rarely isndicated. Previous observers have noted that in mentallyhallenged adults and children rectal prolapse occursore frequently than in the general population and more

ften requires surgical intervention.3,4 Rectal prolapseas been described as a developmental rather than aongenital condition that manifests itself over time.5 Thiss indicated by the fact that the average age of patientsho are mentally challenged present with complete rec-

al prolapse in the second to third decade of life.4 In theeneral population, the disorder presents even later inife, most commonly in elderly women.6 We have iden-ified a subset of mentally challenged children who notnly present with rectal prolapse at an early age (withinhe first 2 decades of life), but also have a similariagnostic and Statistical Manual of Mental Disorders

DSM) IV classification, that of autism.

CASE REPORTS

ase 1

A 9-year-old boy presented with rectal prolapse of 4 months dura-ion. The patient had Williams’s syndrome (microcephaly, mentaloderate mental retardation, and coarctation of the aorta) previously

iagnosed. The patient also met the criteria for the DSM IV classifi-ation of autism. Initially, conservative treatments were tried, but theatient was difficult to manage medically because of the fact that as theectal prolapse increased in size, the patient developed the habit ofrectal digging.” Therefore, a sigmoid resection with rectal suspensionithout a protecting colostomy was undertaken. The patient did wellostoperatively with the exception of one episode of self-inflictedroctitis. There was no evidence of reoccurrence of the prolapse at 11ears.

ase 2

This boy presented at 8 years of age with a rectal prolapse. Initially,t was managed with a strict bowel regime. However, by the age of 12t was apparent that the prolapse was intolerable and that a surgical

rome, obsessive compulsive disorder, and mild mental retardationiagnosed in early childhood. The patient underwent a sigmoid resec-ion and rectal suspension. On the sixth postoperative day the patientad anastomotic leak and was treated with a diverting colostomy. Theolostomy was reversed 6 weeks later with no further complicationsnd no reoccurrence of the rectal prolapse 5 years after the procedure.

ase 3

This patient, another 8-year-old boy, presented with a 3-monthistory of rectal prolapse. Again, this patient had autism, developmen-al delay, and attention deficit hyperactivity disorder diagnosed in earlyhildhood. On initial presentation, it was apparent that the prolapse wasausing the patient significant distress, and the patient underwent aigmoid resection, rectal suspension, and appendectomy. The patientas not given a protecting colostomy, and the postoperative course wasneventful. To date there has been no evidence of reoccurrence at 2ears.

DISCUSSION

Rectal prolapse is associated with a number of medicalonditions, such as straining associated with constipa-ion, acute or chronic diarrhea, parasitic and neoplasticiseases of the rectum, malnutrition, cystic fibrosis, andilliams syndrome.7 It is also associated with childrenho have anatomic abnormalities, either congenital asith myelomeningocele or acquired as after a pull-

hrough for Hirschsprungs disease. The developmentallyelayed child is at risk of constipation, resulting from aombination of factors, for example, suppression byigher cortical function.4 Consequently, this group ofatients are at a higher risk of rectal prolapse earlier in

From the Department of General Surgery and Division of Pediatriceneral Surgery, IWK Health Centre, Dalhousie University, Halifax,ova Scotia, Canada.Address reprint requests to Dr Sarah Jones, IWK Health Centre,

ivision of Pediatric General Surgery, 5850 University Ave, Halifax,S B3J 3G9 Canada.© 2004 Elsevier Inc. All rights reserved.0022-3468/04/3904-0032$30.00/0

doi:10.1016/j.jpedsurg.2003.12.028

643

Page 2: Rectal prolapse in autistic children

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644 VAN HEEST, JONES, AND GIACOMANTONIO

ife.4 We have identified a group of individuals in whichectal prolapse occurred during the first 2 decades of life.ll of these patients have had Autistic disorder (axis I)iagnosed according to the DSM IV classification. Theiagnosis of autism usually is associated with mentaletardation (axis II), and this was certainly the case in ourroup of patients. The diagnostic criteria for autisticisorders are very specific; there must be demonstratedualitative impairment in social interaction and in com-unication and restricted repetitive and stereotyped pat-

erns of behavior. Furthermore, abnormal functioning inhe above areas must be demonstrated before the age ofyears. Williams syndrome is caused by a microdeletionf chromosome 7, causing deletion of the ELN gene thatodes for the structural protein elastin. This elastin de-etion explains some of the characteristics of Williamsyndrome, hoarse voice, bladder and bowel diverticuli,nd rectal prolapse, which occurs in approximately 10%o 15% of patients. The management of rectal prolapsessociated with Williams syndrome appears to be nonop-rative because a review of the literature did not mentionurgical intervention but, rather, bowel management.7

There is no good evidence as to why rectal prolapseccurs in autistic children earlier than in children withental retardation alone. However, what is clear is that

he children do not seem to respond to efforts aimed atinimizing straining with defecation. When prolapse

ccured in this patient population, it was very distressfulor both patients and their caregivers, and it was apparenthat a definitive intervention was necessary.

The surgical procedures reported for the managementf rectal prolapse are many.8 The nature of the prolapsehese children experienced was a full-thickness intussus-eption of the rectum through a very stretched pelvicusculature and anal canal. Resection of the intussus-

epting segments seemed the best definitive option. Therocedure used for all 3 children was full mobilization ofhe rectum and sigmoid colon with resection of enough

ectosigmoid bowel to prevent any significant amount of d

REFEREN

ectum 10:141-145, 1967

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owel to be intussuscepted during the defecation effort.his meant leaving no redundant sigmoid loop and yetttaining a tension free anastomosis. The pelvic floor wasot repaired in any way because it was felt that prevent-ng intussusception would allow the normal architecturen an otherwise healthy and well-nourished child toegain its tone and function. The return to normal bowelunction with continence postsurgery seems to havealidated this surgical approach.One of the children had an anastomotic leak that

equired a temporary diverting colostomy. At the time ofolostomy, the anastomosis was found to have a suturehat had torn through. Although the anastomosis wasade without any obvious tension, the weight of the

owl and possibly the efforts of defecation in the post-perative period was enough strain to have led to a tear.he option of a rectopexy along with the rectosigmoid-ctomy as proposed by others 8 may have prevented this,ut during the time of the procedure, fixation of theectum to the presacral area seemed inappropriate be-ause it would have left a longer loop of bowel tontusscept if intussusception were to occur. The use of aoreign material such as polypropylene mesh to helpuspend the rectum seemed unwise in the presence of aesection and anastomosis in a clean but contaminatedeld. The option of a diverting ostomy was considered,ut an attempt was made to minimize further aggravationf already complicated care in these children. A full bowelreparation preoperatively was given to minimize fecalontamination.

We present 3 children with autism and mental retar-ation who presented with rectal prolapse at an earlierge than would be anticipated with the diagnosis ofental retardation alone. The rectal prolapse was full

hickness in nature and unresponsive to nonoperativeeasures. Full mobilization of the rectum and sigmoid

olon with resection of bowel sufficient to minimizeurther intussusception and prolapse seems appropriate

efinitive management in these cases.

CES

1. Qust N, Rasmussen L, Klassaborg KE, et al: Rectal prolapse innfancy: Conservative versus operative treatment. J Pediatr Surg 21:87-888, 19862. Dorman ML: Rectal prolapse in children. Dis Colon Rectum

8:535-539, 19853. Frederick WC, Hammond J, Almeda IC: Surgery in the mentally

etarded. Am Surg 36:533-537, 19704. Daseler EH: Rectal prolapse in the mentally retarded. Dis Colon

5. Bishop PR, Nowicki MJ: Defecation disorders in the neurologi-ally impaired child. Pediatric Annals 28:322-329, 1999

6. Rectal prolapse. Lancet 338:(8767):605-606, 19917. Policy Statement: American Academy of Pediatrics: Health Care

upervision for children with Williams syndrome (RE0034). Pediatrics07:1192-1204, 20018. Corman ML, Garred JL, Holder TM, et al: Rectal Prolapse:

7-year experience with the posterior repair and suspension. J Pediatr

urg 25:992-995, 1990