Transanal endorectal pull-through for Hirschsprung disease: … · 2016-01-08 · Transanal...

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Seminars in Pediatric Surgery (2010) 19, 96-106

ransanal endorectal pull-through for Hirschsprungisease: technique, controversies, pearls, pitfalls, and anrganized approach to the management of postoperativebstructive symptoms

uis De La Torre, MD,a Jacob C. Langer, MDb

rom the aUniversidad Nacional Autónoma de México, Department of Surgery, Hospital para el Niño Poblano andospital Angeles Puebla, Puebla, Mexico; and the

Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.

The transanal endorectal pull-through emerged in the late 1990s as the most recent step in the evolutionof the surgical correction of Hirschsprung disease. This operation provides the advantages of a minimalaccess approach with shorter hospital stay, shorter time to full feeding, less pain, and improvedcosmesis with excellent outcomes. This article will review the technical principles of the transanalendorectal pull-through, and will address ongoing controversies in the application of this technique. Wewill also discuss an organized approach to the problem of obstructive symptoms that may affect asubgroup of patients after the transanal pull-through.© 2010 Elsevier Inc. All rights reserved.

KEYWORDSSoave;Swenson;Transanalpull-through;Perineal pull-through;Enterocolitis;Botulinum toxin;Internal sphincterachalasia

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Swenson first described definitive surgical managementf neonates and infants with Hirschsprung disease in the late940s. Because these children often presented with severealnutrition or enterocolitis, a preliminary colostomy was

sually done, followed by a pull-through procedure manyonths later. Earlier recognition and diagnosis of the dis-

ase led a number of surgeons in the 1980s to report seriesf single-stage pull-through procedures even in small in-ants, using each of the 3 common operations (Swenson,uhamel, and Soave). Since then, one-stage operationsave become increasingly popular and many reports have

Address reprint requests and correspondence: Luis De La Torre,D, Universidad Nacional Autónoma de Mexico, Hospital para el Nino

oblano and Hospital Angeles Puebla, Av Kepler No. 2143, Cons. 215,ol. Reserva Territorial Atlixcayotl, Puebla 72190, Mexico.

aE-mail address: [email protected].

055-8586/$ -see front matter © 2010 Elsevier Inc. All rights reserved.oi:10.1053/j.sempedsurg.2009.11.016

uggested that this approach is safe, cost-effective, andvoids the morbidity of stomas in infants.1

In the early 1990s, Georgeson et al2 described a minimalccess approach, consisting of a laparoscopic biopsy todentify the transition zone, laparoscopic mobilization of theectum below the peritoneal reflection, and a short endorec-al mucosal dissection from below. The anastomosis wasone from below after prolapsing and excising the rectum.ultiple reports documented a short time in the hospital,

nd early results were equivalent to those reported for thepen procedures. Subsequently, laparoscopic approachesave been described for the Duhamel and Swenson opera-ions,3,4 with excellent short-term results reported.

The transanal Soave procedure represented a natural evo-ution from the laparoscopic operation. Transanal resectionf the rectum was shown to be possible in an animal model,
nd initial series of children with Hirschsprung disease were
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97De La Torre and Langer Transanal Pull-Through for Hirschsprung Disease

ublished by de la Torre and Ortega-Salgado5 and Langer etl6 in the late 1990s. The transanal approach has therincipal benefit of avoiding the need for intra-abdominalobilization of the rectum through either laparotomy or

aparoscopy. Several studies have demonstrated that theransanal approach is associated with less pain, shorterospital stay, and a better cosmetic result than openurgery.7-9 There have not been any studies comparing theransanal to the laparoscopic approach. However, theransanal pull-through can be done by any pediatric surgeon,ncluding those without laparoscopic skills, and by pediatricurgeons in parts of the world where access to appropriatelyiniaturized laparoscopic equipment is limited.10

This article will review preoperative considerations be-ore performing the transanal pull-through, technical as-ects of the operation, pitfalls and pearls the authors haveearned, and ongoing controversies. We will also present anrganized approach to the common problem of obstructiveymptoms after the transanal pull-through.

reoperative considerations

Hirschsprung disease may present as neonatal intestinalbstruction, as constipation in an older child, or less com-only with primary enterocolitis. In the neonate, Hirsch-

prung disease must be differentiated from other causes ofntestinal obstruction, including meconium ileus, intestinaltresia, anorectal malformation, malrotation, and congen-tal bands. Careful history and physical examination, ab-ominal radiogarph, and water-soluble contrast enema arehe initial diagnostic maneuvers in most cases. The defini-ive diagnosis of Hirschsprung disease is either made orxcluded on the basis of a suction rectal biopsy, looking forhe presence or absence of ganglion cells and of hypertro-hic nerves. Some pathologists also use cholinesterasetaining to complement the standard histologic evaluation.he biopsy must not be taken too close to the pectinate line,ecause there is normally a paucity of ganglion cells in thisocation.11 It is also important to initiate early resuscitationn infants with intestinal obstruction or enterocolitis, includ-ng the administration of intravenous fluids and antibiotics,nd a nasogastric tube. Early decompression of the colonsing digital rectal stimulation and/or irrigations through aectal tube is important to prevent and treat enterocolitis ando decrease the diameter of the colon. Children with asso-iated abnormalities, such as cardiac disease or congenitalentral hypoventilation syndrome, must have these prob-ems dealt with before definitive surgical repair. After thehild has been stabilized, the definitive surgical procedurean be done semielectively. During the waiting period, mosthildren can be fed breast milk or an elemental formula, inombination with rectal stimulations or irrigations. Thoseho cannot tolerate oral or nasogastric feeding can beourished with parenteral nutrition.

In the older child who presents with severe constipation,suction biopsy is less likely to give the pathologist enough

ubmucosa to make the diagnosis. In these children a deep o

r full-thickness biopsy must be done, usually under generalnesthesia. In some centers, anorectal manometry is alsosed as a screening test, because the presence of a rectoanalnhibitory reflex effectively rules out the diagnosis ofirschsprung disease. Absence of a recto-anal inhibitory

eflex must be followed by a rectal biopsy to confirm theiagnosis, as there is a significant false-positive rate for thisest. Older children who present with an extremely dilatedolon may require weeks or months of irrigations to bringhe colon to a more normal size before the definitive surgi-al procedure, and in some children with particularly dilatedr thickened proximal colon, a preliminary colostomy maye necessary to achieve adequate decompression.

Most children with Hirschsprung disease have a contrastnema done as part of the diagnostic workup, looking forhe presence and location of a radiological transition zone.owever, many studies have documented that a maximumf 10% of neonates with Hirschsprung disease will not havetransition zone on contrast enema.12 In addition, older

hildren with a very short aganglionic segment may notemonstrate a transition zone on contrast enema, particu-arly if the catheter has been placed above the transitionone in the rectum. Finally, the contrast study is not alwaysompletely accurate in identifying the location of the patho-ogic transition zone, with 12% of cases having a pathologicransition zone which is different from the radiologicalransition zone.13 It may be possible to increase the accu-acy of the contrast enema in identifying the transition zonen older children by waiting until histopathological confir-ation of the disease is available, and discontinuing the

ectal irrigations for 1-3 days before performing the contrastnema. It is also important to recognize that the lateralrojection is better than the anteroposterior view in identi-ying a rectal or rectosigmoid transition zone (Figure 1).

urgical technique

asic principles of the operation

fter induction of anesthesia, before beginning the opera-ion, a single shot caudal block should be placed to mini-ize the need for anesthetic agents. We routinely repeat the

lock again at the end of the procedure to provide earlyostoperative pain relief. All patients should receive pro-hylactic antibiotics, such as cefoxitin to cover Gram-neg-tive bacilli and colonic anaerobes.

The operation starts with a mucosal incision above theentate line. The distance above the dentate line depends onhe surgeon and the size of the child, but it is crucial that thencision be high enough above the dentate line so that theransitional epithelium is not damaged. This is important torevent loss of sensation, which may predispose the child toong-term problems with incontinence.

Fine silk sutures are placed in the mucosa, either before
r after the mucosal incision, to provide traction on the

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98 Seminars in Pediatric Surgery, Vol 19, No 2, May 2010

ucosal edge during the mucosal dissection. The mucosa istripped from the underlying muscle for a variable distance,nitially using electrocautery with a fine-tipped needle, andubsequently using blunt dissection. After the mucosal dis-ection has been completed, the rectal muscle is incisedircumferentially, permitting the dissection to continueroximally along the rectal wall. It is important that theessels be divided just as they enter the bowel wall, to avoidnjury to pelvic nerves and vessels, as well as the prostate oragina. It is also very important to avoid twisting of theowel as the dissection progresses proximally. This can beccomplished by holding the rectum with a curved clamp,aking sure that the orientation of the clamp does not

hange. When the normally innervated bowel is reached, theowel is divided a variable distance above the transitionone, and the anastomosis is performed. Most surgeons useraided absorbable suture material for the anastomosis. Theutures should include a generous bite of the pull-througholon, as well as substantial bite of underlying muscle andsmall bite of distal mucosa. Once again, care must be

aken not to include the dentate line in the sutures, as thisill produce more pain, and may compromise later conti-ence.

The transanal technique is summarized in Figure 2.For children with a transition zone that is more proximal

han the midsigmoid colon, there is usually not enoughength to bring it down without some mobilization of at leasthe descending colon, and in some cases the splenic flexure.

igure 1 (A) Rectogram of a histological-confirmed case of Hirsf the water-soluble contrast solution. (B) Lateral view showing hdilated loop, but the transition zone cannot be defined in this viewiew. This projection is more useful than the AP view in short se

his can be done either laparoscopically or through an d

mbilical incision. If the transition zone is at or proximal tohe splenic flexure, it is usually also necessary to divide theiddle colic vessels, leaving a generous arcade of marginal

essels along the colon, so as to achieve adequate lengthithout undue tension (Figure 3).

ngoing controversies, pearls, and pitfalls

ge at pull-throughSome surgeons prefer to wait until the child is a few

onths old before doing the procedure, and the child isischarged home on rectal stimulation and/or rectal irriga-ions while waiting. There are several expressed reasons forhis, such as a feeling that the operation will be easier withetter visualization, a hope that the dilated proximal colonill decrease in size, and in some cases a practical issue ofperating room time availability. The primary danger of thispproach is the possibility of the child developing entero-olitis during the waiting period. This risk can be minimizedy ensuring adequate decompression of the rectum, the admin-stration of prophylactic metronidazole or probiotics, and these of breastfeeding or elemental infant formula. Many pedi-tric surgeons have now realized that the transanal pull-hrough can be successfully and safely performed as soon ashe diagnosis is made, even in small newborns. The success ofhe procedure lies in magnification with loupes, meticulous

ng disease showing a fluoroscopic lateral view starting instillatione dilated sigmoid is being initially filled. (C) Frontal view shows

adiological transitional zone is clearly demonstrated in the lateralHirschsprung disease.

chspruow th. (D) R

issection, and the fact that the neonatal pelvis is very shallow.

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igure 2 Transanal technique. These examples are from a case done in the prone position. (A) The Lonestar retractor has been used to demonstratehe dentate line, and sutures have been placed in anticipation of a mucosal incision 0.5-1 cm above the dentate line. (B) Submucosal dissection has beenone. (C) The rectal muscle cuff is being incised. (D) The dissection has been continued along the outside of the rectal wall, taking vessels as they enterhe rectal wall using cautery or ties. (E) A biopsy has been done from the dilated rectum above the presumed transition zone. The biopsy should beull-thickness so the pathologist has the best chance possible to make an accurate diagnosis on frozen section. (F) The rectal muscle cuff has been splitn the posterior midline. This step is not necessary if a very short cuff is used. (G) The rectum has been amputated and the anastomosis has been completed.
Color version of figure is available online.)

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se of a colostomy and performance of the operationn a patient with a preexisting colostomy

Although most pediatric surgeons have now abandonedhe routine use of a colostomy, there are still some childrenn whom a colostomy should be considered. Probably theost common scenario in which this would be appropriate

s the child who presents with enterocolitis. Enterocolitisust be treated aggressively with antibiotics, fluid resusci-

ation, and rectal irrigations. Those patients in whom sepsisontinues despite nonoperative measures should have atoma created. Those who respond successfully to nonop-rative treatment can undergo a one-stage pull-through asoon as the child is completely stable. The threshold forreating a colostomy varies from surgeon to surgeon.

Other patient-related indications for a stoma include pre-entation with intestinal perforation, malnutrition, or mas-ively dilated proximal bowel. In addition, some surgeonsork in an environment where there is inadequate pediatricathology support to reliably identify the transition zone onrozen section. In this situation, there are 2 choices. Theurgeon can do multiple biopsies laparoscopically orhrough a small incision, and wait for the final results beforeoing back for a second anesthetic to do the definitiveull-through, or a colostomy can be done.

For those children who require a stoma, or those whoave a routine stoma done by another surgeon, a transanalpproach to the pull-through procedure can still be accom-lished. The colostomy and mucous fistula (if it is a dividedtoma) are taken down first, and the mesenteric vessels tohe distal bowel can be divided through the colostomy site.he transanal dissection is then carried out as describedreviously, and the distal bowel removed through the anus.he colostomy is then grasped with an instrument that isassed transanally, and pulled down for the anastomosis.

Some surgeons choose to perform a proximal colostomyn the right transverse colon in every case of Hirschsprungisease, without “leveling” biopsies. The rationale for this ishat the colostomy will function even if the transition zones proximal to the stoma, and this is therefore the safest

igure 3 Mobilization of the colon for children with a more proxr sigmoid colon, no proximal mobilization is usually required. (Bividing the lateral attachments of the descending colon, with or wone at or just proximal to the splenic flexure, the flexure must bearginal vessel are preserved.

pproach in centers where there is inadequate pediatric p

athology support. However, a transanal approach to theull-through can still be used, leaving the colostomy intact,f there is enough length. If the colostomy results in toouch tension, the surgeon has 3 options: (1) use the right-

ided colostomy as the bowel to be pulled through; (2) closehe colostomy and perform a loop ileostomy; or (3) close theolostomy without defunctioning. If the latter choice isade, the surgeon might consider using a Duhamel rather

han a transanal approach, because the incidence of anasto-otic narrowing and stricture, and therefore the risk of leak

rom the colostomy closure, is lower.

he use of a preliminary biopsy prior to beginning thenal dissection

Most surgeons suspect the most likely location of theransition zone is based on the contrast enema, as well as onhe acuity and severity of the patient’s symptoms. However,s mentioned earlier, the contrast study is not always com-letely accurate, and some surgeons therefore prefer tobtain pathologic confirmation of the transition zone beforeeginning the anal dissection. This approach is particularlyseful for those surgeons who would prefer to do a Duhamelather than a Soave or Swenson procedure if the child hasong-segment disease.

The preliminary biopsy can be done laparoscopically orhrough an umbilical incision (Figure 4). The advantage ofhe umbilical approach is that it can be done by any surgeon,nywhere in the world, regardless of ability or access toeonatal laparoscopy.14 The use of a preliminary biopsyoes not change the outcome of the transanal pull-throughn terms of times to feed, amount of pain, or length ofospital stay.15

rone vs lithotomy positionAdvocates of the prone position argue that the mesen-

eric vessels can be observed and controlled more effec-ively. Most pediatric surgeons are also comfortable withhe prone position, as the same position is used for the

ansition zone. (A) For children with a transition zone in the rectume transition zone is in the proximal sigmoid or descending colon,taking down the splenic flexure, is necessary. (C) For a transitiondown and the middle colic vessels divided, making sure that the

imal tr) If thithouttaken

osterior sagittal approach to anorectal malformations. The

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isadvantage of the prone position is that it does not permitccess to the abdominal cavity, unless the patient is subse-uently turned into the supine position. The lithotomy po-ition allows for preliminary biopsy, as well as mobilizationf the left colon or splenic flexure, and the creation of a

igure 4 An umbilical incision can be used to access the entirebdomen in a neonate. (A) A Hager dilator placed through the anus cane used to push the sigmoid colon out through the incision for prelimi-ary biopsies. (B) The splenic flexure can be mobilized, as can theepatic flexure in cases of long segment disease. (C) The late cosmeticppearance is excellent. (Color version of figure is available online.)

toma if necessary. l

se of retractorsThere are several options for everting the anus at the time

f the mucosal dissection. Many surgeons use an automaticetractor, such as the Lonestar, which uses multiple smallooks attached to a plastic ring. This provides excellentisualization of the anal canal and dentate line, and placinghe hooks just proximal to the dentate line effectivelyhides” the dentate line so that it cannot be injured. In smallabies, however, the Lonestar retractor can easily tear theragile tissue around the anus, and many surgeons prefer tolace sutures to Evert the anus.

Although some surgeons routinely use retractors in thenal sphincter during the mucosal dissection, this practice iso be condemned because it may cause damage to thephincter mechanism which can result in long-term incon-inence.16 A nasal speculum can be gently used to initiallyisualize the mucosa, without the need for stretching ofhe sphincter. During the dissection, the surgeon shouldse fine sutures on the proximal edge of the mucosa toull the bowel down, so that most of the dissection isone outside the anus without the need for retraction.ttention to these fine details is important so as to avoid

ong-term continence issues from sphincter injury duringhe operation.

ow high above dentate line should the dissectiontart

As a basic principle, the anastomosis should be lownough so that there is no significant residual aganglionicegment that may result in persistent obstructive symptomsfter the operation. By contrast, the anastomosis must beigh enough above the dentate line so that normal sensations not interfered with. The age and size of the child is also

factor, with most surgeons starting the dissection some-hat higher in older children than in neonates. Most opin-

ons range from 0.5 to 1.0 cm above the dentate line in aewborn, and 1.0-2.0 cm above the dentate line in an olderhild.17

ength of the rectal cuffIn the original descriptions of the transanal pull-through

rocedure, the mucosal dissection was carried to a pointbove the peritoneal reflection, to ensure that there was nonjury to pelvic structures. Using this technique, the rectaluff is quite long, and most authors advocate dividing orxcising a part of the cuff to prevent it from rolling downnd forming a constricting ring around the pull-throughowel. Although many surgeons continue to leave a longectal cuff, others have moved to a much shorter cuff.18 Inhis modification of the technique, a short mucosal dissec-ion is done for 1.0-3.0 cm, and the rectal wall is thenncised circumferentially. With a very short cuff, the muscleoes not need to be incised in most cases. Some surgeonsave eliminated the mucosal dissection entirely, and per-ormed a transanal Swenson procedure.19 The advantage of
eaving a short cuff or no cuff is the avoidance of a con-

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tricting ring or residual aganglionic bowel, with a lowerisk of obstruction and enterocolitis.15 The disadvantage ishat dissection on the outside of the rectum deep in theelvis may increase the risk of injury to pelvic nerves andessels, and to the prostate, urethra, or vagina.

ow much of the ganglionated bowel should beesected

The bowel is divided and the anastomosis performedsing colon which has been proven to contain ganglion cellsy frozen section, either before beginning the anal dissec-ion or during the transanal resection of the colon. Often thisanglionated bowel is grossly dilated or thickened, andome surgeons choose to resect it back to more normal-ppearing bowel. This decision is based on the theory thatilated bowel does not have normal motility, and will notunction as well as the bowel that has a more normal caliber.n addition, it has been shown that the transition zone is notymmetric around the circumference of the colon. Resectingome of the bowel proximal to the positive biopsy thereforensures that the transition zone will not be used in thenastomosis.20 Recommendations in the published dataange from 1-2 cm to 10-15 cm above the normal biopsy.21

ostoperative care

ntestinal activity is usually normal after a transanal pull-hrough procedure, and most infants have bowel movementsuring the first 24 hours after surgery. Oral feeding can betarted as soon as the child starts to pass stools, assuminghat the abdomen is not distended. Most children do notequire narcotics for pain, as long as regional anesthesia haseen used (a caudal block and infiltration of the umbilical oraparoscopic wounds). Oral acetaminophen is sufficient inost cases.At least 50% of children develop perianal dermatitis

ecause of frequent bowel movements and liquid dischargeuring the initial months after a transanal pull-through op-ration. It is important to prevent this as much as possible bymmediate application of barrier creams, and in some casesntidiarrhea medication. Both the frequency of stools andhe perineal excoriation usually settle down within severaleeks to months postoperatively.There is a controversy about the need for daily rectal

ilatations after a transanal pull-through procedure. Mosturgeons wait 1-2 weeks, and then calibrate the anastomosisith a dilator or finger, depending on the size of the child.ome then teach the parents to do daily dilatations for aaximum of 6 months postoperatively. Others see the childeekly for 6 weeks, calibrating the anastomosis each week,

nd proceeding to daily dilatations only if there is evidencef anastomotic or cuff narrowing.

The most important and dangerous complication after aull-through procedure is enterocolitis, because it is the
ost common cause of death in children with Hirschsprung c
isease.22 It is extremely important that both the parents andhe community pediatrician responsible for the follow-up ofhe patient be educated as to the signs and symptoms ofnterocolitis, and that they bring the child to medical atten-ion early if any of those clinical features occur. Manyreventive measures have been described, including routineostoperative irrigations or rectal stimulation,23 the use ofntibiotics, such as metronidazole, and the use of probiot-cs.24 The latter is currently the subject of a prospectiveandomized trial.

nvestigation and management ofostoperative problems

ll children with Hirschsprung disease are at risk for post-perative incontinence, enterocolitis, and obstructive symp-oms, regardless of which operation is performed. Everyhild should therefore be followed up on a regular basisntil at least the age of 5 years, or longer if they are stillaving problems at that point. The management of inconti-ence and enterocolitis are dealt with in other contributionsn this issue; this section will outline the investigation andanagement of the child who has persistent obstructive

ymptoms after a transanal pull-through procedure.

efinition and incidence of obstructive symptoms

bstructive symptoms may take the form of abdominalistension, bloating, borborygmi, vomiting, or ongoing se-ere constipation. Some children will also have fever withhese episodes, suggesting that an element of enterocolitisay be present. Many children with obstructive symptomsill not pass stools without assistance, using either rectal

timulation or irrigations, and when this is done the stoolsre often foul-smelling, “squirty,” or explosive in nature.any of these children have postoperative symptoms that

re identical to their initial presenting symptoms. The tim-ng of obstructive symptoms also varies. In some cases, thehild will have a good response to the surgical procedurend then develop obstructive symptoms later, and in otherases the child may not have any improvement in the post-perative period.

The incidence of obstructive symptoms varies in theublished data, depending on how they are defined and howlosely the authors are looking for them. Most of the oldereries probably underestimated the incidence. Recent serieseport an incidence of 8%-30%.25 Obstructive symptomsre probably more common in children with Down syn-rome. Children with long-segment disease seem to have aigher incidence of distension, vomiting, and enterocolitis,ut a lower incidence of constipation. Obstructive symp-oms may also be associated with incontinence in some
hildren.

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auses of obstructive symptoms

he 5 major reasons for persistent obstructive symptomsfter a pull-through procedure are listed in Table 1. Thesenclude mechanical obstruction, recurrent or acquired agan-lionosis, disordered motility in the proximal colon or smallowel, internal sphincter achalasia, or functional megacolonaused by stool-holding behavior.26

echanical obstructionMechanical obstruction may be the result of an anasto-

otic stricture (Figure 5). It may also be due to “rollingown” of the rectal muscular cuff after a transanal Soaverocedure. This may occur even if the cuff has been divided,nd is less likely if a short cuff is made.15 These complica-ions are identifiable by a combination of digital rectalxamination and contrast enema.

Many strictures and cuff-related narrowing can be man-ged using repeated dilatations, particularly if the problems identified early in the postoperative period. If dilatationrom below is impossible because the problem is too high oroo narrow, an antegrade approach using Tucker dilatorsay permit safer and more effective dilatation;27 however,

his approach requires the creation of a colostomy. If dila-ation is unsuccessful, the child may require revision of theull-through operation.28

Occasionally, the pulled-through bowel can becomewisted during the operation, which will result in partial oromplete obstruction (Figure 6). This is usually recognized

Table 1 Causes of obstructive symptoms after a pull-through for Hirschsprung disease

Mechanical obstructionRecurrent or acquired aganglionosisMotility disorder in the proximal bowelInternal sphincter achalasiaFunctional megacolon

Figure 5 Revisional surgery using the Duhamel technique for the tr

arly postoperatively, and usually requires surgical revision.bstructive symptoms can also be caused by small bowelbstruction because of peritoneal adhesions, and can occurany years after the initial surgical procedure. This com-

lication appears to be more common after open pull-hrough operations than after a transanal or laparoscopicrocedure. The diagnosis can be made based on observingilated bowel loops with air-fluid levels on plain abdominaladiographs, and in some cases where the obstruction isntermittent or partial, a contrast study with small bowelollow-through may be helpful.

ersistent or acquired aganglionosisSome children may develop persistent obstructive symp-

oms as a result of aganglionosis after a pull-through pro-

igure 6 Contrast enema showing twisting of the colon after aransanal pull through. This complication almost always requiresevision of the pull through.

eatment of a recalcitrant stricture after a transanal pull-through.

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edure. This may be due to pathologist error,29 or a pull-hrough which has been done using the transition zoneather than normally innervated bowel. It has been shownhat the transition zone is often asymmetrical,20 and mosturgeons advocate removing at least several centimeters ofowel proximal to their positive biopsy to avoid this prob-em. In other children, there are clearly normal ganglionells at the proximal resection margin, but the child subse-uently “loses” the ganglion cells.30 Some children mayave acquired aganglionosis associated with a stricture be-ause of poor blood supply and/or tension at the initialnastomosis. Although this problem is relatively rare, it ismperative to perform a biopsy of the pulled-through bowelegment above the anastomosis to determine whether therere normal ganglion cells present in any child with persis-ent obstructive symptoms.

Most children with persistent or acquired aganglionosishould undergo a repeat pull-through procedure. It is im-ortant to document the level of aganglionosis first at lap-rotomy or using a laparoscopic approach. The pull-throughperation can be done using a Swenson, Soave, or Duhamelpproach, either open, laparoscopically, or transanally.31

ome authors have advocated simple anal sphincter myec-omy for this problem, especially if the aganglionic segments short.

otility disorderIt is well recognized that children with Hirschsprung

isease may have associated motility disorders in the prox-mal bowel that contains ganglion cells. This problem maye either focal (usually involving the left colon) or diffuse.n some cases the abnormal motility may be associated withistologic abnormalities, such as intestinal neuronal dyspla-ia, and in some children the histology may be completelyormal. In children who have been shown not to have aechanical obstruction and who have normal ganglion cells

n rectal biopsy, investigations for motility disorders shoulde undertaken. Options include a radio-opaque marker tran-it study, nuclear colonic motility scan,32 colonic manom-try,33 and laparoscopic full-thickness biopsies looking forntestinal neuronal dysplasia.34 In children who have ahronically distended or thickened colon, investigations todentify a motility disorder may produce false positive re-ults. In this situation, it is usually best to do a defunctioningtoma initially, and to do the motility investigation after ateast 6 months of decompression.

If a focal motility disorder is found, resection and aepeat pull-through procedure using normal bowel shoulde considered. If the abnormality is diffuse, there is no roleor resection, and the options include bowel management,rokinetic agents, or in severe cases, ileostomy.

nternal sphincter achalasiaAll children with Hirschsprung disease lack a recto-anal

nhibitory reflex, and therefore cannot relax their internal
nal sphincter. Most are able to overcome this nonrelaxation fi
nd achieve normal defecatory function, but in some chil-ren sphincter nonrelaxation may result in or contribute toersistent obstructive symptoms. The traditional treatmentor this was internal sphincterotomy or myectomy, which istill recommended by many surgeons.35 However, it is wellecognized that the obstructive symptoms related to internalphincter achalasia tend to improve over time. Becausepplying a permanent sphincter-injuring solution to thisemporary problem may be a suboptimal approach, someuthors have suggested the use of reversible techniques toelax the internal anal sphincter, including intrasphinctericotulinum toxin36 (Figure 7) or the application of topicalitric oxide.37 In many cases, repeated injection of botuli-um toxin or application of nitroglycerin paste is necessaryhile waiting for resolution of the problem, which usuallyccurs around the age of 5 years. Injection of intrasphinc-eric botulinum toxin is also very useful as a diagnostic test,ecause absence of a clinical response to botulinum toxin

igure 7 Injection of botulinum toxin into the anal sphincter forhe treatment of internal sphincter achalasia. The procedure is donender general anesthesia as an outpatient, and is effective inelieving the obstructive symptoms in more than half of the pa-ients. Injection can be repeated if necessary when the effect wearsff. Injection of botulinum toxin also serves as a diagnostic test toetermine whether nonrelaxation of the sphincter is an importantactor in causing the obstructive symptoms. (Color version of
gure is available online.)

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uggests that the sphincter is not the problem, and thatyectomy is likely to fail.

unctional megacolonThere remain a group of children who do not have an

dentifiable cause for their symptoms and who do not re-pond to surgical or chemical relaxation of the sphincter.ost of these children suffer from stool-holding behavior,

nd are best treated using a bowel management regimenonsisting of a combination of diet, laxatives, and behaviorodification, as well as support for the child and family. In

ome severe cases of obstructive symptoms, the child maye best served by use of a cecostomy38 and administrationf antegrade enemas, or by the creation of a proximal stoma.

An algorithm for the investigation and management ofhe child with obstructive symptoms is shown in Figure 8.

ong-term prognosis

espite the fact that postoperative problems, such as ob-tructive symptoms, enterocolitis and incontinence are rel-tively common, long-term follow-up studies would suggesthat most children with Hirschsprung disease overcomehese issues and do very well, regardless of the surgicalechnique used.39 Sexual function, social satisfaction, anduality of life all appear to be relatively normal in the vastajority of patients. It is too early to determine what effect

he single-stage pull-through procedure and the use of min-mal access surgical techniques, such as laparoscopy and theransanal approach, will have on the postoperative compli-

igure 8 Algorithm for the diagnosis and management of theisease. (Color version of figure is available online.)

ation rate and long-term outcome, but this is an important

uestion that will require close follow-up and analysis.lthough some authors initially raised concerns that the

ransanal pull-through procedure might be associated withn increased rate of incontinence and a higher stool fre-uency than the open Soave procedure,16 these concerns doot appear to have been borne out in a larger multicentertudy (Teitelbaum, personal communication). It is fair toay, however that strict attention to meticulous technique,nd in particular a focus on avoiding stretching of thephincter mechanism during the anal dissection, is crucial inptimizing long-term functional outcomes.

eferences

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