Lecturer of Pediatric Surgery, Tanta University, Tanta, Egypt, E-mail:
hassanh_30@hotmail.com
Annals of Pediatric Surgery, Vol 5, No 1, January 2009 PP 21-26 Original Article
One-Stage Transanal Endorectal Pull- through Procedure for Hirschsprungs Disease in Neonates Hussam S. Hassan Pediatric surgery unit, Departments of Surgery, Tanta University, Tanta, Egypt
Background/ Purpose: Traditionally, Hirschsprung's disease (HD) was treated in a staged procedure of colostomy, the definitive procedure then closure of colostomy. Eventually many pediatric surgeons became more interested in the one-stage approach, and results have been favorable when compared with a staged procedure. This study aims at evaluation of management of HD using one-stage transanal endorectal pull-through (TEPT) early in the neonatal period. Materials & Methods: A retrospective review of 42 cases operated in the neonatal period between 2003-2007 was done. Data collected from records included age, sex, diagnostic procedure, length of aganglionic segment, operative time, blood loss, length of resected segment, and post operative complications. Results: Twenty two males and 15 females were included in the study. The mean operative time was 80 min, mean blood loss was 20 mL, and mean hospital stay was 5 days. Perianal excoriation occurred in 11 cases, enterocolitis in 5 cases, cuff abscess in 2 cases and anastomotic stricture in 2 cases. Cases completed 3 years follow up showed complete continence in 83.3% of cases, while the remaining cases showed good resting sphincter tone and powerful squeeze pressure on manometry. Conclusion: One stage TEPT is both feasible and safe in the neonatal period. The mucosectomy, operative time, and intraoperative blood loss are favourable compared to previously published large series in older children. Likewise, the postoperative complications and the functional outcome are comparable to cases operated in infancy and childhood period.
Index Word: Hirschsprung's disease, neonatal period, transanal endorectal pullthrough.
INTRODUCTION irschsprung's Disease (HD) affects one in 5,000 newborns. The diagnosis often is suspected when a newborn fails to pass meconium in the first 48 hours of life, has abdominal distension and vomiting. 1
Surgical therapy for HD implies removal of aganglionic bowel and bringing of normally innervated intestine to the anus. Traditionally, this was achieved by creation of a colostomy followed by one of the pull-through procedures (Swenson, Duhamel, Soave), then colostomy closure in the same setting or later (2 or 3 stages). 2 Eventually many pediatric surgeons became more interested in the one- stage approach, and results have been favorable when compared with a staged procedure. 3, 4
One-stage pull-through has been noted to be particularly beneficial in neonates, due to the fact that the colon above the aganglionic segment is less dilated, and episodes of enterocolitis are less frequent, making dissection much easier. 5
Transanal endorectal pull-through (TEPT) represents the latest development in the concept of the minimally invasive surgery for HD; the novel H Hassan H..
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description of an entirely TEPT approach by De la Torre-Mondragon and Ortega-Salgado in 1998 6 was rapidly followed by similar promising early experiences from various centers. 7-9
This study aims at evaluation of the feasibility and safety of TEPT in the neonatal period with respect to early and late complications compared to the reported complications in previously published large series of patients treated later after neonatal period.
PATIENTS AND METHODS A retrospective review of the files of all patients with HD operated between June, 2003 and April, 2007 was done. The inclusion criteria included only patients with proven tissue diagnosis of HD, who were operated as one stage TEPT in the neonatal period. The exclusion criteria included older children or neonates with either severe enterocolitis or neglected bowel obstruction not responding to bowel decompression, or those who were referred to us after initial colostomy was done elsewhwere. Neonates with preoperative known long aganglionic segments (proximal to sigmoid colon) were excluded from this series. The diagnosis was made by contrast study and rectal biopsy. The feasibility of an entirely TEPT at the neonatal period was assessed by the level of the transitional zone and the general condition of the patient. If the radiologic transitional zone was at rectosigmoid or sigmoid colon, the patient was considered eligible for one stage TEPT. All patients had the following data extracted from records for further analysis: (1) Age and sex; (2) clinical presentation and investigations; (3) operative details including position of patient, the level of starting the submucosal dissection, degree of difficulties in submucosal dissection and/or colon mobilization, length of the remaining cuff, iatrogenic injury of any structure during surgery, length of the excised specimen, estimated blood loss and blood transfusion, conversion to laparotomy and its cause, and operating time; (4) early postoperative course particularly time at regain of peristalsis, timing of first passage of stool, and starting of oral feeding; (5) postoperative complications such as significant perineal excoriation, anastomotic leak, anorectal stricture, enterocolitis, and perineal or pelvic infection; (6) functional outcome as judged by bowel habits, recurrent abdominal distension, and anorectal continence status; and (7) need for a secondary surgical procedure such as myectomy, or redo pull- through. Postoperative investigations including contrast enema, anorectal motility, and electromyogram (EMG) were performed only for patients with complications or problems with bowel control. All cases had undergone one-stage TEPT. Preoperative bowel preparation of the colon using warm saline was done until effective decompression of the bowel was achieved. Surgical technique: After the induction of general endotracheal anesthesia and the placement of intravenous lines, the patient was given one of the third generation cephalosporin intravenously. Rectal irrigation was performed with a dilute solution of betadine. The patient was then placed supine with the pelvis elevated at the end of the operating table with the lower limbs attached to an inverted U-shaped bar. The abdomen and perineum were prepared in the standard fashion. A bladder catheter was not routinely inserted. The anal canal was exposed with Loan-Star anal retractor. Submucosal injection of epinephrine or saline was not routinely used. A circumferential row of 4-0 silk stay sutures was inserted approximately 0.5 to 1 cm above the dentate line. The rectal mucosa was incised just distal to the traction sutures and lifted circumferentially using fine diathermy needle to develop the submucosal plane. Once the submucosal plane was established, the dissection was easily continued proximally using blunt dissection and cauterization of submucosal infiltrating vessels. The traction on mucosal tube facilitated proximal extension of mucosal dissection until the level proximal to peritoneal reflection (approximately 10 to 15 cm above the dentate line). Four stay sutures were inserted to control the upper end of muscular cuff, which was incised circumferentially allowing exposure of the full-thickness sigmoid colon. Mobilization of the colon was continued as proximal to the grossly obvious or histologically confirmed transition zone as possible by dividing the rectosigmoid vessels after cauterizing them. Ligatures were rarely needed in neonates. The long seromuscular cuff was inverted outside the anus and shortened to less than 5 cm in length before returning it to its normal position. After resection of the aganglionic segment, the normally innervated bowel was pulled through the muscular cuff and anastomosed to the remaining mucosa above the Hassan H.
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dentate line using 4-0 slowly absorbable suture material. Feeding was allowed at first or second postoperative day. The patients were discharged 3-5 days after surgery if no complications occurred. First rectal digital examination was performed after 10 days. Routine anal dilations were performed in all neonates once or twice weekly for at least 3 weeks. Follow-up was arranged once weekly for 3 weeks followed by once monthly for 3 months then every 3 months thereafter.
RESULTS The study included 42 neonates; all of them were referred from paediatricians for evaluation of neonatal intestinal obstruction or constipation and distension.. There were 27 males and 15 females. Their age ranged from 2 weeks to 2 months. The level of aganglionosis was rectum, n = 11 (26.5%); rectosigmoid, n = 19 (45%); sigmoid, n = 12 (28.5%);
Operative details: All cases were operated as planned (TEPT). No case required conversion to laparotomy. The mean operating time was 80 minutes 23.5 (range, 75-100 minutes). Submucosal dissection and colon devascularization was easy, due to the fact that there is no enough time to have repeated episodes of enterocolitis, no long standing dilated hypertrophied colon and thickened mesentery. The average length of resected bowel was 20.8 12.4 cm (range, 15 to 30 cm). The average estimated intraoperative blood loss was 20 ml 2.4 mL (range, 20-25mL). No case required blood transfusion. The average time of the first passage of stools post operatively was 1 day. Feeding was allowed 3 days post operatively. The mean hospital stay was 7 days (5-10 days). There was no mortality in this series.
Early Postoperative morbidity: Significant transient perianal excoriation occurred in 11 cases (26%), which responded to medical management including the use of zinc oxide ointment, keeping the baby dry and frequent change of diapers, and use of constipating medication to decrease frequency of bowel motions. Enterocolitis after the pull-through was noted in 5 cases (11.9%). The clinical grade of enterocolitis was grade 1in 3 patients; grade 2 in 1 patients; and grade 3 in 1 patient; according to the grading system established by Elhalabyet al. 13 All patients with grade 1 enterocolitis were treated successfully as outpatients, whereas those of clinical grade 2 or 3 were hospitalized treated with colonic decompression and antibiotics. Two patients had cuff abscess; 1 required drainage under anaesthesia, the other one drained spontaneously after digital rectal examination.
Late Postoperative morbidity: Two patients (4.8%) had significant anastomotic stricture that necessitated dilatation at least once under general anesthesia. A tight sphincter was reported in another 4 patients (9.5%), but all of them responded adequately to anal dilatation. (table 2).
Table 1. Patients' characteristics. Characteristics No. of patients (%) Sex Male 27 (64%) Female 15 (36%) Transitional zone Rectum 11 (26.5%) Rectosigmoid 19 (45%) segmoid 12 (28.5%) Operative time (min) 80 23.5 Operative blood loss (mL) 20 2.4 Average Hospital stay (days) 5
Table 2. Post operative complications. Post operative Complications No. of patients (%)
Early postoperative (within10days) Perianal excoriation. Enterocolitis. Cuff abscess.
11 (26%) 5 (11.9%) 2 (4.8%)
Late postoperative Anastomotic stricture. Tight sphincter.
2 (4.8%) 4 (9.5%)
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Fig 1. Anal exposure using Loan Star retractor Fig 2. Traction stitches 2 cm above dentate line
Fig 3. Anorectal mucosectomy Fig 4. Circumferential division of the cuff and starting the full thickness mobilization of the colon
Fig 5. The seromuscular cuff is everted and then shortened to < 5 cm Fig 6. Coloanal anastomosis
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Follow up: The median follow up period was 20 months (range 5- 36 months). Five cases had no regular follow up. The mean stool frequency was 7 (6-9) at first month of follow up. This gradually improved to average of 5 times at 3 months post operatively, then to average of 3 times at 1 year of follow up period. Twelve cases completed 3 years follow up). Complete anorectal continence was confirmed in 10 of those 12 children (83.3%), whereas soiling still occur in 2 patients who continue to show a steady improvement of their continence status. Anorectal motility and EMG mapping of the anal sphincter of those 2 patients showed good resting tone and powerful squeeze pressure.
DISCUSSION The aim of this study was to evaluate the feasibility of one-stage TEPT procedure in the neonatal age group, with special emphasis on the operative time, difficulty of mucosal dissection and colon mobilization, blood loss, post operative complications. The age of patients included in the study ranged from 2 weeks to 2 months. All cases were referred to us by the neonatology unit; with whom we conducted seminar to discuss the merits of performing surgery early in neonatal period once the final diagnosis is made . When cases were referred to us, a Barium enema was done. In many cases, the characteristic features of HD were evident as early as 2 weeks of age. In all cases the diagnosis was established by rectal biopsy. The mean operative time in this series (80 min) is shorter than in other series including older ages. 10, 11
This is because mucosal dissection is much easier in neonatal age due to the presence of less adherent mucosa (no repeated attacks of enterocolitis), and the mesentery and colon are less substantial and more easy to mobilize. The average amount of intra operative bleeding was 20 ml 2.4 mL. This amount is less than reported in literature series for older infants and children. 12 This is explained by the fact that dissection is easier in neonates due to the same factors described above. In all cases, the seromuscular cuff was shortened to become less than 5 cm to avoid stenosis and recurrence of obstructive symptoms, which may predisposes to enterocolitis. There was no case that had to be converted from TEPT to laparotomy. This is because cases were selected in this series when they have a clear transitional zone not further than the sigmoid colon. Enterocolitis has been considered one of the main problems in patients with HD both before and after definitive treatment. 13,14 The incidence of postpull- through enterocolitis reported in the literature varies widely, with some studies reporting rates as high as 32% to 42%. 15,16 Hackman et al 15 studied the risk factors for postoperative enterocolitis and found that both the presence of anastomotic leak or stricture and the development of postoperative intestinal obstruction secondary to adhesions increased the relative risk and subsequent enterocolitis by approximately 3-fold. The relative low incidence of enterocolitis after one stage TEPT in the current series may be related in part to the short seromuscular cuff, the low coloanal anastomosis, and the policy of routine postoperative anal dilatation. There is a general tendency to reserve anal dilation or bouginage to cases with existing or potential risk of stricture formation. 7 We believe that postoperative routine anorectal bouginage is an effective tool to prevent the occurrence of anal stricture and to decrease both the frequency as well as the severity of enterocolitis particularly in neonates and young infants. Cuff abscess occurred in 2 (4.8%) patients. This complication occurs when the mucosal tube is damaged during dissection, when there is retraction of the anastomosis, and when there is poor blood supply to the pull-through colon. These events cause spillage of intestinal contents to surrounding tissues, which initiate an inflammatory process that culminates in abscess formation. This incidence is less than reported in some series, 17 perhaps due to the extra care taken during dissection to avoid mucosal injury as possible and avoiding anastomosis under tension by avoiding excessive traction on the colon before excision and anastomosis (to avoid upwards retraction of the colon pulling on the anastomosis). These precautions also are responsible for not having any case of anastomotic leak. Although every effort was done to avoid anastomosis under tension, and meticulous coloanal anastomosis to avoid leakage, we had two cases (4.8%) of anastomotic stricture. The two cases, however, responded to repeated anal dilatation. Regarding to the concern that delicate structures such as the muscular sphincters may be over stretched and injured during TEPT, 83% of cases who completed 36 months follow up had complete anorectal continence. Anorectal motility and EMG mapping of the anal Hassan H..
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sphincter of the remaining 17% of patients showed good resting tone and powerful squeeze pressure. These results are the same as in children operated later in life and those whom operated by other techniques than TEPT 18
CONCLUSION We conclude that one stage TEPT is feasible in the neonatal period. Operative time and blood loss are less than in published series of older children. Post operative complications occur at similar rate to cases operated in infancy and childhood period. Continence results are similar to cases operated later in life using techniques other than TEPT. We recommend early operation, in the neonatal period, for cases of HD. We also recommend good communication with the paediatricians to speed up the diagnostic workup and to convince them about the potential benefits and limitation of one stage surgery at neonatal period.
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