Stooling patterns and colonic motility after transanal one-stage pull-through operation for Hirschsprung's disease in children

Background

The transanal one-stage endorectal pull-through operation for Hirschsprung's disease is relatively new and makes assessment of the functional outcome and colonic motility difficult. The aim of this study was to evaluate the stooling patterns and colonic motility after a one-stage transanal pull-through operation for Hirschsprung's disease in children.

Methods

Twenty-two children who underwent a one-stage transanal pull-through operation for Hirschsprung's disease were followed up for at least 6 months. The children (17 boys and 5 girls) were from 12 months to 13 years of age (mean age, 4 years). All patients had an aganglionic segment confined to the rectosigmoid area (confirmed by preoperative barium enema and postoperative histology). Clinical outcome was assessed by interviews and questionnaires, and children were divided into symptomatic and nonsymptomatic groups. Contrast barium enema and defecography and determination of total and segmental colonic transit time (using radio-opaque markers) were performed on all 22 children.

Results

The stooling patterns were considered satisfactory in 17 children. Of all the children, the mean stool times were 1 to 2 per day and only 2 were 8 to 10 per day; postoperative soiling was found in 4, constipation was observed in 2, and Hirschsprung-associated enterocolitis in 1. There was no incontinence, cuff infection, anastomotic leak, or mortality noted. Barium enema showed that the dilated and spastic colonic segment disappeared in all 22 children. The dilated sigmoid loops decreased in 17 (2 symptomatic, 15 nonsymptomatic) and disappeared in 5 (4 symptomatic, 1 nonsymptomatic). There was a significant difference between the decreasing and disappearing loop group in regard to stooling disorders (P < .05). Postoperative defecography showed that the anorectal angle of all children was open, fixed, and significantly larger than that of the preoperative and control groups (123.3° ± 15.1° vs 84.7° ± 8.3° vs 79.0° ± 11.6°, P < .01) and larger in the symptomatic group when compared with the nonsymptomatic group (135.6° ± 15.9° vs 111.0° ± 14.3°, P < .05). Postoperatively, the total gastrointestinal transit time, left colonic transit time, and rectosigmoid colonic transit time of all the children were shorter than preoperatively (26.8 ± 8.2 vs >188 hours, P < .01; 6.3 ± 4.1 vs >60 hours, P < .01; 11.8 ± 4.4 vs >120 hours, P < .01) and similar to controls. The total gastrointestinal transit time and rectosigmoid colonic transit time of the symptomatic group were significantly shorter than the nonsymptomatic group (25.2 ± 5.6 vs 28.1 ± 10.1 hours, P < .05; 12.2 ± 6.7 vs 9.8 ± 4.0 hours, P < .05).

Conclusions

The stooling pattern and colonic motility are satisfactory in most children after the one-stage transanal pull-through operation for Hirschsprung's disease. Normalization of colon appearance and total and segmental colonic transit time are signs of recovery of colonic motility. Stooling disorders were noted in a few cases and may be related to decrease or disappearance of the sigmoid loop, dysfunction of the “neorectosigmoid”, an open and fixed anorectal angle, and ischemia of the pull-through segment.     

Postoperative functional and manometric evaluation of patients with Hirschsprung's disease

Sixty-two patients with Hirschsprung's disease (operated on according to Duhamel, Swenson, or Soave) were evaluated for quality of defecation and studied manometrically. Follow-up was 30 years. Stools were normal in 30% to 50% of patients, while the rest had either constipation, loose, or increased frequency of stools per day. Continence was complete in 30% to 60%. Enterocolitis was seen in 12 patients preoperatively and in 20 patients postoperatively, regardless of type of operative procedure. This complication improved with age. To correct some of these problems, 18 had post pull-through internal sphincter myectomy with 50% improvement. Manometrically, 50% to 70% experienced a sensation of fullness and an urge to defecate following rectal balloon inflation. The resting external sphincter pressure was high in the majority of patients and further increased following rectal balloon inflation. The internal sphincter pressure showed a normal anorectal reflex in only 10% of patients postoperatively and did not seem to be related to clinical fecal continence. We concluded from the study that an appreciable number of patients in this select group with Hirschsprung's disease suffered from a variety of stooling disorders postoperatively. The majority of these complications were managed medically and/or socially but some were quite incapacitating.     

Clinical outcome of laparoscopically assisted endorectal pull-through in Hirschsprung's disease: comparison of abdominal and perineal approaches

Background/Purpose

Laparoscopically assisted endorectal pull-through (EPT) via a perineal approach using a prolapsing technique (PA) for Hirschsprung’s disease (HD) has been reported. However, the clinical outcome after this approach has not been reported. The purpose of this study was to compare the clinical outcome of PA and the conventional transabdominal approach (TA).

Methods

In the period between 1990 and 2001, 20 cases of HD underwent EPT with TA (group O), and 21 underwent EPT with PA (group L). There was no difference in age and weight distribution between the 2 groups. Clinical outcome was assessed 3 years after surgery.

Results

The operation time was comparable in the 2 groups (4.9 ± 0.8 v 5.2 ± 0.8 hr), whereas blood loss (98 ± 52 v 36 ± 30 mL) and postoperative complications requiring surgical intervention (26% v 0%) were significantly lower in group L. The incidence of postoperative enteritis (27% v 28%) and voluntary defecation (more than once every/2 days) were compatible in the 2 groups (70% v 87%). Soiling (small amount of involuntary stooling; >1 per month) was significantly less frequent in group L (45% v 14%).

Conclusions

Laparoscopically assisted ETP with PA is less invasive and can provide a better clinical outcome compared with TA in terms of postoperative soiling.     

Anorectal manometric evaluation of children and adolescents postsurgery for Hirschsprung's disease

Background: Little is known about anorectal function in patients after surgery for Hirschsprung's disease. Therefore, the authors evaluated anorectal sphincter function after corrective surgery. Methods: Thirty-five patients were studied after corrective surgery for Hirschsprung's disease. Sixteen of them had anorectal manometry performed also before surgery. The clinical outcome, the highest anal resting pressure, the presence of the rectosphincteric reflex (RSR), and of high amplitude propulsive waves were evaluated. Results: A total of 89% were doing poorly. Three had a fair and only one had a good outcome at the time of manometry 4.4 years after corrective surgery. RSR was absent in 33 and abnormal in 2 patients. The preoperative anal resting pressure was 45 [plusmn] 14 mm Hg and postoperatively 45 [plusmn] 13 mm Hg (P [gt ] .3). The anal resting pressure was 44 [plusmn] 16 mm Hg in the 19 patients evaluated only postoperatively. Propulsive waves [ge ]50 mm Hg were present in 60% of patients. Conclusions: Four years postsurgery, patients had a persistent absence of RSR regardless of the type of surgery. There was no significant difference in anal resting pressure between patients with and without sphincterotomy or between different surgical procedures. The presence of propulsive waves was not a prognostic indicator for achieving bowel control. J Pediatr Surg 38:191-195.     

Modified transanal rectosigmoidectomy for Hirschsprung's disease: clinical and manometric results in the initial 20 cases

Purpose

The authors describe a modified technique of primary transanal rectosigmoidectomy for Hirschsprung’s disease (HD), using a Swenson like procedure to perform the anastomosis between the colon and the rectum, and the preliminary results from this in children.

Methods

Twenty children, of whom, 90% were boys and 10% girls, 50% white and 50% nonwhite, aged 15 days to 10 years and with HD proven via biopsy, underwent a transanal pull-through procedure over a 29-month period. Postoperative follow-up ranged from 29 to 5 months. The proximal cut edge of the mucosal and submucosal cuff was tagged with multiple polypropylene 4-0 sutures, which were used for traction of the intestinal layers outside. The rectal mucosa was incised circumferentially using cautery, to perform rectal dissection approximately 1.5 cm from the dentate line, except in newborn case, in which the proximal cut edge was 0.5 cm from the dentate line. The dissection extended in an upward direction around the entire rectal circumference as far as the opening of the peritoneal reflection. The full thickness of rectum and sigmoid were mobilized outside through the anus, with division and coagulation of the rectal and sigmoid vessels using cautery or ligatures with cotton 4-0. The dissected colon then was divided above the transition zone, which was confirmed via full-thickness biopsy sections and with frozen section confirmation of ganglion cell presence. The authors performed a modified Swenson anastomosis technique, using a seromuscular polyglactin 4-0 separate-stitch suture. No drains were used.

Results

Normal bowel movements were displayed by all patients at the follow-up. All patients underwent a defecogram and anorectal computerized manometry at 3 months after surgery that showed an absence of stenosis and good anorectal sphincter muscle complex function. The incidence of complications in our series was 10%.

Conclusions

During the follow-up period of 29 months, all patients had normal bowel movements and normal anorectal manometric pressure profiles.     

A manometric assessment of anorectal pressures and its significance in the diagnosis of Hirschsprung's disease and idiopathic megacolon

Anorectal manometric studies were performed on nine patients with Hirschsprung's disease and ten patients with idiopathic megacolon for the purpose of differentiation, comparing with those of the normal subjects. The anorectal reflex was absent in all nine of the patients with Hirschsprung's disease while in all of ten patients with idiopathic megacolon rectal distension produced a relaxation in the anal canal like that of the normal subjects. Manometric studies proved to be a reliable and noninvasive technique for the diagnosis of Hirschsprung's disease and were of particular value as simple screening tests in patients with a clinical suggestion of this disease. The manometric studies of the patients with Hirschsprung's disease or idiopathic megacolon in the present series also demonstrated that the pronounced internal sphincter contraction combined with a total absence of reflex relaxation or an inadequate response of the sphincter to rectal distension might be responsible for the obstructive symptoms in these diseases.     

Long-term outcome after Hirschsprung's disease: patients' perspectives.

BACKGROUND: The results of treatment of Hirschsprung's disease (HD) are not uniformly successful, and the parents' and child's interpretation of the outcome may be different from that of the surgeon. METHODS: One hundred seven children treated for HD over 22 1/2 years were reviewed retrospectively. Adequate clinical data regarding functional outcomes were available in 78, of which, an additional questionnaire was completed by 69. Follow-ups were divided into type of pull-through (PT), age at time of PT (< 4 months and > 4 months) and age at last follow-up (< 5 years, 5 to 15 years and > 15 years). Degrees of constipation and incontinence were determined using standard scoring systems. RESULTS: The median age at presentation was 9 days (range, 1 day to 9.4 years), and 41% presented within the first 72 hours of life. Aganglionosis extended to the rectosigmoid region in 75%, proximal to the splenic flexure in 11%, and total colon in 6.5%. Treatments included a Soave procedure in 57, Duhamel in 31, Swenson in 10, and sphincterotomy-myectomy in two. No surgical treatment or only a decompressing enterostomy was performed in two and three children, respectively, and two underwent a PT elsewhere. Complications related to the enterostomy occurred in 39 patients. Postoperative enterocolitis and bowel obstructions occurred in nine and 13 patients, respectively. Staple-line fusion, anastomotic stenosis, and anastomotic leak occurred in nine, 17, and two patients, respectively. Seven children died, only one directly related to the PT. Aside from more constipation associated with the Duhamel procedure, functional outcomes were not significantly different among the types of PTs. No differences were found between patients who underwent a PT at less than 4 months of age and those at greater than 4 months of age. Significantly, however, fecal continence was relatively poor in those less than 15 years of age (50% with only fair to poor continence), but improved markedly once the child reached later adolescence (8%, P < .0002). Correspondingly, the negative impact on the child's social life was much greater in the younger age groups compared with later adolescence (50% of those aged 5 to 15 years v 18% of those > 15 years; P = .007). The effect on the families' lives mirrored this. Finally, only 64% of patients were interpreted as having "normal" stooling habits, yet 90% of parents were moderately or very satisfied with their child's outcome, with no differences found among the three sets of comparison groups. CONCLUSIONS: The outcomes for HD are not always as good as surgeons may perceive; long term follow-up is important. With time, most children significantly improve with respect to fecal continence, but this may not be until later adolescence. In the meantime, the impacts on their social and family lives may be significant. Despite high complication rates, often poor continence, and relative infrequency of normal stooling habits, most parents are satisfied with their child's outcome and adapt to their functional abnormalities along with them.     

Single-stage versus multi-stage pull-through for Hirschsprung's disease: Practice trends and outcomes in infants

PurposeThe aim of this study was to evaluate surgical treatments and outcomes in a multi-institutional cohort of neonates with Hirschsprung's disease (HD).MethodsUsing the Pediatric Health Information System (PHIS) from 1999 to 2009, neonates diagnosed with HD were identified and classified as having a single stage pull-through (SSPT) or multi-stage pull-through (MSPT). Diagnosis and classification algorithms and clinical variables and outcomes were validated by multi-institutional chart review. Groups were compared using logistic regression modeling and propensity-score matched analysis to account for baseline differences between groups.Results1555 neonates with HD were identified; 77.2% underwent SSPT and 22.8% underwent MSPT. Misclassification of disease or surgical treatment was < 2%. Rates of SSPT increased over time (p = 0.03). Compared to SSPT, patients undergoing MSPT had significantly lower birth weights and higher rates of prematurity, non-HD gastrointestinal anomalies, enterocolitis, and preoperative mechanical ventilation. Patients undergoing MSPT had significantly higher rates of readmissions (58.5 vs. 37.9%) and additional operations (38.7 vs. 26%). Results were consistent in the propensity-score matched analysis.ConclusionMost neonates with HD undergo SSPT. In patients with similar observed baseline characteristics, MSPT was associated with worse outcomes suggesting that some infants currently selected to undergo MSPT may have better outcomes with SSPT. However, there remains a subgroup of MSPT patients who were too ill to be adequately compared to SSPT patients; for this subgroup of severely ill infants with HD, MSPT may be the best option.     

Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung's disease

Background/Purpose: In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and retained aganglionosis after a previous operation. Duhamel or Swenson method is used commonly for redo operations. The pelvic dissection may be difficult, especially in Swenson's type of operation, because of fibrosis resulting from previous surgery or its complications. To overcome this, the authors used a combined abdominal and posterior sagittal approach to perform redo pull-through of Swenson's type in 4 children. Methods: Four boys (2.5 to 12 years) underwent redo pull-through for failed endorectal pull through (n = 2), persistent symptoms after 2 myectomies (n = 1) and late anastomotic disruption after Swenson's PT (n = 1). Abdominal dissection was done first to mobilize colon and resect aganglionic segment as far as the mid pelvis. The mobilized ganglionic colon was tacked to the pelvic rectal stump, hemostasis checked, and the abdomen closed. The lower pelvic dissection was done through the posterior sagittal route, under direct vision. The remainder of diseased rectum was excised, and the pull-through colon was retrieved and anastomosed to the anal stump. No covering colostomy was done. Results: A rectocutaneous fistula developed in one patient, which healed spontaneously. All patients had increased stool frequency in the early postoperative period but improved with time. All patients have attained normal voluntary bowel actions, but one child has infrequent minor soiling. There was no anastomotic narrowing in any case. Conclusions: Posterior sagittal approach is a useful alternative in difficult redo pull-through surgery. It offers excellent exposure, precise dissection, and direct anastomosis. There are minimal chances of complications, and continence is retained. J Pediatr Surg 37:1156-1159.     

A manometric assessment of anorectal pressures and its significance in the diagnosis of Hirschsprung's disease and idiopathic megacolon.

Anorectal manometric studies were performed on nine patients with Hirschsprung's disease and ten patients with idiopathic megacolon for the purpose of differentiation, comparing with those of the normal subjects. The anorectal reflex was absent in all nine of the patients with Hirschsprung's disease while in all of ten patients with idiopathic megacolon rectal distension produced a relaxation in the anal canal like that of the normal subjects. Manometric studies proved to be a reliable and non-invasive technique for the diagnosis of Hirschsprung's disease and were of particular value as simple screening tests in patients with a clinical suggestion of this disease. The manometric studies of the patients with Hirschsprung's disease or idiopathic megacolon in the present series also demonstrated that the pronounced internal sphincter contraction combined with a total absence of reflex relaxation or an inadequate response of the sphincter to rectal distension might be responsible for the obstructive symptoms in these diseases.     

婴幼儿经肛门巨结肠根治术后直肠肛管三维向量测压的研究

目的探讨经肛门巨结肠根治术后直肠肛管三维向量测压改变,以评价其肛门括约肌功能。方法对58例在婴幼儿期接受经肛门巨结肠根治术儿童进行随访,平均随访时间为15．8个月,全部为普通型巨结肠。对所有患儿进行排便功能问卷调查,根据临床症状分为无症状组和有症状组,并进行肛门括约肌三维重建,做肛管最大压力、向量容积、对称指数和直肠肛管反射检查。结果临床问卷调查显示58例均有便意,54例排便次数平均为1～2次／d,仅4例8～10次／d,9例污便, 5例便秘,2例有小肠结肠炎,无便失禁。直肠肛管反射3例阳性,2例弱阳性。便秘组的最大静息压 (mmHg)明显高于无症状组和对照组[(167±36)比(157±47)比(152±33),P<0．05;(211±36)比 (200±65)比(190±38),P<0．05]。污便组向量容积(cm×cmHg2)和对称指数较术前及对照组显著降低[(381±109)比(520±254)比(662±31),P<0．05;(0．69±0．32)比(0．75±0．19)比(0．70± 0．07),P<0．05]。便秘组对称指数高于对照组[(0．74±0．02)比(0．70±0．07),P<0．05]。结论婴幼儿经肛门巨结肠根治术后肛门括约肌功能良好。少数病例排便功能障碍的发生可能与术后“新直肠”储便功能代偿不全及(或)肛门括约肌痉挛失迟缓或损伤修复有关。     

经肛门巨结肠根治切除术后结肠形态及功能的初步评价

目的探讨经肛门巨结肠根治术后结肠形态及功能的变化。方法对58例经肛门巨结肠根治术后儿童进行排便功能问卷调查、钡灌肠X线和结肠传输时间检查,评价其结肠形态及功能的变化。结果本组58例儿童均有便意,54例排便良好,4例便频或稀便,9例污便,5例便秘, 2例患有小肠结肠炎,无便失禁。钡灌肠X线显示结肠形态恢复良好,乙状结肠迂曲减少或消失,与切除肠段的长短相关。全部病例术后直肠肛管角(度)较术前及对照组显著开大[(123±15)°比 (85±8)°比(79±12)°,P<0．01],有症状组更为显著[(136±16)°比(111±14)°,P<0．05]。全部病例术后全结肠传输时间较术前均明显缩短[(27±8)h比>168 h,P<0．01],与对照组比较略有降低但不显著[(27±8)h比(29±8)h,P>0．05],有症状组显著缩短[(25±6)h比(29±8)h,P< 0．05]。结论经肛门巨结肠根治术后结肠功能良好。少数患儿排便功能障碍可能与术后乙状结肠曲减少或消失、“新直肠”储便功能代偿不全以及拖出结肠致直肠肛管角开大有关。     

One-stage Soave pull-through for Hirschsprung's disease: a comparison of the transanal and open approaches

PURPOSE: The authors reviewed their experience using the transanal Soave technique, to determine (1) if it offers any advantages over the standard open approach and (2) whether routine laparoscopic visualization is necessary. METHODS: The case reports of 37 consecutive children less than 3 years old undergoing Soave pull-through were reviewed. Patients were excluded from analysis if they had total colon disease or had a previous colostomy. The patients were divided into 3 groups: open Soave (OS, n = 13), transanal Soave with routine laparoscopic visualization (LVS, n = 9), and transanal Soave with selective laparoscopy or minilaparotomy (TAS, n = 15). Cost was calculated based on hospital stay, operating room time, and use of laparoscopic equipment. RESULTS: In the TAS group, suspicion of a longer segment led to the selective use of laparoscopy with or without biopsy in 2 children, and the use of a small umbilical incision for mobilization of the splenic flexure in 2. There were no differences among groups with respect to age, weight, gender, transition zone, operating time, blood loss, intraoperative complications, enterocolitis, or stricture or cuff narrowing. Hospital stay was significantly longer in the OS group (median, 7 days; range, 3 to 47) than the LVS (median, 1; range 1 to 6) or TAS (median, 1, range, 1 to 3) groups. Cost (in thousands of dollars) was also higher in the OS group (median, 6.9; range, 3.9-25.7) than the LVS (median, 3.9; range, 3.6 to 6.4) or TAS (median, 3.4; range, 2.2 to 9.4) groups. Repeat surgery was necessary for 4 OS patients: 2 adhesive small bowel obstructions (1 of whom died), 1 twisted pull-through, and 1 recurrent aganglionosis. Three TAS patients required repeat surgery: 1 twisted pull-through, 1 anastomotic leak, and 1 cuff narrowing. CONCLUSIONS: These data suggest that the transanal pull-through is associated with a significantly shorter hospital stay and lower cost than the open approach, without an increased risk of complications. Because there is no intraabdominal dissection, there probably is a lower incidence of adhesive bowel obstruction. Routine laparoscopic visualization or minilaparotomy is not necessary but should be used in children who are at higher risk for long segment disease.     

Hirschsprung's disease: Progress in management and diagnostics

Each of the basic operative methods for Hirschsprung's disease (the rectosigmoidectomy developed by Swenson, the retrorectal transanal approach developed by Duhamel, and the endorectal procedure developed by Soave and modified by Denda and Boley) can give good results. A protective colostomy should be routine. If only an occasional case is prevented from progressing to incontinence or septicemia and death, then I think this outweighs the hazards of the colostomy. The 25-year review of 84 of the first 87 Swenson-type operations carried out at our hospital revealed that in adult life every patient is continent even though some had a degree of lack of control for a considerable time during childhood. A follow-up of the group of Soave-type operations from our clinic, followed between 10 and 17 years, also showed maintenance of normalcy. Our first Duhamel operations were carried out in 1958 and these patients also maintained control. From the technical point of view, the remaining problem is that of the infant with a segment extending significantly proximally into the small intestine. Kimura's operation may prove to be the answer, although the rarest condition of total intestinal aganglionosis seems likely to remain insuperable and is mainly of clinical importance because of evidence that it recurs frequently in the family. Bodian preferred to call this condition agenesis of the intramural plexuses since there was no evidence of increased nerve bundles found in congenital aganglionosis. Enterocolitis remains the main threat in management. The urgency of treatment needs wider recognition. Hirschsprung's disease should be diagnosable almost entirely in the neonatal period now that reliable and minimally disturbing rectal suction or punch biopsy is available. It must be accepted, however, that this diagnostic tool requires an expert and experienced pathologist and means, therefore, some centralization of the examination of the material even if not always of the baby itself. The finding of overt neuropathological conditions in the presence of ganglia makes it likely that more subtle variations also exist and their recognition might account for some poor results of operation which are not explained by technical errors. The difficult case, and in particular the child whose superficial biopsy is reported ganglionic but not normal, should be investigated by full-thickness biopsy studied by such special techniques as histochemical and silver staining, electron microscopy, and perhaps hormonal immunofluorescent studies as indicated. Nevertheless, it appears that these conditions are not sufficiently common to justify the recommendation of full-thickness biopsy under general anesthesia or the use of these elaborate studies as routine, whereas by the encouragement of early rectal suction biopsy diagnosis, the course of the vast proportion of children who have typical Hirschsprung's disease will be made simpler for the surgeon, the child, and the family.

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Resumen Cada uno de los procedimientos quirúrgicos básicos para la enfermedad de Hirschsprung (la rectosigmoidectomía desarrollada por Swenson, el abordaje retro-rectal transanal desarrollado por Duhamel y el procedimiento endo-rectal desarrollado por Soave y modificado por Denda y Boley) puede dar buenos resultados. La colostomía de protección debe ser rutinaria: si sólo se logra prevenir un caso ocasional de evolución hacia la incontinencia o hacia la septicemia y muerte, personalmente considero que ello sobrepasa las inconveniencias de la colostomía.Una revisión en el curso de 25 años de 84 de los primeros 87 procedimientos de tipo Swenson realizados en nuestro hospital reveló que todos los pacientes se encontraban continentes en la vida adulta, aún cuando algunos presentaron algún grado de falta de control por un tiempo considerable durante la niñez. El seguimiento del grupo de pacientes con operaciones del tipo Soave en nuestra clínica, en el curso de 10 a 17 años, también reveló mantenimiento de la normalidad funcional. Nuestra primera operación de Duhamel fué hecha en 1958, y estos pacientes igualmente han demostrado control de su función.Desde el punto de vista técnico nos queda el problema del infante con un segmento anormal que se extiende por una longitud significativa en el intestino delgado. La operación de Kimura puede llegar a ser la solución, aun cuando es probable que la más rara condición clínica, la aganglionosis intestinal total, continúe siendo terapéuticamente insuperable y sólo de importancia clínica debido a que evidentemente presenta frecuentes recurrencias en la familia. Bodian ha preferido denominar esta condición agenesis de los plejos intramurales debido a que no hay evidencia del incrementos en los haces nerviosos que se observa en la aganglionosis congénita.La enterocolitis sigue siendo la mayor amenaza en el manejo de estos pacientes. La urgencia de iniciar tratamiento demanda un más amplio reconocimiento. La enfermedad de Hirschsprung debe ser diagnosticable casi enteramente en el periodo neonatal ahora, cuando existen procedimientos de biopsia confiables y de mínimo riesgo, tales como la succión rectal o biopsia de sacabocado (punch biopsy). Debe ser aceptado, sin embargo, que este procedimiento diagnóstico demanda un patólogo idóneo y experimentado y significa, por lo tanto, alguna centralización de los materiales a ser examinados, si no del infante mismo.El hallazgo de clara neuropatología en presencia de ganglios indica que pueden existir alteraciones sutiles, cuyo reconocimiento puede justificar los resultados pobres después de la operación que no puedan ser explicados por errores técnicos.El caso difícil y en particular el niño cuya biopsia superficial se informa como gangliónica, pero biopsia anormal, debe ser investigado por medio de una biopsia de espesor total con técnicas especiales tales como histoquímica y coloración de plata, microscopía electrónica y tal vez estudios de inmunofluoroscencia, según indicación. Sinembargo, parece que estas condiciones no son suficientemente comunes para justificar la recomendación de una biopsia de espesor total bajo anestesia general o el uso de tan laboriosos estudios en forma rutinaria; por el contrario, al estimular el diagnóstico precoz por biopsia por succión rectal se logrará simplificar la evolución clínica de una vasta proporción de niños con enfermedad típica de Hirschsprung tanto para el cirujano, como para el niño y su familia.

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Résumé Chacune des opérations de base pour traiter la maladie de Hirschprung: rectosigmoïdectomie de Swenson, voie rétro-rectale transanale de Duhamel, méthode endorectale de Soave modifiée par Denda et Boley peut donner de bons résultats sous la protection d'une colostomie de routine dont les inconvénients sont acceptables dès lors qu'elle permet d'éviter les risques d'incontinence, d'infection sépticémique ou de mort.La révision de 84 des 87 opérations de Swenson pratiquées pendant 25 ans a permis de constater que tout sujet adulte était devenu continent alors même qu'il avait pu présenter un certain degré d'incontinence dans son enfance. Le suivi pendant une période de 10 à 17 ans des sujets ayant subi l'opération de Soave a permis de noter des faits identiques. Il en a été de même chez les opérés ayant été soumis dès 1958 à l'opération de Duhamel.Sur le plan technique, le problème qui reste à résoudre concerne les nourrissons dont l'affection s'étend à l'intestin grêle. L'opération de Kimura en représente une solution éventuelle mais la très rare aganglionose intestinale totale paraît impossible à traiter. Bodian préfère d'ailleurs qualifier cet état d'agénésie des plexus intramuraux du fait de l'absence de fibre nerveuse.L'entérocolite constitue la complication toujours menaçante de l'affection. Il est donc nécessaire de la traiter d'urgence, ce fait étant à souligner. La maladie de Hirschprung doit donc être reconnue tôt au cours de la période néonatale alors que la biopsie par suction ou par ponction est possible sans conséquence sérieuse. La méthode est très fiable mais on doit savoir cependant qu'elle implique d'être réalisée par un pathologiste expérimenté, d'où la nécessité de la pratiquer dans un centre spécialisé. La découverte d'un fait neuropathologique évident au niveau des sites ganglionnaires n'est pas constante si bien qu'en présence de variations difficiles à interpréter les résultats de l'opération ne sont pas toujours satisfaisants alors même que l'intervention a été bien conduite.Le cas difficile est représenté par l'enfant dont la biopsie superficielle n'est pas parfaitement normale ce qui conduit à pratiquer un prélèvement complet de la paroi pour le soumettre à des explorations spéciales: études histochimiques avec imprégnation à l'argent et immunofluorescence hormonale. Cependant cette condition n'est pas si fréquente pour qu'il faille considérer la pratique de la biopsie épaisse sous anesthésie générale comme une pratique de routine. En fait la biopsie par suction au niveau du rectum, dès lors qu'elle est pratiquée précocemment permet dans la majorité des cas de reconnaître la maladie de Hirschprung avec sécurité et cela au moindre frais pour le nourrisson, la famille et le chirurgien.

     

巨结肠次全或左半结肠切除术后的远期排便功能比较

目的 比较先天性巨结肠次全或左半结肠切除术后患儿不同时期的排便功能,了解两组切除不同范围的结肠对患儿远期排便功能及生长发育的影响.方法 将128例先天性巨结肠行心形吻合术患儿分为两组:一组为左半结肠切除,共56例;另一组为结肠次全切除,共72例.两组随访4～12年,比较两组的排便功能、生活质量、生长发育及便秘复发等情况.结果 术后半年的排便功能比较,左半结肠切除组患儿评估明显好于次全切除组,而2年后两组的排便功能、生活质量比较差异无统计学意义.次全切除组便秘复发1例,左半切除组便秘复发5例.对身高/年龄及体重/年龄评估得出,两组患儿在各百分位范围内的比例差异无统计学意义.结论 结肠次全切除患儿远期排便功能与左半切除患儿相比差异无统计学意义,生活质量及生长发育两组均可以达到正常范围,左半结肠切除的患儿便秘复发率比次全切除要高.     

Hirschsprung's disease: Clinical and experimental observations

Hirschsprung's disease (HD) is a relatively common cause of intestinal obstruction in the newborn. It is characterized by an absence of ganglion cells in the distal bowel beginning at the internal sphincter and extending proximally for varying distances. The etiology of HD-associated enterocolitis remains a complex issue. This study has provided further support for a possible infectious etiology of enterocolitis complicating HD.

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Resumen La enfermedad de Hirschsprung es una causa relativamente común de obstrucción intestinal en el recién nacido. Se caracteriza por ausencia de las células ganglionares en el colon distal a partir del esfínter interno hasta distancias variables en sentido proximal. La aganglionosis aparece confinada al rectosigmoide en 75% de los pacientes, al sigmoide, ángulo esplénico o colon transverso en 17%, y a la totalidad del colon con un corto segmento del ileon en 8% de los pacientes. La aganglionosis intestinal total con ausencia de células ganglionares desde el duodeno hasta el recto constituye la forma más rara dse enfermedad de Hirschsprung. Se estima la incidencia de la enfermedad de Hirschsprung en 1 en 1.500 nacimientos vivos. Spouge y Baird estudiaron la incidencia en la prpovincia de British Columbia y reportan una tasa de 1 en 4.417. La entidad es más común en los niños, con una relación masculino: femenino de 41, pero esta mayor prevalencia en el sexo masculino es menos evidente en los pacientes con largos segmentos afectados, donde la relación de sexos es 1.52.1. En este artículo se revisa la etiología, patofisiología, patología, cuadros clínicos, métodos de diagnóstico y manejo. La enterocolitis se mantiene como la complicación más grave de la enfermedad de Hirschsprung y se asocia con alta morbilidad y mortalidad.

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Résumé La maladie de Hirschprung est rare (une sur 5000 naissances), atteint surtout les garçons, mais constitue une cause fréquente d'occlusion chez le nouveau-né. Elle se caractérise par l'absence de cellules ganglionnaires dans l'intestin distal d'extension proximale variable. Dans 75% des cas, elle n'intéresse que le rectum et colon sigmoïde. Le diagnostic repose sur la clinique, les données de la manométrie anorectale et surtout l'examen anatompathologique, qui seul confirme la zone aganglionnaire. Le traitement est une reconstruction colorectale, soit traditionnellement en deux-temps, soit, de plus en plus souvent, en un seul temps opératoire. L'association d'une entérocolite est une complication grave et nécessite, en urgence, une réanimation adaptée et souvent une colostomie.

     

腹腔镜辅助与单纯经肛门手术行一期Soave拖出术治疗先天性巨结肠的对比研究

目的:探讨腹腔镜手术行一期经肛门Soave拖出术的优点及常规腹腔镜探查的必要性。方法:回顾分析110例巨结肠患者行经肛门Soave拖出术的临床资料。分为两组:常规腹腔镜辅助经肛门Soave组(LTS,n=73),单纯经肛门并选择性应用腹腔镜手术组(TS,n=37)。分析两组手术过程,比较肠蠕动恢复时间、手术时间、并发症及近期排便情况。结果:TS组中3例因拖出结肠移行段不明确选择性应用腹腔镜,3例长段型巨结肠单纯经肛门游离系膜困难用腹腔镜游离系膜和脾曲。两组患者年龄、性别、移行区位置、小肠结肠炎、肛门狭窄、吻合口并发症、肠蠕动恢复时间、手术时间等无显著差异。LTS组肛门部解剖时间51～71min,平均61min,明显短于TS组;手术费用LTS组比TS组平均高约2 000元。LTS组2例患儿因吻合口瘘行肠造瘘术。TS组5例患者需再次手术,1例吻合口瘘行肠造瘘,1例肠扭转,1例肛门回缩、狭窄,2例便秘复发。术后随访3个月～8年,平均4年6个月,两组近期排便功能相似。结论:单纯经肛门Soave术能完成多数短段型和常见型巨结肠的诊治,费用低于腹腔镜Soave手术,近期排便功能良好,不需常规应用腹腔镜。但当拖出困难、疑为长段型时则应使用。腹腔镜辅助Soave术是更全面的技术,适于不同类型的患者。