限制性后人字入路直肠肛门成形及外括约肌成形术治疗中低位无肛合并直肠会阴瘘

目的 针对中低位无肛合并直肠会阴瘘的患儿改良一种新的手术方法,使之较现行的方法如Pena、后切术更加简便、更符合生理以减少手术并发症.方法 切口采用限制性(即限制在3 cm以内)后人字切口,显露瘘管和直肠下端,在直肠下端的后方将肛门外括约肌复合体部分切开,将两侧的肌纤维环包于直肠下端.完成外括约肌成形术,在中线后切瘘管及直肠壁,于正常肛隐窝处完成肛门成形.结果 24例先天性无肛合并直肠会阴瘘(X线侧位片直肠盲端在P-C线或至Ⅰ线间)患儿进行了该手术,术后全部病例进行随诊:除1例5岁患儿术后便秘,全部患儿恢复自主排便,可控制,无失禁及溢粪.1例患儿术后肛门切口轻度感染经切开引流治愈.结论 该手术较Pe(n)a手术操作更加简便,局部创伤小,术后避免直肠回缩,减少了术后并发症,且术后不必扩肛;不同于传统的"后切术",术中进行肛门外括约肌成形,术后随诊疗效满意.

Abstract：

Objective To study the clinical outcomes of anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision to repair intermediate and low anorectal malformation complicated with rectal perineal fistula in children. Methods Twenty four patients with intermediate and low anorectal malformation complicated with rectal perineal fistula were enrolled in this study. Via the posterior approach, a Y-shaped incision (＜3 cm) was made to expose the lower rectum and fistula. The external anal sphincter was identified and dissected at the posterior aspect of the lower rectum. The external anal sphincterplasty was performed by overlapping the ends of the muscle around the lower rectum. The rectal perineal fistula was resected and anorectoplasty was performed. Results All surgeries were successfully performed. Voluntary bowel movement and fecal continence was restored on 23 patients after surgery. One 5 years old patient still had constipation. Mild incision infection was found on 1 patient, and was cured after opening the incision and draining the pus. No fecal incontinence or anastomotic stricture was noted. Conclusions Compared with Pena surgery, anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision is less complicated and invasive to repair intermediate and low anorectal malformation complicated with rectal perineal fistula. By using this procedure, better sphincter shape and fecal function is achieved on patients.     

Anterior sagittal anorectoplasty for anovestibular fistula

Anterior sagittal anorectoplasty (ASARP) was used for the definitive correction in 107 cases of anovestibular fistula (AVF) between 1996 and 2005. These cases were subjected to three different types of treatment regimes during the same period. Majority of the cases (78) were operated in one stage where postoperatively an early oral feed was started (A). Cases were discharged in 2–4 days. In the second group (B), there were ten cases who were also operated in one stage but with prolonged fasting of 9–10 days postoperatively. Nineteen cases (C) were operated under cover of colostomy during the same period. In the immediate postoperative period, among the group A, one case had a major wound disruption requiring a colostomy and a redo surgery. Three cases had subcutaneous leak. In seven cases there was premature dehiscence of mucocutaneous or skin sutures. In groups B and C, there were no significant complications in the immediate postoperative period. In the follow-up period, out of 107 cases, 63 (58.8%) had constipation at the end of 3 months. However, at the end of one year, only 24.3% (26 cases) cases had constipation. Regarding fecal continence, 86 cases (90.5%) were totally continent. Seven had history of occasional soiling and in two cases, soiling was more frequent. As far as repair or correction of AVF or vestibular anus is concerned, we feel that anterior sagittal approach is more suitable as it requires less pelvic dissection. Separation of posterior vaginal wall from rectum, which is considered, is the most important step of the operation, takes place under direct vision. We also feel that AVF can be repaired in one stage with an early postoperative oral feed, provided we are meticulous in pre and postoperative bowel management. It reduces hospital stay and the cost of treatment. This provides a good option to cases who are not able to afford prolonged hospitalization (fasting) or are not willing for a colostomy.     

Is posterior anal transfer a good treatment for vestibular anus?

Eleven females who had a posterior anal transfer (PAT) for a vestibular anus were reviewed in order to: (1) assess the long-term functional success of this operation clinically; (2) evaluate the anorectal manometry profile; and (3) assess defaecation by video proctography. The cosmetic appearance was excellent in all patients. Seven had a good result, 2 a fair result, and 2 a poor result when assessed clinically (Kelly score) in terms of constipation and soiling. The anorectal manometry profile remained within normal limits except in the 2 with a poor result who had diminished sensory awareness of stool in the rectum. PAT does not appear to damage the muscle-sphincter complex, and the children with a large rectum full of faeces seemed to behave like children with acquired megacolon and constipation. Video proctography showed normal defaecatory patterns in all patients. Patient selection is important for this procedure. When PAT was used as the initial treatment a good result was obtained; when it was used to relieve constipation following previous surgery the result was less satisfactory.     

Functional outcome in correction of perineal fistula in boys with anoplasty versus posterior sagittal anorectoplasty

Optimal surgical therapy for low anorectal anomalies remains controversial. We compared functional outcome after correction of perineal fistula in boys with either anoplasty (AP) or limited posterior sagittal anorectoplasty (PSARP). Thirty-nine boys from two centres treated for perineal fistula with either AP (n = 24) or PSARP (n = 15) from 1996 to 2001 underwent prospective follow-up for functional outcome. In order to minimize heterogeneity of the study groups, only boys with perineal fistula were included. Functional outcome was assessed using a validated bowel function score (maximum score 20). Day and night time wetting as well as the age at potty training were also recorded. An independent nurse specialist interviewed caregivers. AP and PSARP groups were comparable regarding age, associated malformations and frequency of sacral dysplasia. No significant differences between the groups were observed in the overall median bowel function score, AP 18 (11–20) versus PSARP 18 (11–20), or in the age at potty training, AP 32 (14–66) versus PSARP 36 (18–60). Covering colostomy was employed significantly (P < 0.05) more often with PSARP (10/15) than with AP (1/24). Surgery for local complications was carried out significantly (P < 0.05) more often after PSARP (5/15) than after AP (2/24). Two patients in both groups required a temporary salvage colostomy. Overall functional outcome is comparable after AP and PSARP for perineal fistula in boys. As a more straightforward procedure AP is safer and less prone to complications avoiding the need for covering colostomy.     

Perineal canal

Perineal canal (PC) is a rare anomaly constituting 4% of all anorectal malformations. Sixty patients (56 females and 4 males) with PC managed over the past 27 years are reported. The ages ranged from 2 days to 13 years. The chief symptom was passage of fecal matter through both the anus and the fistula. One girl had undergone previous, unsuccessful surgery. All our patients were treated by anterior sagittal anorectoplasty (ASARP), which allowed anatomic exposure and accurate repair of the anomaly. In 49 patients without any perineal inflammation primary ASARP was undertaken. Surgery was delayed in 11 patients with perineal excoriations and/or active inflammation. One patient died post-operatively due to unrelated causes and 1 developed a recurrence. Anal dilation was required in 7 cases. Fifty patients were seen at first follow-up 12 weeks after surgery. All were continent and had normal defecation without the use of laxatives. Thirty-four could be followed up to the age of 3 years; they were continent with normal bowel habits. There was no shift in the position of the anus and no instance of rectal dilation. Individualization of the management and operation by the anterior sagittal approach thus offers good results in this uncommon anorectal anomaly.     

Management of perineal canal anomaly

A 6-year-old girl presented due to passage of stool through her vulva since birth. Examination revealed a fistulous tract between vestibule of the vagina and an otherwise normally formed anal canal. The tract was successfully excised through an anterior sagittal approach with a defunctioning sigmoid colostomy, which was closed 12 weeks later. The embryology, morbid anatomy, and treatment of this rare congenital anomaly are discussed along with a review of the literature.     

Laparoscopic assisted endorectal pull-through with posterior sagittal approach to the repair of postoperative rectourethral and rectovaginal fistula

Rectourethral or rectovaginal fistula is a troublesome complication after anorectal surgery. The pelvic and perineal dissection may be difficult because of severe fibrosis adhesion around the fistula. The authors applied a novel technique: a combined laparoscopic assisted abdominal and posterior sagittal approach (PSA) to perform the redo surgery. Three boys and two girls (3–13 years old): case 1 had rectovaginal fistula after rectal dialation and modified Swenson’s procedure; case 2 had rectovestibular fistula after twice perineal anorectoplasty; case 3 had rectourethral fistula after twice anorectoplasty; case 4 was imperforate anus with Hirschsprung’s disease and rectourethral fistula that had been misdiagnosed; case 5 had rectourethral fistula after abdominoperineoanoplasty and Mollard procedure and posterior sagittal anorectoplasty. Laparoscopic assisted abdominal dissection was done first to mobilize the colon as far as the mid pelvis, and the normal colon was marked with a suture. The lower pelvic dissection was performed through the posterior sagittal route, the proximal rectum was mobilized and servered, the distal rectum was left undisected, endorectal mucosectomy with electric ablation was performed, then the fistula was closed from inside the rectum, and the stump of the colon was pulled through the rectum, the stump and the dentate line were anastomosed extraanally. Colostomy was done in case 2 and case 5. The postoperative follow-up showed no recurrent fistula, and all patients had attained normal voluntary bowel actions, but one child had infrequent minor soiling. Laparoscopic assisted endorectal pull-through of the intact colon can offer precise dissection, minimal abdominal injure, and spare troublesome mobilization of the fistula, and can prevent the recurrent of fistula. Posterior sagittal approach provides a direct repair of the fistula and anastomosis.     

Posterior sagittal approach for reconstruction of an unusual type of cloacal anomaly

Posterior sagittal anorectoplasty is a popular procedure for treatment of high anorectal malformations. A female infant underwent posterior sagittal anorectovaginourethroplasty for treatment of an unusual type of cloacal anomaly associated with three fistulas: a high and a low rectovaginal fistula, and a high urethrovaginal fistula. A satisfactory anatomic and functional result was obtained. Offprint requests to: S.-Y. Yoo     

Physical function assessment tools in pediatric rheumatology

Pediatric rheumatic diseases with predominant musculoskeletal involvement such as juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis(JDM) can cause considerable physical functional impairment and significantly affect the children's quality of life (QOL). Physical function, QOL, health-related QOL (HRQOL) and health status are personal constructs used as outcomes to estimate the impact of these diseases and often used as proxies for each other. The chronic, fluctuating nature of these diseases differs within and between patients, and complicates the measurement of these outcomes. In children, their growing needs and expectations, limited use of age-specific questionnaires, and the use of proxy respondents further influences this evaluation. This article will briefly review the different constructs inclusive of and related to physical function, and the scales used for measuring them. An understanding of these instruments will enable assessment of functional outcome in clinical studies of children with rheumatic diseases, measure the impact of the disease and treatments on their lives, and guide us in formulating appropriate interventions.     

Internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations — technical considerations

Posterior sagittal anorectoplasty (PSARP) has gained overall acceptance as a standard surgical approach for the repair of high and intermediate anorectal malformations. PSARP allows superior exposure for the identification of the spectrum of these defects and enables anatomic reconstruction of the anal canal and voluntary sphincter muscle complex. In the original PSARP procedure, the involuntary internal anal sphincter, which in embryologic and clinical studies has been shown to be present in the region of the fistulous bowel termination, is not preserved. Internal sphincter-saving PSARP was performed in 65 patients with high or intermediate anorectal malformations. Manometrically, a positive rectoanal inhibitory reflex was noted in 82% of the patients, suggesting the presence of a functional internal sphincter. There was a statistically significant difference in the anorectal resting pressures between patients with a functional internal sphincter and patients without this structure. The technical details of internal sphincter-saving PSARP are described and illustrated in the present communication.     

Vestibular fistula: the operative dilemma — cutback,fistula transplantation or posterior sagittal anorectoplasty?

Vulvovestibular fistulas usually have a good prognosis. Most pediatric surgeons use operations such as cutback, fistula transposition without colostomy, and lately, posterior sagittal anorectoplasty with colostomy. Since 1978, the authors have been involved in the surgical treatment of 18 patients with vestibular fistulas without colostomy using a modified Santulli technique. Our results are satisfactory. We discuss voluntary bowel movements, soiling, constipation, and cosmetic effects. Comparisons are made between the three techniques with a discussion of advantages and disadvantages. We personally prefer anal transposition without colostomy. Offprint requests to: S. ivkovi     

Urogenital sinus with sinorectal H-fistula — a hitherto undescribed anorectal anomaly

In a urogenital sinus anomaly, the urethra and vagina are conjoint for a variable distance and the anorectum is usually intact and complete. We report a case of a urogenital sinus with a fistula between the rectum and the sinus. The anomaly was successfully repaired at 2.5 years of age through a midline muscle-splitting incision in the anterior perineum. The anterior wall of the anorectum was divided in the line of the incision in order to deal with the fistula. Repair was done in layers.     

Hirschsprung disease—Bowel function beyond childhood

Hirschsprung disease is a developmental defect of the enteric nervous system characterized by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term outcomes indicating that impaired bowel function is common. Recently, several controlled studies show that bowel function outcomes are affected beyond childhood, in adolescents and adults, compared with healthy control subjects. Constipation and fecal incontinence are common. The impaired bowel function appears to have a negative impact on quality of life, although, a majority of patients have adapted to their symptoms. On the other hand, Hirschsprung disease seems to have limited impact on education and occupation in adult life.The aim of this review was to summarize current knowledge of bowel function outcome beyond childhood in patients with Hirschsprung disease.     

Repair of anorectal anomalies in the neonatal period

In a 3-year period between 1986 and 1988, 147 patients with anorectal anomalies were seen in our department; 93 were neonates, of which 35 had low malformations treated by anoplasty soon after birth. Fifty-eight had high and intermediate types; 26 of these had defunctioning colostomies. One of the 26 patients had the definitive repair done in the neonatal period, the other 32 were operated on with posterior sagittal anorectoplasty (PSARP) as a one-stage procedure in the neonatal period. The majority (85%) were operated on within 4 days of birth. There was no operative mortality. The commonest complication was wound infection, which was mild in 15 patients and severe in 2. There were no anal strictures. The functional results were good in 30 and average in 3 patients. A comparison with 60 older children who had a similar operation done as a staged procedure in the same period showed equally low morbidity and good functional results. Based on these results, it was found that one-stage repair of high and intermediate types of anorectal anomalies in the neonate using PSARP is safe and feasible.     

Intraoperative damage to the male urethra and pelvic visceral motor nerves during posterior sagittal anorectoplasty

The use of the sagittal surgical approach to the rectum and urinary fistula in male patients with anorectal agenesis is addressed. Iatrogenic damage to the urethra (1 in 60 cases) and pelvic (2 in 60 cases) occurred when the operator had difficulty identifying the rectum intraoperatively and where inappropriate midline dissection was done. The importance of an adequate preoperative contrast rectogram and the intraoperative identification of the rectum with the aid of a catheter is emphazied. Perirectal blunt dissection is discouraged, the need for deep lateral directed tissue retraction is avoided by applying direct traction onto the freed rectum, and a long and wide portion of rectal wall is left in the midline on the urethra and bladder neck. The anatomy of the autonomic pelvic plexus at risk in the normal and congenitally abnormal case is described.     

Normal monocyte-derived dendritic cell function in patients with Langerhans-cell-histiocytosis

BACKGROUND: Langerhans cell histiocytosis (LCH) is a histiocytic disease, characterized by the lesional accumulation of dendritic Langerhans cells together with T cells and eosinophils. The cause of this disease is unknown. Langerhans cells are bone marrow-derived dendritic cells (DCs), which can develop from CD34(+) hematopoietic progenitor cells as well as from monocytes. PROCEDURE: To test whether LCH patients have a general functional defect present in cells of their DC lineage, we generated immature DCs by culturing monocytes from nine patients with single- or multisystem LCH with GM-CSF and IL-4, and analyzed their phenotype and function before and after an in vitro maturation stimulus. Immature DCs were analyzed for their phenotype and cytokine production, DCs matured in response to TNF-alpha plus PGE(2) were analyzed for their phenotype, their stimulatory capacity in MLR, cell aggregation, and activation-induced apoptosis. RESULTS: In summary, no difference was found between both immature as well as mature DCs generated from patients and controls regarding the expression of CD1a, CD80, CD86, MHC class I, and MCH class II antigens. Similarly, no difference was found regarding IL-10, -12, and TNF-alpha production, as well as regarding cell aggregation and apoptosis in response to external stimuli. CONCLUSIONS: The absence of gross functional abnormalities in DCs generated from monocytes from patients with LCH makes the existence of a severe functional defect affecting all cells of the DC lineage in these patients unlikely.     

Rectal atresia: pathogenesis and operative treatment

Rectal atresia (RA) with a normal anus is a rare anomaly mostly described as part of a series of anorectal malformations. Most authors believe it to be an acquired lesion with a vascular genesis. One of the arguments quoted is the lack of other congenital anomalies. Several operative procedures are recommended for this lesion. We describe four patients with RA who had other significant congenital anomalies; two other cases were found in the literature. A lack of other congenital anomalies in patients with RA does not seem to be a strong argument for an acquired lesion. All four patients were treated by a posterior sagittal approach with good functional results.     

A rare anorectal malformation: a very large H-type fistula

A very large H-type rectovaginal fistula was observed in a 9-year-old girl. The diameter of the abnormal opening was about 15 mm. The fistulous tract originated from the stenotic segment 1 cm above the pectinate line of the anus and was connected to the back wall of the vagina. The patient underwent posterior sagittal transrectal surgery with a protecting colostomy. At present, she has good bowel function and sphincter control without recurrence of the fistula. Accepted: 19 December 2000     

Mucosal colonic tube fistula with antireflux wrap for antegrade colonic enema

Antegrade enemas can provide children with excellent faecal continence in situations where adequate control has been compromised because of underlying congential anomaly or poor surgical outcome in their treatment. The enema is often delivered through an appendicostomy. If the appendix is absent or utilized for another purpose, then placement of a chait tube or caecostomy button can provide access to the colon for the enema. However, these devices may be associated with breakages, accidental removal and leakage and replacement may require another operative procedure under a general anaesthetic. Full thickness colonic tubes can also be constructed at any point along the colon but in the author’s experience, they can be associated with significant leakage of both gas and faecal material. The construction of a mucosal colonic tube with anti-reflux wrap is a technique that avoids the above problems and offers a distinctive advantage in selected situations. The technique relies on tabularising mucosa alone to create a continent fistula. Six children with severe soiling underwent the technique. The outcomes were evaluated using a modified quality of life score (QOLI) [1, 2]. The score included assessment of soiling, staining, odour, self-esteem and socialization measure. Technical evaluation included analysis of the ease of catheterization and continence of the mucosal fistula site. All six patients had dramatic improvement in their faecal continence with complete resolution of soiling in all six. Follow up median is 42 months and the range is 6–48 months. QOLI scores improved from a total of 4.75 to 18.5. Possible range is 0–21. All the six fistula sites catheterize easily and no stenosis or faecal leakage has occurred. Two patients required treatment of minor granulations at the entry site of the fistula during the early healing phase.     

Urethral advancement in hypospadias with a distal division of the corpus spongiosum: Outcome in 158 cases

ObjectiveOutcome of urethral mobilization and advancement (Koff procedure) in hypospadias with a distal division of the corpus spongiosum and redo cases with distal urethral failure.Materials and methodsFrom January 1999 to November 2012, 158 children with a distal hypospadias (115 primary cases and 43 redo cases) underwent surgical repair using the Koff technique with a median age at surgery of 21 months (range, 12–217 months).ResultsMean follow-up was 19 months (median, 14 months). Thirty patients (19%) presented with a complication (13.9% in primary cases and 32.5% in redo surgery) mostly at the beginning of our experience. Meatal stenosis was the most common one (3.5% in primary case, 6% overall). Ventral curvature (>10°), which is considered as a possible long-term iatrogenic complication of the Koff procedure, was not found in patients with fully grown penis except in one redo patient who had, retrospectively, an inadequate indication for this type of repair. Of 158 patients, 33 reached the age of puberty (>14 years old) with a mean follow-up of 34 months, only one presented with a significant ventral curvature.ConclusionUrethral mobilization and advancement is a reasonable alternative for anterior hypospadias and distal fistula repair in selected cases. It has two major advantages compared to other techniques: it avoids any urethroplasty with non-urethral tissue and eliminates dysplastic tissues located beyond the division of the corpus spongiosum, which may not grow at the same pace as the rest of the penis. Significant iatrogenic curvature in fully grown penis is not supported by this series.