Treatment Of Anorectal Malformations Research Articles

Clinical application of middle descending colon-double lumen ostomy with distal stoma narrowing in the treatment of anorectal malformation

BackgroundAnorectal malformations (ARMs) are the most common congenital anomaly of the digestive tract. And colostomy should be performed as the first-stage procedure in neonates diagnosed with intermediate- or high-type ARMs. However, the most classic Pe˜na’s colostomy still has some disadvantages such as complicated operation procedure, susceptibility to infection, a greater possibility of postoperative incision dehiscence, difficulty of nursing and large surgical trauma and incision scarring when closing the stoma. We aimed to explore the effectiveness of middle descending colon-double lumen ostomy (MDCDLO) in the treatment of high and intermediate types of anorectal malformations.MethodsWe retrospectively reviewed the data of patients who underwent MDCDLO for high or intermediate types of ARMs between June 2016 and December 2021 in our hospital. The basic characteristics were recorded. All patients were followed up monthly to determine if any complication happen.ResultsThere were 17 boys and 6 girls diagnosed with high or intermediate types of ARMs in our hospital between June 2016 and December 2021. All 23 patients were cured without complications such as abdominal incision infection, stoma stenosis, incisional hernia, and urinary tract infection in the postoperative follow-up time of 6 months to 6 years except one case of proximal intestinal prolapse was restored under anesthesia.ConclusionMDCDLO offers the advantages of simplicity, efficiency, safety, mild trauma, and small scarring in the treatment of high and intermediate types of anorectal malformations.

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021.

Anorectal malformations (ARMs) are complex congenital anomalies. The corrective operation is demanding and schedulable. Based on complete national data, patterns of care have not been analyzed in Germany yet. All cases with ARM were analyzed (1) at the time of birth and (2) during the hospital stay for the corrective operation, based on the national hospital discharge data (DRG statistics). Patient's comorbidities, treatment characteristics, hospital structures, and the outcome of corrective operations were analyzed with respect to the hospitals' caseload. From 2016 to 2021, 1,726 newborns with ARM were treated at the time of birth in 388 hospitals. Of these hospitals, 19% had neither a pediatric nor a pediatric surgical department. At least one additional congenital anomaly was present in 49% of cases and 7% of the newborns had a birthweight below 1,500 g.In all, 2,060 corrective operations for ARM were performed in 113 hospitals in the same time period. In 24.5% of cases, at least one major complication was documented. One-third of the operations were performed in 56 hospitals, one-third in 20 hospitals, and one-third in 10 hospitals with median annual case numbers of 2, 5, and 10, respectively.Hospitals with the highest caseload operated cloacal defects more often than hospitals with the lowest caseload (7 vs. 2%) and had more early complications than hospitals with the lowest caseload (30 vs. 21%). This difference was not statistically significant after risk adjustment. Children with ARM are multimorbid. Early complications after corrective surgery are common. Considering the large number of hospitals with a very low caseload, centralization of care for the complex and elective corrective surgery for ARM remains a key issue for quality of care.

"The People Criticized Me and Insulted Me Due to Having a Child with Defect": Experiences of Somali Parents of Children with Anorectal Malformation.

Treatment of anorectal malformations (ARM) and the management of clinical outcomes are challenging for families. While most studies focus on mothers suffering from care burden, high stress, and low quality of life, there is limited knowledge of what fathers of children with ARM experience; therefore, this study aimed to examine how both mothers and fathers have experience caring for children with ARM and how beliefs and cultural issues affect the perception of congenital anomalies. A qualitative design. Consolidated criteria for reporting the qualitative research guidelines were used in this study. The guidelines for thematic analysis were followed for data analysis. Ten mothers and six fathers were interviewed, and their mean age was 32.5 ± 4.2 years. Of the 75% (n = 12) illiterate and 93.7% (n = 15) had an expanded family type, all their religious affiliations were Muslim. The four main themes were (1) ambiguity, (2) challenges, (3) stigma, and (4) coping, which were determined in line with the results of the data analysis of parents' views on having and caring for children with ARM. In this study, many Somali parents reported that they lacked disease and treatment knowledge, physical exhaustion, and some difficulties related to a lack of access to the hospital because of living in rural areas, caring for the child, and dealing with long-term complications, colostomy, anal dilatation, and enema before or after anoplasty. While all parents mentioned that having a child with ARM was a fate, it was seen as punishment by their close social environment. Consequently, this study could serve as a foundation for planning comprehensive healthcare and physical and psychosocial support for multidisciplinary health professionals.

Posterior Sagittal Approach for Nonanorectal Malformation: Widening Spectrum.

Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to the deep pelvic structures. It reduces risk of injury to important structures as dissection remains in midline. To access feasibility of posterior sagittal approach for non-anorectal malformation indications and to widen the spectrum. We present a series of 10 cases of non-anorectal malformations operated by this approach for 4 years. Six patients included in the study were of Disorders of Sexual Differentiation with pseudovagina, three of Y duplication of the urethra, and one of cervical atresia. All patients had good results. Posterior sagittal approach is feasible, safe with minimal bleeding, and no postoperative incontinence. It can safely be used for non-anorectal indications.

ОПИСАНИЕ СЕРИИ СЛУЧАЕВ ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ РЕКТАЛЬНОГО ПРОЛАПСА ПО МОДИФИЦИРОВАННОЙ ТЕХНОЛОГИИ ДЕЛОРМА У ДЕТЕЙ С АНОРЕКТАЛЬНЫМИ МАЛЬФОРМАЦИЯМИ

Rectal prolapse (RP) is one of the most expected complications following the treatment of anorectal malformations that occurs in 3.8% to 60% of cases. The main method of surgical treatment of RP is anoplasty with a recurrence rate after surgery of up to 33%. The authors describe the indications, technique and results of transanal rectal mucosectomy and muscular plication (TRMMP) proposed by Dr. Luis De La Torre-Mondragon, MD, Pediatric Colorectal Surgeon with the Children's Hospital Colorado (CHCO), Aurora, Colorado, United States, for the treatment of RP in children with anorectal malformations (AM). Materials and methods used: in a retrospective analysis of 234 patients with AM, 8 patients with RP (all males) were identified. In 2 patients, a displacement of the rectum was detected, and subsequently they underwent the posterior sagittal anorectoplasty (PSARP). Another 6 patients underwent the TRMMP. Results: all patients had previously undergone the open surgery without PSARP. The average age of patients at the time of surgery was 4.6 years (Q1-2.5, Q3-7.9; SD-2.2; min/max 2.0-5.0; 95% CI: 2.1-5.5). In all patients, the RP length averaged 2.3 cm (Q1-1.5, Q3-3.9; SD-0.9; min/max - 1.6-4.0; 95% CI: 1.4-3.3). There were no intraoperative and postoperative complications. There were no relapses of RP and stenoses of anastomoses during the dynamic follow-up after surgery for 7.6 months recorded as well (Q1-5.0, Q3-10.9; SD-2.4; min/max - 5.0-11; 95% CI: 5.1-10.2). Conclusion: the authors’ first experience confirms the efficacy of TRMMP in RP in children with AM.

Laparoscopic Approach in Anorectal Malformations: How We Do It.

Laparoscopy has a major role in the treatment of anorectal malformations (ARM) since Georgeson et al. introduced the laparoscopic assisted anorectoplasty in 2000. The majority of ARM can be repaired through a posterior sagittal incision without the need to enter the abdomen. Some ARM types require an abdominal approach to detach the rectum from the urinary tract in males or the vagina in females. Those malformations benefit from laparoscopy. In this article, we aimed to highlight the technical aspects relevant to the current laparoscopic repair of ARM.

Long-term urologic and gynecologic follow-up and the importance of collaboration for patients with anorectal malformations

Anorectal malformations (ARM) are well recognized to be associated with anomalies in other organ systems. The introduction of screening protocols has increased the diagnosis of these anomalies and greater collaboration with other specialties has influenced the treatment and follow-up of patients with ARMs. Much of the medical literature regarding the treatment of anorectal malformations has focused on technical details of operations and early post-surgical outcomes. Recently, an increase in published data regarding the long-term sequelae of an ARM diagnosis has resulted in an emphasis extended follow up in this population. Patient support groups have highlighted complex issues in ARM patients persist into adulthood have advocated for improved transitional care. This article describes the benefits of long-term follow-up and identifies key issues in ARM patients with respect to urologic and gynecologic health. A collaborative model of care is outlined and suggested timings of screening for potential problems is described.

From Prenatal Diagnosis Through Transition to Adult Care: What Everyone Should Know About Anorectal Malformations

This article provides a general summary of the current state-of-art on the treatment of anorectal malformations (ARM). Anorectal malformations occur in one in every five thousand births (Hohlschneider and Hustson 2006). With the current birth rate of 12 per 1000 in the USA, we expect between 600 and 800 babies with anorectal malformation to be born every year. Associated anomalies are frequently found in patients with anorectal malformations. The most common are urologic and are found in 50% of children with ARM. They include vesicoureteral reflux, renal agenesis, dysplastic kidneys, or duplicated collection system. Gynecologic abnormalities can include the 34% of females with duplication of Mullerian structures. Thirty percent of females born with a cloacal anomaly have a hydrocolpos, and 5.6% of females with an ARM have vaginal agenesis. Congenital heart disease is found in approximately 30% of ARM patients. Commonly identified anomalies include VSD, ASD, Tetralogy of Fallot, truncus arteriosus, and transposition of the great arteries. Spinal abnormalities can be observed as well, and 25% of ARM patients have a tethered cord. Finally, other digestive tract abnormalities consist of esophageal atresia in 8% of cases and duodenal atresia in 3%. (Hohlschneider and Hustson 2006). Understanding the original ARM anatomy with its associated prognosis and sequelae is important to provide appropriate care throughout the life of the patient. Medical care has been arbitrarily divided into pediatric and adult categories, without taking into consideration the individual patient’s needs. Individuals born with an anorectal malformation require management of the sequelae of these conditions throughout their entire life. There is an unmet need for the proper transition of care for these patients that requires further action and collaboration between pediatric and adult specialists.

REOPERATIONS IN CHILDREN WITH ANORECTAL MALFORMATIONS

The aim of the study was to assess the possibility of using a surgical approach – anterior sagittal anoproctoplasty in children who had previously been operated with various techniques for anorectal malformations. Material and methods . The analysis of postoperative results was performed using a scale for assessing the long-term results of treatment of anorectal malformations before and after surgery. A retrospective study of the patients who was previously operated for various forms of anorectal malformations for the period from 2016 to 2019 in pediatric surgery department of State Novosibirsk district hospital was performed. The early and long-term outcomes were assessed. Results. The criteria for repeated surgical treatment were: rectal mislocated outside of the sphincter mechanism, low social adaptation, secondary changes from the perineum and external genital organs. After investigation (identification of the sphincter mechanism using a myostimulator, irrigoscopy, MRI or MSCT of the pelvic organs), all patients underwent surgical correction – the anterior sagittal anoproctoplasty. Postoperative results were assessed using Holschneider score. The results obtained in the course of this study indicate the possibility of using this method of surgical treatment. Discussion. Children previously underwent surgery for anorectal malformations but having severe functional disorders in the anorectal region, are a complex group of patients who must be determined specific criteria for the reoperations need. The efficiency of surgical treatment depends on the presence of associated congenital malformations (myelodysplasia, pathology of the sacrum and tailbone), as well as the severity of cicatricial changes in the sphincter mechanism. Surgical intervention in 33.3 % of cases led to satisfactory and in 66.7 % of cases to good results.

Megarectosigmoid in anorectal malformations: the role of laparoscopic resection.

Surgical treatment for anorectal malformations may lead to chronic constipation or stool incontinence. The first condition is mostly linked to an abnormal dilation of rectum and sigma and it is primarily managed with medical therapy (laxatives, diet and enemas). When medical therapy fails to improve the symptoms, a surgical resection of the dilated colon is advocated. When performing the procedure it is mandatory to consider all the previous operations the patient undergone. We present a laparoscopic left emicolectomy for an extremely dilated megarectosimoid after posterior sagittal anorectoplasty in childhood for a recto-urethral fistula. (www.actabiomedica.it)

ANTERIOR SAGGITAL ANORECTOPLASTY

… Anorectal malformation is the common congenital malformation. Ectopic anusand vestibular fistula are Intermediate types of anorectal malformations (ARM), which are themost common in female babies. Many surgical procedures have been described for the treatmentof ARM. Anterior Saggital Anorectoplasty (ASARP) is not only convenient for the anesthetist formaintenance of anesthesia but also gives better exposure of surgical structures during surgery.Objectives: To determine the technical suitability and outcome of ASARP in intermediate typesof ARM in female children. Design: This Descriptive study with prospective collection of dataaccording to a set protocol. Setting: The study was carried out at the department of PediatricSurgery, Military Hospital, Rawalpindi, Pakistan. Period: November 2010 to March 2014, overthe period of 3 years and 5 months. Patients and Methods: The data of all female patientspresenting with intermediate types of ARM and undergoing ASARP, during the study periodwere analyzed, with respect to age, type, associated anomalies, complications and cosmeticoutcome. Results: A total of 36 patients of intermediate variety underwent ASARP. Age rangedfrom 6 months to 22 years. All patients had colostomy prior to this procedure. During surgery,posterior vaginal wall tear occurred in 2 patients (5.5%). Postoperatively, 2 patients (5.5%) hadretention of urine, 2 patients (5.5%) developed wound infection with superficial disruption, analstenosis occurred in 2 patients (5.5%) and 1 patient (2.7%) had rectal mucosa prolapse. Noneof them required re_ do surgery. Cosmetic outcome was excellent in 31 patients (86.1%), while itwas satisfactory in 5 (13.8%) patients. Conclusions: Anorectoplasty through anterior approachis not only technically easy but has good cosmetic results in intermediate type of imperforateanus in female children.

Bone morphogenetic protein 4 expression in the developing lumbosacral spinal cord of rat embryos with anorectal malformations

Although there are improvements in treatment of anorectal malformations (ARMs), patients can still develop fecal incontinence, constipation, and soiling with loss in quality of life. Recent evidence suggests that malformations in the lumbosacral spinal cord are one of the factors that affect postoperative anorectal function. However, the underlying mechanism that produces these malformations has yet to be elucidated. The bone morphogenetic proteins (BMPs) comprise a large group of highly conserved molecules that are involved in multiple processes and play important roles in the formation, development, and differentiation of the spinal cord. This study was designed to investigate the levels of BMP4 expression in the lumbosacral spinal cord in ARMs rat embryos induced by ethylenethiourea (ETU). Specifically, we assessed the association of BMP4 levels with the maldevelopment of the lumbosacral spinal cord and whether BMP4 acted through the canonical intracellular pathway in embryonic rats with ARMs. BMP4 expression was confirmed with immunohistochemical staining, RT-qPCR and western blot analyses of embryonic day (E) 16, E17, E19 and E21 embryos, moreover Smad1/5 and pSmad1/5 expression were confirmed with western blot analyses at peak time point of BMP4 expression. Our results reveal that BMP4 expression in the lumbosacral spinal cord of ARMs rat embryos is decreased at both the mRNA and protein levels and could decrease the phosphorylation of smad1/5, when compared with their expression levels in normal tissue. These results also suggest that reductions in BMP4 expression were possibly responsible for dysfunction of the lumbosacral spinal cord during late developmental stages in ARMs fetal rats. Taken together, we conclude a role for BMP4 in the pathogenesis of lumbosacral spinal cord maldevelopment in developing ARMs rats.

Fecal Incontinence after Posterior Sagittal Anorectoplasty for Anorectal Malformation: A Single-Center Study.

Background Fecal incontinence is one of the worst functional complications of posterior sagittal anorectoplasty for treatment of anorectal malformation. Objectives In this study, we aimed to identify the prevalence of fecal incontinence in patients with the diagnosis of high or low anorectal malformation who underwent three-stage posterior sagittal anorectoplasty surgery in our center. Patients and Methods Children with the diagnosis of anorectal malformation who underwent posterior sagittal anorectoplasty at the Department of Pediatric Surgery of Besat Hospital, Hamadan University of Medical Sciences, Iran, from 2012 to 2016 were enrolled in the study. Parents or guardians were recruited and asked to fill the study questionnaire including the Templeton and Ditesheim Scoring System to assess the status of fecal continence of the patients. Results Thirty-four patients including 10 (29.4%) males were enrolled in the study. High type of anorectal malformation was diagnosed in 23 (67.6%) patients. The overall mean scores of fecal continence were 4.57 ± 0.84 (range 1.5–5) after a mean follow-up time of 50.7 (range 22.5–69.8) months. Good fecal continence was observed in 91.3% of patients with low type compared to 72.8% of patients with high type of anorectal malformation; however, the difference was not significant (P=0.13). Conclusion Posterior sagittal anorectoplasty surgery in patients with anorectal malformation may result in acceptable fecal continence.

Outcome of Laparoscopic Treatment of Anorectal Malformations in Children.

Operative correction of anorectal malformations (ARMs) remains a challenge in pediatric surgery. The study aimed to evaluate the outcomes of laparoscopic treatment of ARM in children. From 2007 to 2014, we performed 104 laparoscopic-assisted anorectal pull-through procedures in children with a mean age of 11.3±0.4months and ARMs. Clinical assessment, surgical durations, complications, and postoperative outcome were investigated. The mean duration of the operation was 126.5±17.2min. Mean intraoperative bleeding was 20±5.7g. Three (2.9%) children required conversion to laparotomy. One (0.9%) child developed a pelvic abscess, requiring an additional intervention. Three to six months after laparoscopic-assisted anorectal pull-through, 72 patients were hospitalized for stoma closure. Good functional results were achieved in 39 (54.2%) patients. On examination of the perineum, 11 (15.3%) patients were found to have mucosal prolapse. The circular symmetric anal reflex to tactile stimulation was confirmed in 53 (73.6%) patients and a tactile weakened anal reflex in 5 (6.9%) children. At the 1-year follow-up, constipation was present in nine (12.5%) patients and soling in two (2.8%) patients. A barium enema study performed after the operation showed good outcomes in 67 (93.1%) patients and fair outcomes in 5 (6.9%) patients. None had a poor outcome. Our experience confirms that laparoscopic-assisted anorectal pull-through enabled complete correction of ARM in the meanwhile avoiding damage to the rectum and anus. Also, the technique of double bipolar myostimulation of muscle complex in laparoscopic ARM might lead to these better results.

Single-Stage Repair versus Traditional Repair of High Anorectal Malformations, Functional Results Correlation with Kellys Score and Postoperative Magnetic Resonance Imaging Findings

Introduction: Posterior sagittal anorectoplasty (PSARP) is the standard treatment for anorectal malformations. In the present study, the clinical evaluation of anal continence was carried out using Kelly’s scoring system and the results of primary PSARP or abdomino-PSARP were compared with the traditional three-stage procedure and the functional outcome was correlated with the findings of MRI, which was used as an objective method of evaluation.Patients and methods: A total of 40 patients with intermediate and high anorectal malformations were studied and were divided into two groups on the basis of a random number table. The patients in group A were treated with a single-stage operation, whereas the patients in group B were treated with a standard staged operation (either PSARP or abdominoperineal pull-through). After clinical evaluation using the Kelly score, patients were divided into three clinical groups irrespective of whether they were operated in one stage or in three stages. All patients were subjected to MRI at the age of 3 years and the findings were correlated with the clinical scoring system.Result: Patients were categorized according to their Kelly’s scores as follows: group 1: clinically good (score 5–6); group 2: clinically fair (score 3–4); and group 3: clinically poor (score 0–2). The proportions of good development of the muscles (puborectalis, external sphincter muscle, and levator muscle hammock) were 78.9% in group 1, 40% in group 2, and none in group 3. Development of muscles was found to be a significant factor for anal continence. Other significant factors for anal continence are rectal diameter and anorectal angle.Conclusion: Clinical assessment using the Kelly score was similar for the single-stage operation and the staged procedure, and this was supported by MRI findings. Therefore, we recommend the single-stage procedure to achieve a better outcome in intermediate and high anorectal malformation.Keywords: Anorectal Malformations, MRI, Posterior Sagittal Anorectoplasty

One stage correction of anorectal malformations in newborns.

The retrospective analysis of case histories of 35 infants (19 boys, 16 girls), treated in the department of reconstructive surgery of Dnipropetrovsk center of mother and child health in the period 2008-2013 was done. All the patients underwent one-stage treatment of anorectal malformations in neonatal period. Surgery was performed in the first 48 hours of life. Body weight of infants ranged from 2,8 to 4,4 kg (average weight 3,2 kg). Among related conditions there were observed vesico-ureteral reflux (VUR) – in 11 (31,4%), VATER association - in 6 (17.1%), malformations of the spine – in 18 (51,4%), of the heart – in 2 (5,7% ). During cystoscopy fistula in the urinary system was revealed in 16 boys. Surgical treatment was carried out by the posterior sagittal anorectoplasty (PSARP) by A.Pena. 2 patients additionally underwent laparotomy. Among 16 patients with fistula in the urinary system, prostatic urethra fistula was revealed in 14, bladder neck fistula – in 2. Children were discharged from the hospital 2 weeks after surgery. All the children re­ceived anti­biotics due to the presence of VUR. After 1 month after surgery VCUG was performed. Long-term results were studied for a period of 10 to 24 months. All patients had VUR without evidence of hydronephrosis. In the period of 3-6 months after surgery 2 patients presented anal stenosis due to violation of dilatation. The evaluation results in the late period (10-24 months after surgery) noted the presence of normal stool 2-3 times a day, 3 children had periodic con­stipations treated by diet. Available merits of the described method: only one operation and general anesthesia is per­for­med, no risk of prolonged contamination by pathogenic flora of the urinary system through the fistula, potential risk of complications of laparotomy and colostomy decreases, fistula visualization in cystoscopy allows to avoid the X-ray.

Fifteen years of experience in the treatment of anorectal malformations

To analyze our experience in the treatment of anorectal malformations (ARM) with the posterior sagittal anorectoplasty (PSARP), and our modifications through the last few years and the outcomes. We reviewed 107 cases divided into two groups: Former (F: 1994-2003) and Recent (R: 2004-2008). Type of ARM, associated anomalies, management and complications were noted. A telephone questionnaire regarding continence outcome was addressed to the 74 cases older than 3 years. According to the type of ARM, there were 53 perineal fistulas, 2 anal stenoses, 11 no fistulas, 12 rectourethral fistulas (5 rectobulbar and 7 rectoprostatic fistulas), 22 vestibular fistulas, 1 rectovesical fistulas and 6 cloacas. A total of 47 patients presented with 73 associated malformations. As much as 45 colostomies were performed, including 5 perineal fístulas, with 6 of 7 vestibular fístulas in group F and only 8 of 15 in group R. We had 19 complications of PSARP. The most frequent one was rectal mucosa prolapse in 14 (12F and 2R) and 2 wound infections (F). Continence was good in 62, poor in 3 and fair in 5. Seven out of eight children with poor or fair continence had associated malformations. All perineal fístulas can be managed without colostomy. Vestibular fístulas can be safely treated without colostomy in otherwise healthy patients without severe malformations. Overall, continence is good, and fair/poor results are related to associated malformations. Cumulative experience helps avoid colostomies and reduce complication and reoperation rates.

Early results of the posterior saggital anorectoplasty in the treatment of anorectal malformations in Nigerian children

CONTEXT: The ultimate goal of reconstruction in anorectal malformations (ARM) in children, is to create a functional and anatomically aesthetic neoanus. AIMS: To document our experience with the technique of PSARP in ARM and the immediate anatomical and functional outcome SETTINGS AND DESIGN: A retrospective study 39 patients with high and intermediate ARM, managed by the posterior saggital approach, at the Obafemi Awolowo University Teaching Hospital in Ile Ife, Nigeria MATERIALS AND METHODS: Patient's bio-data were collected, as well as, the presentation, investigations, associated malformations, complications, average bowel opening per day and follow up. RESULTS: There were 17 males and 22 females (male: female ratio 1: 1.3). Twenty-three patients (59%) and 15 patients (38.5%), had intermediate and high malformations respectively, while 1(2.5%) had cloaca malformation. The mean age at presentation was 13.9±30.1 month (range 2 day to 15 years), while the mean age at PSARP was 21.6±29.9 months (range 2.5 days to 15 years). 3(7.6%) patients required laparotomy to mobilize the rectal pouch from the abdomen. Mucosal prolapse was the most common significant postoperative problem, occurring in 4(10.3%) patients. The average bowel motion was 3 times daily (range 1 to 8 times per day). CONCLUSIONS: The PSARP is a useful procedure for the correction of ARM, in children in developing countries, even in the absence of muscle stimulators. The immediate anatomical outcome is satisfactory, but functional results are difficult to access because of poor follow up.

Anorectal anomalies in Ilorin, Nigeria: a 10-year review

Background: The treatment of anorectal malformations has been a difficult challenge for surgeons and there is may be a need to modify management in the light of new developments. Method: Forty-five patients admitted with anorectal malformations from January 1991 to December 2000 were retrospectively studied. Results: There were 26 males and 19 females. Twenty-one (80.8%) males presented within three days of birth with lower large bowel obstruction. Only 7 (36.8%) of the females presented within the first week with abnormal anal orifices. Associated anomalies occurred in 12 (26.7%) patients. Fifteen patients (33.3%) had high type of anomalies, 14 (31.1%) intermediate, and 11 (24.4%) low type. Nineteen males (73% of males), and 11 females (60% of females) had initial diverting colostomies. Only 8 males (30.8% of the males) and 9 females (47% of the females) had definitive operations and eventual colostomy closure. Nine males died in the neonatal period, 2 discharged against medical advice, 2 were lost to follow-up after attending the clinic with colostomy for over a year, 4 were awaiting definitive operation at the time of the review. One female died after neonatal colostomy, 2 refused colostomy, 4 were lost to follow-up (3 with colostomy, 1 without colostomy) 2 were referred to other hospitals. Conclusion: Colostomy is socially unacceptable in many third world countries. Colostomy related morbidity and mortality are high. Preliminary colostomy may be unnecessary in most females and certain categories of males. Definitive operations should be done early so that patients carry colostomy for as short a period as possible. Key Words: Anorectal malformations, posterior sagittal anorectoplasty, colostomy Nigerian Journal of Surgical Research Vol.5(1&2) 2003: 148-151

Preoperative Enzymo-Histochemical Diagnosis of Dysganglionoses Associated with Anorectal Malformations (ARM) with Recto-Vestibular and Recto-Perineal Fistula

The posterior sagittal anorectoplasty (PSARP) is widely recognized as the best technique available today for the surgical treatment of anorectal malformations (ARM). However, different retrospective studies on the functional results of PSARP in the treatment of ARM have shown different postoperative degrees of constipation. In particular, even in patients with normal sacrum, about 70% of operated ARM with vestibular fistula and about 50% with perineal fistula can be complicated by fecal constipation and pseudoincontinence. In order to identify preoperatively whether ARM patients present abnormal innervation patterns of rectal pouch and fistula (as reported by Holschneider et al [7]), we decided to study suction rectal biopsies performed by introducing SBT-100 rectal suction biopsy tool into the fistula at 6, 4, 3 and 2 cm from the meatus. To date, this approach has been adopted in 22 ARM cases (15 females and 7 males, age range 7 days-4 years), 6 of them with recto-vestibular fistula and 13 with recto-perineal fistula. Biopsies were frozen in isopentane at liquid nitrogen temperature and cryostatic sections were studied by acetylcholinesterase (AChE), succinic-dehydrogenase (SDH) and alpha-naphthyl-esterase (ANE) enzymo-histochemical techniques. The results concerning the innervation-type of fistula and proximal rectal pouch were confirmed by the biopsies obtained during PSARP. Our overall incidence of rectal innervation intrinsic disorders was 81.82%. In particular, all our cases of vestibular fistula presented associated dysganglionoses. The incidence of associated Hirschsprung's disease was high, corresponding to 18% of cases. Our results suggest that the high frequency of constipation in low forms of ARMS depends on primary intestinal neuronal malformations and it cannot be ascribed to a denervation secondary to rectal dissection and to PSARP procedure. We propose the introduction of this type of preoperative enzymo-histochemical diagnosis in ARM cases because it can select those patients with severe associated dysganglionoses. In our opinion, if this diagnosis is available preoperatively, PSARP can be performed without using abnormally innervated structures and reducing postoperative functional complications.