Rectal Pouch Research Articles

More Than Just Stool: Hypereosinophilic Syndrome Presenting as Persistent Diarrhea in a Patient With Ulcerative Colitis

We highlight a case of non-infectious diarrhea that progressed despite supportive care, prompting further workup. A 50-year-old man presented with 1 week of voluminous diarrhea, nausea, emesis, and abdominal pain. His history included ulcerative colitis, primary sclerosing cholangitis, Clostridioides difficile treated via total colectomy with ileoanal pouch, treated disseminated Mycobacterium avium complex, and international travel. On physical exam he had normal vital signs, diffuse abdominal tenderness without peritoneal signs, and a rash. Lab work revealed leukocytosis with eosinophilia, hyponatremia, and elevated creatinine. Lactate, inflammatory markers, stool studies, and computed tomography of his abdomen were normal. Tissue biopsies of the duodenum, ileum, and rectal pouch collected during colonoscopy and push enteroscopy revealed prominent mucosal eosinophilia, confirming the diagnosis of hypereosinophilic syndrome (HES). Treatment with steroids led to significant improvement in all symptoms and blood eosinophilia. This case highlights the importance of aggressive workup of diarrhea with peripheral eosinophilia, especially in patients with ulcerative colitis: waiting 6 months to confirm the diagnosis via blood work would delay treatment. Notably, infection with Strongyloides stercoralis was ruled out in this case prior to treatment. This is an important step in the workup and management of HES to prevent progression of Strongyloidiasis.

Rethinking Fistula Preservation in Anorectal Malformation Surgery: A Histopathological Perspective.

This study aims to assess the histopathological characteristics of the fistulous tissue in males with anorectal malformation (ARM) to determine its suitability for use in constructing a neoanus. This prospective observational study included male ARM patients with recto-urethral fistula. All other types were excluded. The tissue specimens comprised 0.5-2.0 cm of the most distal part of the rectal pouch and the fistulous tissue. Hematoxylin and eosin-stained sections were prepared. Histological features viz. internal sphincter, anal grand and crypts, ganglion cells, presence of subepithelial fibrosis, thickened nerve trunks, and metaplasia were evaluated. Of 65 patients of ARM admitted, 24 met the inclusion criteria. Gross and microscopic internal sphincter was not found in any sample. The anal mucosa was visualized in all cases; however, crypts were irregular and distorted architecture was seen in 4 (16.67%) samples. Urothelial metaplasia was identified in 10 (41.67%) cases. Adequate ganglion cells were seen only in 6 (25%) samples. Significant submucosal fibrosis was seen in all samples. Thickened nerve trunks were identified in 4 (16.67%) samples. All normal anal histological features could not be found together in the fistula tissue. With the absence of normal features, such as internal anal sphincter muscles, and the presence of abnormal histopathological features, such as subepithelial fibrosis, thickened nerve trunks, and metaplasia there are high chances of abnormal bowel function like constipation if the fistulous tissue is used for neoanus construction. It may have an impact on the quality of life of patients.

Infracoccygeal/transperineal window: new method to prenatally diagnose and classify level of anal atresia.

To introduce a two-dimensional sonographic method to assess the fetal anus, and to evaluate the feasibility of this method to diagnose anal atresia prenatally and identify the presence or absence of anoperineal fistula (in males) and anovestibular fistula (in females). This was an observational study of suspected cases of anal atresia referred to a single center in Israel between August 2018 and October 2023. In addition to conventional evaluation of the perineum in the axial plane, fetuses referred to our center for suspected malformation were scanned with a new method termed the 'infracoccygeal/transperineal window'. This window consisted of a midsagittal view of the fetal pelvis, including the distal rectum and the anal canal. Normal anatomy was confirmed when the anal canal was continuous with the rectum and terminated at the expected location on the perineum. In female fetuses, the normal anal canal runs parallel to the vaginal canal and diverges posteriorly, terminating at the perineal skin, distant from the vestibule. In male fetuses, the normal anal canal diverges posteriorly in relation to the corpora cavernosa, terminating at the perineal skin, distant from the scrotum. High anal atresia was identified when a blind-ending rectal pouch was demonstrated in the pelvis without a fistula to the perineum or vestibule. Low anal atresia was determined when a rectal pouch was continuous with an anteriorly deflected fistula. In females, the fistula converges with the vaginal canal, terminating at the vestibule; in males, the fistula deflects anteriorly, terminating at the base of the scrotum. Postnatally, the diagnosis and type of anal atresia were confirmed through physical examination with direct visualization of the fistula, radiographic studies, surgical examination and/or postmortem autopsy. Of the 16 fetuses diagnosed prenatally with anal atresia, eight were suspected to have low anal atresia and eight were suspected to have high anal atresia. The median gestational age at diagnosis was 23 (range, 14-37) weeks. All cases showed additional structural malformation. Eleven patients opted for termination of pregnancy, of which four had low anal atresia and seven had high anal atresia. Postnatal confirmation was not available in four cases due to curettage-induced mutilation or in-utero degradation following selective termination of the affected twin, leaving 12 cases for analysis, of which seven were diagnosed with low anal atresia and five with high anal atresia. In these 12 cases, all prenatal diagnoses were confirmed as correct, rendering 100% sensitivity and 100% specificity in this high-risk fetal population. The infracoccygeal/transperineal window is an effective method to detect and classify the level of anal atresia prenatally. This may improve prediction of postnatal fetal continence and optimize prenatal counseling. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Rectal duplication in adult presented with constipation: a case report and literature review

BackgroundRectal duplication is a rare congenital disease which is mostly diagnosed during childhood. Diagnosis in adulthood could be obscured as the presenting symptoms including difficult defecation, bloating, and abdominal distension would lead to other diagnosis. Constipation is common in female adult and conservative treatment is usually successful. Refractory constipation requires further work-up. However, rectal duplication is a rare cause of adult constipation.MethodsWe reported a 42-year-female who presented with chronic constipation which became intractable after total abdominal hysterectomy. Barium enema, CT scan, and colonoscopy revealed an additional rectal pouch connecting to the native rectum containing large fecaloma. Anorectal manometry showed dyssynergic defecation, rectal hyposensitivity but presence of rectoanal inhibitory reflex.ResultsTransabdominal resection of the rectal duplication pouch and protective colostomy was performed. After a course of biofeedback, the colostomy was reversed. The patient remains healthy at 4 years of follow up.ConclusionRectal duplication could be a cause of refractory constipation in adulthood. A collection of fecal material via a connection with the native rectum led to a large symptomatic fecaloma.

How to approach cases of anal atresia with rectoscrotal fistula: Low or intermediate?

Anorectal malformation is a congenital condition where the anus, which opens into the rectum, is missing, malformed, or has a blockage that does not allow feces to pass from the body. These malformations often present with an enteroperineal or enterourinary tract fistula in male children. Anorectal atresia with rectoscrotal fistula is rare. Herein, we present the case of a neonate with imperforate anus and a fistula on the scrotal raphe. A fistula in front of the scrotum, described in our case, is very rare and not incorporated in the current classification and treatment algorithms. Scarce reports on misjudgment concerning the position of the blind rectal pouch in similar cases, led us to perform a colostomy instead of a one-stage correction. We believe that in cases with a rare fistula presentation, the position of the rectal pouch is not predictable and the surgeon should proceed with caution.

Persistent Cloaca and Cloacal Variants in Males: Qualitative Review of a Neglected Anomaly.

Cloacal malformations are rare and are typically reported in females. There are a few scattered reports in males. It is not clear why they are so rare in males since both sexes negotiate this stage of embryonal development. The present study aims to share our experience and review all the cases of persistent cloaca and cloacal variants in males reported in the literature. The male cloaca is defined as a single common channel of varying lengths with separate inlets for the urinary tract (urethra) anteriorly and the rectum posteriorly at its cranial end and with a solitary perineal orifice/opening for external drainage. We also carried out an electronic literature search for cloaca, persistent cloaca, common cloaca, cloacal dysgenesis, cloacal malformation, cloacal membrane agenesis, urorectal malformation sequence, rectourinary perineal fistula, sirenomelia, and caudal regression syndrome. After eliminating other cloacal anomalies and persistent cloaca in females, we found a total of 22 males with persistent cloaca or cloacal variant reported in the literature. In addition, we are adding two cases we have managed since our previous report. An effort should be made to search for the presence of the common channel in male patients with a single perineal opening. Recognition of the anomaly, width of the common cloacal channel, location of the rectal pouch with relation to the sacrum or pubis, status of the spine and sacrum, and nature of the anal sphincter are vital pieces of information to successfully manage the anomaly. It would be worthwhile if future reports on the subject also include long-term information about urinary and fecal functions and continence.

Curative Effect Analysis of Endoscopic Submucosal Dissection in Giant Laterally Spreading Rectal Tumors.

The objective of this study was to investigate the clinical efficacy of endoscopic submucosal dissection (ESD) in the treatment of giant lateral developing rectal-type tumors (laterally spreading tumors, LSTs). There are no specialized studies on the efficacy of ESD in the treatment of LSTs measuring >5cm in diameter, surgery was often used in the past, but it has the disadvantages of large trauma, many complications, and high cost. The data of 185 patients with rectal LSTs who had undergone ESD in the digestive endoscopy center of our hospital from January 2012 to June 2020 were retrospectively analyzed. Based on the size of the lesions, the patients were divided into 2 groups: diameter ≤5cm (110 cases) and diameter >5cm (75 cases), and we summarized and analyzed the en bloc resection rate, curative resection rate, procedure time, muscle injury, bleeding, perforation, postoperative stricture, and recurrence. There was no difference in the en bloc resection rate and R0 resection rate between the 2 groups ( P =0.531). Moreover, there was no difference in the incidence of delayed perforation, postoperative stenosis, and recurrence, but the incidence of delayed bleeding was significantly higher in the giant LST group than the small LST group ( P =0.001). Moreover, for giant rectal LSTs, the growth pattern of the lesion, JNET classification, and the extent of postoperative mucosal defect do not significantly affect the efficacy of ESD. It is worth mentioning that the operation time was longer in the group with a diameter >5cm, in which perforation was more frequent and the muscle layer was more likely to be injured during ESD ( P <0.001). The muscle injury during ESD was mainly related to the diameter of the lesion, the crossing the rectal pouch, and the operation time. The use of ESD to treat giant rectal LSTs (>5cm) is relatively difficult and can easily lead to intraoperative muscle injury, perforation, and late postoperative bleeding. However, if active intervention is performed, patients can still achieve good efficacy and prognosis, which can be applied in hospitals with certain conditions.

Labeling male anorectal malformations: objective evaluation of radiologic imaging before surgery.

Prone cross-table lateral x-ray (CTLxR) and colostogram aid surgical planning for anorectal malformations (ARMs) without perineal fistulas. We suggest objective imaging tools to classify ARMs. Three observers prospectively evaluated CTLxR and colostograms of male ARM patients (2012-2022) without perineal fistulas. The level of the rectal pouch was estimated with pubococcygeal (PC) and ischiatic (I) lines. On CTLxR, we described the "pigeon sign", defined as the rectal pouch ending with a beak-like image, suspicious for a rectourinary fistula. ARM was defined as rectobulbar when the rectal pouch was below the I line, rectoprostatic when between PC and I lines, and rectovesical when above the PC line. Concordance was assessed with Fleiss' kappa. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the "pigeon sign" were calculated. Thirteen patients were included in this study. The interobserver agreement on CTLxR was 69.2% (k = 0.54) on pouch ending, 84.6% (k = 0.69) on the "pigeon sign", and 76.9% (k = 0.69) on diagnosis; concordance between observers and intraoperative diagnosis was 66.6% (k = 0.56). The "pigeon sign" had 75% sensitivity, 100% specificity, 100% PPV, and 50% NPV. The interobserver agreement on colostograms was 84.6% (k = 0.77) on pouch ending and 89.7% (k = 0.86) on diagnosis; concordance between observers and intraoperative diagnosis was 92.3% (k = 0.90). PC and I lines and the "pigeon sign" are useful tools in examining CTLxR and colostograms. Adequate CTLxR interpretation may modify surgical strategy.

Endoscopic and chemopreventive management of familial adenomatous polyposis syndrome.

Familial adenomatous polyposis (FAP) is an autosomal dominant syndrome predisposing affected individuals to gastrointestinal (GI) cancers through a high burden of polyposis. Colorectal cancer rates reach 100% by the age of 45, making early colectomy a mainstay of treatment. While most patients undergo colectomy at an early age, ongoing screening and surveillance of the upper gastrointestinal tract and rectal pouch must continue throughout adulthood. Endoscopic therapy of gastric, duodenal, ampullary and rectal pouch polyps is critical to reduce morbidity and cancer related mortality. Management of these lesions is not uniform, and is dependent on their location, size, histology, and risk of malignant potential. Medical therapies targeting pathways that reduce the malignant progression of pre-cancerous lesions have been studied for many years. While studies on the use of aspirin and non-steroidal anti-inflammatories (NSAIDs) in chemoprevention have shown encouraging results in Lynch syndrome and primary colorectal cancer, the potential benefits of these medications have not been duplicated in FAP cohorts. While data remains limited on chemoprevention in FAP, a number of randomized trials are currently underway examining targeted therapies with the potential to slow the progression of the disease. This review aims to provide an in-depth review of the literature on current endoscopic options and chemopreventive therapies targeting FAP. While the endoscopic management has robust data for its use, chemoprevention in FAP is still in its infancy. The complementary use of chemopreventive agents and endoscopic therapy for FAP patients is quickly becoming a growing and exciting area of research.

Single Stage Reconstructive Surgery to Treat Anorectal Malformations in Neonates; Ten Years Experience

Objective: To present single surgeon’s ten years’ experience of Single stage reconstructive surgery in 26 neonates born with anorectal malformations (ARMs) and review of literature. Methodology: This is a retrospective study of 26 neonates admitted in NICU with diagnosis of ARMs between period of June 2011 to Oct 2021 and managed by single stage reconstructive surgery with an average follow up of three years. All these patients were full term. The diagnosis was confirmed on clinical examination supported by cross table lateral film in prone position after 24 hours and ultrasound evaluation of level of rectal pouch. Single stage reconstructive surgery was done under general anesthesia within 24 to 48 hours after birth. The outcome of single stage reconstruction of neonates with anorectal malformations is evaluated post operatively during an average regular follow up of 3 years. Results: All the 26 neonates are treated by single stage reconstructive surgery between 24 to 48 hours after birth during the period from June 2011 to Oct 2021 who were admitted with anorectal malformations in NICU after excluding associated congenital anomalies.  All the patients had excellent cosmetic and functional outcome without significant problem of wound infection. Only 3 female patients with recto-vestibular fistula had mild superficial wound infection and were managed conservatively. Conclusion: Single stage reconstructive surgery to treat ARMs in neonates I effective, safe and feasible with good continence. It avoids morbidity and higher cost associated with three stage surgeries and colostomy. This fact may further be confirmed through multi-institutional experience in large number of patients.

Embryology and anatomy of anorectal malformations

The etiology of anorectal malformations (ARM) is complicated because of the spectrum of anomalies seen clinically, many of which are isolated and seemingly unique. Recent studies suggest that ARMs result from abnormal cloacal membrane (CM) development that contributes to disrupt normal local muscle and nerve development. If CM maldevelopment is severe, the rectal pouch lies above the pelvic floor, resulting in asymmetric and/or deviated musculature, so a midline incision is likely to cause trauma or be detrimental. Autonomic nerve plexuses can be associated with a fistula tract in ARMs and are also at risk for damage during surgery and contribute to genitourinary complications. Understanding the anatomy and development of the perineum is crucial for treating the broad spectrum of anomalies associated with ARMs and may assist in predicting/managing other related morbidity.

Robotic surgery in Hirschsprung disease: a unicentric experience on 31 procedures.

Robotic surgery has been increasingly applied to Hirschsprung patients with encouraging results. We report the results of a 5year unicentric experience. All consecutive HSCR patients older than 12months who underwent a surgical procedure with robotic approach between September 2017 and August 2022 were prospectively included. We collected data regarding demographics, extent of aganglionosis, associated anomalies, indications to surgery, and a number of perioperative data such as surgical details, intraoperative and perioperative complications, length of surgery, length of hospital stay, and functional outcome. A total of 28 patients underwent 31 robotic procedures during the study period. Median age at surgery was 82months. Eleven primary Totally Robotic Soave Pull-Through, 12 redoes, 5 innervative mapping, 2 redundant rectal pouch excision, and 1 Miles' procedures have been performed. Median console time was 145min. No conversion to either laparoscopy nor to laparotomy was required. Median length of hospital stay was 6days. Two patients experienced complications requiring reiterative surgery. One patient experienced mild postoperative enterocolitis. Normal continence was achieved by 70% of patients after a median of 16months postoperatively (80% for primary pull-throughs, 55% for redoes). To conclude, robotic surgery for older HSCR patients proved to be feasible, safe, and effective. Patients with complex surgical requirements seem to benefit most from this promising approach. Provided the economic burden is addressed and solved, robotic surgery will represent an excellent alternative for the surgical treatment of HSCR patients.

Perirectal Abscess and Fistula Secondary to a Fecalith in Rectal Pouch Occurring 51 Years After Duhamel Operation for Hirschsprung’s Disease: Minimally Invasive Treatment

Perirectal Abscess and Fistula Secondary to a Fecalith in Rectal Pouch Occurring 51 Years After Duhamel Operation for Hirschsprung’s Disease: Minimally Invasive Treatment

S2123 Refractory Case of Malignant Eosinophilic Colitis Leading to Ischemic Colitis

Introduction: Eosinophilic colitis (EC) is a rare inflammatory condition involving eosinophilic infiltration of the mucosa in the absence of a secondary cause or peripheral eosinophilia. Clinically, EC presents with non-specific symptoms. We present a case of malignant EC that is refractory to different therapies. Case Description/Methods: A 30 years old male with known EC on azathioprine, pulmonary embolism and superior mesenteric venous (SMV) thrombosis onrivaroxaban, had multiple admissions with abdominal pain and hematochezia secondary to Clostridioides difficile (C. diff). His rivaroxaban and azathioprine were held given his hematochezia and recurrent infections. He presented a month later with worsening symptoms but C. diff was negative. CT abdomen/pelvis showed non-occlusive SMV and portal venous (PV) thrombosis with signs of ischemia of the sigmoid colon (Figure 1b). Patient underwent percutaneous transhepatic SMV/PV aspiration thrombectomy that was complicated by a large right hemothorax, causing hemorrhagic shock. Subsequent flexible sigmoidoscopy showed signs of colonic ischemia (Figure 1a). Colonic biopsies demonstrated active colitis without evidence of chronicity, and 60 eosinophils per high powered field. Patient underwent a subtotal colectomy with end ileostomy with clinical improvement immediately postoperatively. He was discharged on a steroid taper and apixaban. He was started on the interleukin-5 (IL-5) inhibitor benralizumab for his EC. Outpatient follow-up was notable for continued rectal bleeding. Flexible sigmoidoscopy showed diffuse severe erythema, contact bleeding, and friable mucosa throughout the rectal pouch (Figure 1c). Vedolizumab was started in combination with benralizumab, however, on long-term outpatient follow-up, no notable clinical or endoscopic improvements were noted on combination therapy. Multi-disciplinary decision in conjunction with patient resulted in planned proctocolectomy with permanent end ileostomy. Discussion: There are currently no FDA approved management options for EC. The IL-5 inhibitor Benralizumab is FDA approved for treatment of eosinophilic asthma and therefore it was worth a trial in this patient. In a small number of case reports, Vedolizumab has shown some clinical improvement as well as reduction in eosinophilia in EC patients. Due to the rarity of EC diagnosis, data for therapies in EC is severely lacking. To our knowledge, this is the first case of a patient with severe refractory EC that led to the development of ischemic colitis.Figure 1.: (a) shows an endoscopic image of ischemic colitis. (b), the red arrow points at the portal vein thrombosis and (c) shows diffuse severe erythema, contact bleeding, and friable mucosa throughout the rectal pouch.

Laparoscopic-Assisted Anorectoplasty for Rectovestibular Fistula: A Comparison Study with Anterior Sagittal Anorectoplasty.

Posterior or anterior sagittal anorectoplasty (ASARP) is the mainstream for correcting rectovestibular fistula (RVF). However, the intermediate RVF has the potential risk of wound complications when applying ASARP due to its high rectal pouch, long fistula tract, and difficulty separating the rectum and vagina. We developed laparoscopic-assisted anorectoplasty (LAARP) for surgical correction of RVF, which has acceptable preliminary outcomes. The purpose of this study is to evaluate the safety and efficacy of LAARP in comparison with ASARP for patients with RVF. Twenty-five patients with RVF who underwent LAARP between October 2017 and December 2020 were retrospectively reviewed. The outcomes were compared with 43 patients who underwent ASARP between April 2015 and August 2018. The age, weight at operation, sacral ratio, operative time, and postoperative hospital stay were evaluated. The results were assessed for complications, perineum appearance, and bowel function. The two groups were comparable in terms of demographics. The median operative time of the LAARP group was significantly longer than that of the ASARP group (113 vs. 95 minutes; p = 0.015). The mean length of the resected rectum in the LAARP group was also longer than that in the ASARP group (6.75 ± 5.07 vs. 3.31 ± 3.06 cm; p = 0.001). Compared with the LAARP group, complications in the ASARP group were more frequent (4.0 vs. 27.9%, p = 0.036). No intraoperative or postoperative wound-related complications occurred in the LAARP group. However, in the ASARP group, one patient had an intraoperative vaginal injury and four had postoperative anastomosis-related complications. The incidence of redo operation in the ASARP group was significantly higher than that in the LAARP group (p = 0.000). Cosmetic satisfaction was higher in the LAARP group (96.0 vs. 76.7%; p < 0.05). In terms of voluntary bowel movement, soiling, and constipation, the LAARP group had similar results compare with the ASARP group. The LAARP technique has shown several unique strengths in treating intermediate type RVF, including lower risks of complications, and minimal muscular injury, with a comparable bowel function.

A 73-year-old woman with delayed intra-abdominal and systemic sepsis following complicated aortobifemoral bypass

A 73-year-old woman with delayed intra-abdominal and systemic sepsis following complicated aortobifemoral bypass

Vestibular anus, a surgical dilemma

It is a pleasure for me to draw attention in this issue of your admirable journal to recto-vestibular fistula. Vestibular anus means congenital presence of the anus in the vestibule behind the vaginal orifice. It shares a common wall between the vagina and the rectum. In addition to an abnormal cosmetic appearance, it leads to recurrent attacks of urinary tract and vaginal infections. In adult female patients, it leads to dyspareunia. Several techniques have been described for the treatment of this type of anomaly, however; the postoperative continence is not always satisfactory. Optimal surgical repair would be easy to assess minimum dissection to spare pelvic nerves and guide the rectal pouch through the muscles of continence and intact sphincter [1]. The surgeon must cautiously during surgical separation of the posterior vaginal wall from the anterior rectal wall to avert rectal wall tear.

Successful treatment of type III atresia ani and rectovaginal fistula in a kitten

A six-weeks-old, female kitten presented with dehydration, depression, atresia ani and a discharge of watery feces from the vulvar opening. On radiographic examination, the blind-end of the rectum was determined to be &gt;1 cm cranial from the anal dimple, classifying it as type III atresia ani. Atresia ani combined with a rectovaginal fistula is an uncommon congenital defect of the terminal rectum and anus that can occur in kittens. After supportive medical treatment for three days, the patient was strong enough for surgical correction. The distal rectal pouch was connected to the unperforated anus and the rectovaginal fistula was closed. The kitten recovered uneventfully and was able to defecate normally at 38 weeks follow-up. In this case report, the importance of early surgical correction to avoid potential life-threatening complications from this congenital abnormality is demonstrated.

Robot-assisted anorectal pull-through for anorectal malformations with rectourethral and rectovesical fistula: feasibility and short-term outcome.

Multiple structures in the anorectal area are closely related to defecation, voiding and sexual function. Although laparoscopic-assisted anorectal pull-through is widely accepted as a minimally invasive surgical technique, controversy still exists for ARMs with rectourethral fistulas. Intraoperative injuries more or less involve the perirectal sphincters and neurovascular tissue. Seventeen selected infants with ARMs underwent robot-assisted anorectal pull-through (RAARP) between October 2016 and January 2018. The application of nerve- and sphincter-sparing technique in RAARP was detailed. The feasibility and early outcomes were evaluated. All procedures were completed without conversion. The robotic system facilitated clear dissections between different anatomical layers. Under direct vision, the fistula was easier to repair, and the rectal pouch was precisely placed in the center of the striated muscle complex. During the follow-up of 11.6months, 13 patients resumed normal defecation. The other four children experienced mild constipation or fecal incontinence. Their continence and defecation functions showed favorable evolution. RAARP is a safe and effective alternative for the treatment of ARMs, which provides an advantage in further minimizing the injury to perirectal nerves and sphincters.

Currarino Syndrome (SC): A case at the University Hospital of Mother and Child Lagune Cotonou

Introduction: The Currarino syndrome (CS) is one of the rare syndromic forms of anorectal malformations (ARM). Observation: This is the clinical case of a 3 days male newborn admitted for high ARM in the pediatric surgery Department of the Lagune Mother and Child University Hospital of Cotonou. An emergency colostomy was performed. At the age of 7 months as a prelude to anorectoplasty, the distal colography revealed a semilunar pelvic opacity in front of the sacrum. The posterior sagittal approach according to Peña could not reveal the distal end of the intestine. The reconstruction of the sphincter-muscular complex was done on a tracheal tube. The abdominal approach allowed the discovery and resection of a presacral mass. The rectal pouch end found was then lowered. Post-operative follows up was uneventful. The standard postoperative X-ray revealed a partial agenesis of the sacrum. The histology of the operative specimen found a cystic lipoma. The diagnosis of CS was done. The search of a HLXB9 mutation was not possible. Conclusion: The clinical observation associated with standard radiological investigations highlighted the triad: anorectal malformation, presacral tumor, and partial agenesis of the sacrum, which upheld the diagnosis of CS; but the search for an indispensable HLXB9 mutation was lacking for the genetic link.