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.

Tubular sigmoid duplication in an adult man: an interesting incidental finding.

A 61-year-old man attended an outpatient colorectal clinic for a chronic, non-specific abdominal pain, associated with rectal bleeding. He underwent a number of investigations including a CT pneumocolon, which revealed an incidental finding of 20 cm of additional sigmoid colon. This case is interesting because tubular sigmoid duplication is an extremely unusual condition, rarely diagnosed in adults; only a few cases have been reported of this condition in the adult population. Our team chose to treat this patient conservatively, in order to avoid putting the patient at risk of an unnecessary surgery.

Primary sigmoidectomy and appendicostomy for chronic idiopathic constipation.

PURPOSE: To present our experience in patients with chronic idiopathic constipation complicated by megarectosigmoid and fecal incontinence who underwent a primary sigmoidectomy and appendicostomy. METHODS: We reviewed eight patients referred to the Colorectal Center from 2014 to 2016 with chronic idiopathic constipation and undergoing a sigmoidectomy and appendicostomy. We analyzed the previous medical treatment, indications for the surgical procedure, and outcomes. RESULTS: Age at operation was 5-19 years. Time with constipation was 4-15 years. All patients received multiple laxatives, mainly polyethylene glycol, and all had severe social problems. Four patients have autism. The indication for surgery was an unsuccessful laxative trial, refusal to continue with rectal enemas or both, and social fear of continued fecal incontinence. Post-operatively, all patients were having daily bowel movements without fecal accidents. CONCLUSION: Selected patients with chronic idiopathic constipation complicated by megarectosigmoid and fecal incontinence can obtain great benefit from primary sigmoidectomy and appendicostomy.

A de novo microtriplication at 4q21.21-q21.22 in a patient with a vascular malignant hemangioma, elongated sigmoid colon, developmental delay, and absence of speech.

The widespread application of array comparative genomic hybridization (aCGH) has provided new insights into the clinical significance of copy number variations (CNVs) in the human genome. Many microdeletion syndromes have recently been linked to corresponding reciprocal microduplication syndromes related to CNVs in the same chromosomal regions. However, the extent of CNVs may not be restricted to only microduplications but may also include microtriplications or even quadruplications. 4q21 microdeletion syndrome is one of these recently described syndromes. The phenotype includes growth restriction, neonatal hypotonia, severe developmental delay, absent or delayed speech, and distinct facial features. The minimal critical deleted region, which is 1.3 Mb in size, contains the PRKG2, RASGEF1B, HNRNPD, HNRPDL, and ENOPH1 genes. Here, we report a 5.4-year-old girl with developmental delay, absence of speech, muscular hypertension, macrocephaly, a broad forehead, frontal bossing, relatively elongated extremities, a vascular malignant hemangioma in anamnesis, and elongated sigmoid colon. aCGH revealed a microtriplication at 4q21.21-q21.22 that was 1.61 Mb in size. This de novo microtriplication included nine genes (BMP3, PRKG2, RASGEF1B, HNRNPD, HNRPDL, ENOPH1, TMEM150C, LINC00575, and SCD5) and overlapped with the minimal critical region for 4q21 microdeletion syndrome. Some clinical features of the patient were similar to those of 4q21 microdeletion (macrocephaly, frontal bossing, developmental delay, absence of speech, and anxiety), whereas others were mirrored (elongated extremities and muscular hypertension). The first identified case of a de novo microtriplication at 4q21.21-q21.22 emphasizes the clinical significance of CNVs at 4q21 for patients with developmental delay and absence of speech. © 2016 Wiley Periodicals, Inc.

Vaginal reconstruction with sigmoid colon in patients with congenital absence of vagina and menses retention: a report of treatment experience in 22 young women.

INTRODUCTION AND HYPOTHESIS: We evaluated the surgical feasibility, sexual satisfaction and complications of vaginal reconstruction with sigmoid colon in patients with congenital absence of vagina and menses retention. METHODS: Retrospective analysis of surgical techniques and long-term postoperative follow-up was performed for 22 patients who underwent vaginal reconstruction with sigmoid colon at a single hospital between 1977 and 2011 to treat congenital absence of vagina with menses retention. RESULTS: All patients achieved satisfactory sexual function after marriage. No patients experienced enterospastic abdominal pain during sexual intercourse. The neovaginas accommodated two or more fingers and had depths >10 cm. The mucous membranes were soft and flexible, and secretions of the sigmoid mucosa provided adequate and acceptable lubrication. No patient required vaginal stents, and none developed vaginal stenosis or reported pain with vaginal expansion. Fifteen of the 22 patients underwent hysterectomies due to cervical agenesis; seven retained their uterus and had onset of normal menses postoperatively. Two patients became pregnant 1 year after marriage; one achieved 38-week gestation, underwent cesarean section due to premature rupture of membranes, and delivered a healthy boy. The other experienced natural incomplete abortion and underwent curettage at her local hospital. CONCLUSION: This study confirms that sigmoid colon vaginal reconstruction is a good choice for treating congenital absence of vagina and menses retention and results in the closest approximation to the physical function of a normal female vagina. Reproductive ability can be retained in many cases for patients with a well-developed uterus and cervix.

Rectal and sigmoid atresia: transanal approach.

We report 2 patients with rectal and low sigmoid atresia operated on, respectively, at 6 and 3 months of age using the transanal approach, similar to the transanal technique for Hirschsprung disease, after exploratory laparotomy with colostomy at birth. There were no intraoperative or postoperative complications after a follow-up time of 2 years. After closure of the colostomy, both patients had no fecal incontinence. The transanal approach is a safe and effective technique in the management of rectal and sigmoid atresia.

Concomitant urethral triplication, bladder, and colon duplication.

The concomitant presence of urethral triplication and caudal duplication is extremely rare with no previous reported cases. We report a case of urethral triplication associated with bladder, sigmoid, and rectum duplication. The patient was initially referred with a history of fecaluria and recurrent urinary tract infection. Physical examination revealed 2 meatal opening on the glans penis. Further investigation revealed three distinct urinary streams, two terminating on the glans penis, and one in the rectum in voiding cystourethrography and retrograde urethrography. Computed tomography demonstrated the bladder divided into two compartments by a complete sagittal septum. The patient was managed by the excision of the rectal ending urethra and removal of the bladder sagittal septum during which, two sigmoidal and rectal segments (the right one filled with fecal) were revealed. The right sigmoid and rectum was resected. The two ventral urethras were kept intact. The postoperative course was uneventful. At his 4 month readmission for colostomy closure, the patient reported good urethral voiding with no complication and recurrence of urinary tract infection and the colostomy was closed with no major complication.

Segmental dilatation of sigmoid colon in a neonate: atypical presentation and histology.

Segmental dilatation of the colon is a rare disorder of colonic motility in children, often presenting with severe constipation in older infants, children, and occasionally adults. It may mimic the commoner Hirschsprung disease clinicoradiologically but differs in that the ganglion cell morphology and distribution are typically normal in the colon. We report a neonate with segmental dilatation of the sigmoid colon who had an atypical clinical presentation and describe certain abnormalities in bowel histology (hypertrophied muscularis propria, nerve plexus, and ganglion cells located within the circular layer rather than the normal myenteric location), for the first time in the English literature.

Laparoscopic resection of Y-shaped tubular duplication of the sigmoid colon: report of a case.

Tubular-type colonic duplications are rare congenital malformations that are usually diagnosed during childhood. We report the first case of a Y-shaped tubular sigmoid colonic duplication in an adult who was successfully treated by laparoscopic resection.

Rectosigmoid tubular duplication presenting as perineal sepsis in a neonate.

Tubular rectal duplication is a very rare congenital anomaly. We report a case of tubular rectal duplication in a newborn baby who presented with perianal sepsis. The diagnosis was confirmed by barium enema, magnetic resonance imaging, and at operation. We performed total mucosectomy through a posterior sagittal incision combined with laparotomy. The patient was doing quite well at 17-month follow-up examination.

Individualized management of upper rectal atresia.

BACKGROUND/PURPOSE: Congenital colonic atresia (CA) or stenosis is an infrequent cause of low intestinal obstruction in the neonate. Atresias can occur at any level, and the management of CA is determined by the atretic site and by the presence or absence of associated anomalies. We report our experience dealing with upper rectal atresia during a 5-year period. METHODS: Between January 2004 and December 2008, 3 female newborns with upper rectal atresia with or without associated anomalies were treated. Modes of clinical presentation, methods of diagnosis, associated anomalies, alternative management techniques, and clinical outcome were retrospectively analyzed. RESULTS: All 3 patients had progressive abdominal distension, bilious vomiting, and failure to pass meconium. Contrast enema showed an atresia at the upper rectum in 2 patients. At laparotomy, case 1 was found to have type III atresia of the upper rectum. Resection of the dilated portion of the proximal colon with end sigmoid colostomy was accomplished in the neonatal period followed by a transanal mucosectomy with takedown of the colostomy and a pull-through procedure at age 3 months. Case 3 had multiple jejunoileal atresias and an upper rectal atresia. The initial management was multiple resections of atretic bowel and anastomoses and an end sigmoid colostomy. The secondary procedure was a takedown of the colostomy and transanal mucosectomy with a pull-through procedure. Case 2 had type I upper rectal atresia in association with imperforate anus complicated by colon perforation during performance of a distal colostogram leading to a complicated and protracted clinical course. All the patients are currently well with voluntary bowel movements, and one has occasional soiling with follow-up of 9 months to 3 years. CONCLUSIONS: Colon atresia, especially at the level of the upper rectum, is uncommon. Whether to proceed with an ostomy or to individualize the operative procedure according to the location of the atresia is still controversial. Transanal mucosectomy was a useful technique at the time of the definitive pull-through for the treatment of upper rectal atresia. In cases of upper CA associated with imperforate anus, delay in diagnosis and potential complications may result if the diagnosis of upper rectal atresia is missed.

An unusual presentation of a cystic duplication of the sigmoid colon entirely lined with squamous epithelium.

Alimentary tract duplications are rare congenital malformations that occur most commonly in the jejunoileal part of the gastrointestinal tract. Management of this pathologic condition is usually drawn up. We report a case of descending colonic communicating duplication in which clinical presentation and anatomopathologic results were unexpected. A slightly echogenic abdominal mass reaching 72 x 36 mm in the left flank was diagnosed in a female fetus during the third trimester ultrasound examination. At birth, volume of the mass rapidly evolved, and despite no intestinal obstruction was observed by compression of the adjacent gastrointestinal tract, abdomen was distended. Abdominal plain film showed a large air collection, and the barium enema demonstrated a slight leak of contrast in the aerated mass, suggesting a communication with the sigmoid colon. No other abnormalities were seen. The patient underwent surgery in emergency. The mass was then totally excised through an antimesenteric resection of the tubular tract joining cystic mass and sigmoid colon. A lateral suture of the colon was subsequently performed. The wall of the duplication is usually composed of a smooth muscle layer covered by an epithelium, mostly of intestinal type. Herein, we describe a descending colonic duplication completely lined with nonkeratinizing squamous epithelium. Therefore, the association of a colonic mucosa (of endodermic origin) and a squamous epithelium (derived from the ectoderm) in our case is an interesting finding and is not explained by the various theories. Furthermore, the clinical characteristics, diagnosis, and treatment of intestinal duplications are discussed with regard to literature.

Sigmoid colon duplication cysts.

Enteric duplication cysts are uncommon congenital anomalies that can occur anywhere along the length of the alimentary tract or nearby organs. Overall, the colon is the least common site of congenital alimentary duplications. Colonic duplication cysts can present with symptoms of diverticulitis and can be confused with acquired giant sigmoid diverticula. We present a case of a sigmoid colon duplication cyst presenting as persistent diverticulitis in an adult male. We review the literature and attempt to differentiate congenital colonic duplication cysts from the more common, acquired giant colonic diverticula.

Anatomy of the sigmoid colon, rectum, and the rectovaginal pouch in women with enterocele and anterior rectal wall procidentia.

This study describes the anatomy of the rectovaginal pouch, the sigmoid colon, and rectum in women with posterior enterocele and anterior rectal wall procidentia. The anatomy of rectovaginal pouch, sigmoid colon, and rectum was described in 36 women with an enterocele (group A) and compared with those of 43 women (group B) without pelvic organ prolapse. Women with previous incontinence or prolapse surgery were excluded. The mean age in group A was 58 years (40-75) and in group B 35 years (19-64; P < 0.001). There were 15 nulliparas in group B. Nine women in group A had an internal anterior rectal wall procidentia, and one woman had an external anterior rectal wall procidentia. In group A, the rectovaginal pouch was significantly deeper, the sigmoid mesocolon at S1 shorter and showed more often a straight course (P < 0.05). These characteristics (termed "grande fosse pelvienne") were present in 23 women (64%) in group A and in 6 (14%) in group B, three of the latter were young nulliparas (P < 0.001). Age, parity, menopausal status, body mass index, constipation, and varicose veins were not associated with a grande fosse pelvienne. The typical anatomy in women with an enterocele and anterior rectal wall procidentia was a sigmoid colon with a straight course and a short mesentery at S1 and a rectovaginal pouch that covered more than half of the vaginal length. It may be a congenital condition and important in the development of an enterocele and rectal wall procidentia.

Laparoscopic resection of duplicated sigmoid colon under the guidance of intraoperative colonoscopy.

Colonic duplications are rare congenital malformations. Complete surgical removal is the treatment of choice. The authors report a case of sigmoid colonic duplication successfully treated by laparoscopic resection under the guidance of intraoperative colonoscopy.