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.

Wandering spleen, gastric and pancreatic volvulus and right-sided descending and sigmoid colon.

Wandering spleen is a rare condition, characterized by a mobile spleen that is attached only by an elongated vascular pedicle, allowing it to migrate to any part of the abdomen or pelvis. Mesenteroaxial gastric volvulus usually occurs in children and may be associated with wandering spleen. Both entities result from abnormal laxity or absence of the peritoneal attachments due to abnormal fusion of the peritoneal mesenteries. Pancreatic volvulus is a very rare anomaly, with only a few isolated case reports described in association with wandering spleen. Anomalous right sided descending and sigmoid colon is a very rare entity and its association with wandering spleen has not been previously reported. We report a case of wandering spleen associated with mesenteroaxial gastric volvulus, pancreatic volvulus and rightward shift of the splenic flexure of the colon and right sided descending and sigmoid colon in a young female.

Enterobacter cloacae pericardial effusion in a frail elderly patient.

We report a case of a frail 82-year-old man with seronegative rheumatoid arthritis and a recent pacemaker insertion, admitted with pulmonary oedema and a symptomatic pericardial effusion. He was treated with diuretics and an urgent pericardiocentesis, a sample from which cultured Enterobacter cloacae. A subsequent abdominal CT scan revealed faecal loading, an abnormal anorectal canal and sigmoid colon and a bowel perforation. Endoscopy, biopsies and histopathology confirmed a diagnosis of cytomegalovirus (CMV) colitis with coexistent fungal infection. The E. cloacae infection was successfully treated with 6 weeks of intravenous meropenem, while the CMV and fungal infections were treated with a combination of valganciclovir and fluconazole. We postulate that the bowel perforation resulted from a combination of CMV colitis, faecal loading and steroid therapy and led to bacterial translocation of E. cloacae and the development of the pericardial effusion. This case represents an unusual pathophysiology for the development of an E. cloacae pericardial effusion.

A case of sigmoid colon duplication in an adult woman.

Colonic duplication is a rare congenital anomaly that is often diagnosed in childhood, but may go unrecognised until adulthood. It often presents with chronic abdominal pain and constipation, and the preoperative diagnosis may be difficult. We present a case of sigmoid duplication in a 33-year-old Indonesian woman who presented with right-sided colicky abdominal pain and vomiting. Clinical examination was unremarkable and radiological investigations raised the possibility of a giant colon diverticulum. The patient underwent exploratory laparotomy that revealed a tubular sigmoid duplication. A sigmoid colectomy with end-to-end anastomosis was performed. She was discharged a week later and remained well at 1 year follow-up. Colon duplications rarely present in adult life and the accurate diagnosis is often made at laparotomy.

[Peculiarities of a large bowel microstructure in children, suffering chronic constipation, caused by its inborn anomaly].

Layer-by-layer electron microscopy investigation of the large bowel microstructure in children, suffering chronic constipation (CHC), caused by inborn anomaly (dolichosigma), was conducted. There were investigated 20 intraoperative specimens of the sigmoid colon intestine. Essential changes in all layers of the large bowel were noted. For example, in CHC in decompensated stage there were characteristic features: changes in mucosal epithelium, inhibition of the goblet cells secretion activity, oedema of lamina propria and submucosal layer, the filamental structures disorders. The gut layers trophic function was disordered by the blood vessels and the hemocapillars wall damage. Destructive changes in the smooth myocytes in muscular layer reflects disorder of the organ contractile function. Destructive changes in the large bowel structure are accompanied by the lymph nodes hyperemia, diffuse leukocytic infiltration, witnessing reaction of lymphoid tissue on pathological changes in the organ. Peculiarities of the CHC clinical course, the dolichosigma staging in children were caused by morphological changes of the large bowel wall. The revealed structural reorganization of the gut wall may be considered as a "critical cusative agents", in which propulsive function of sigmoid colonic intestine is inhibited significantly and do not provide an adequate passage of a large bowel contents.

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.

[Hepatodiaphragmal interposition of the right sigmoid segment--Chilaiditi's syndrome].

Two patients (man and woman aged 28 and 82 years respectively) are reported with accidentally diagnosed Chilaiditi's syndrome and sign (abnormality without clinical manifestation). Pathogenesis of both the syndrome and the sign appears complicated and ambiguous. The abnormality was recognized when the patients visited the clinic for planned medical examination. The patient with Chilaiditi's syndrome periodically complained of abdominal discomfort. His physical examination revealed the "lack" of hepatic dullness and the presence of very loud peristaltic sounds heard through a phonendoscope in the hepatic region. The elderly woman presented without abdominal symptoms. Intestinal interposition was associated with the upper position of the diaphragmatic cupola. Chilaiditi's syndrome requires treatment in case of serious clinical manifestations or symptoms of acute abdomen. This abnormality should be considered if invasive intervention being planned may be fraught with a risk of gut injury. The outcome of surgical correction is as a rule fairly good.

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.