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Query: UMLS:C0042961 (volvulus)
 4,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cecal volvulus occurring in a child with CHARGE syndrome is presented. This boy was known to have CHARGE syndrome with multiple congenital anomalies, including coloboma, ventricular septal defect, choanal atresia, growth and mental retardation, bilateral cryptorchidism, dysplasia of the right ear, cleft lip, and hydrocephalus. Nissen's fundoplication had been previously performed for severe hiatal hernia and gastroesophageal regurgitation at the age of 1 year. Cecal volvulus occurred with a 540-degree clockwise rotation of terminal ileum to the right transverse colon and a displacement of the rotated loop to the right upper quadrant of the abdomen when he was 10 years old. Right hemicolectomy with divided ileo- and colostomy was performed. A second staged ileocolostomy was performed uneventfully 3 months later. The midline structural defects with nonfixation of the cecum and ascending colon, chronic constipation, and previous abdominal surgery might have been the predisposing factors.
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PMID:Cecal volvulus in a child with CHARGE syndrome. 1667 64

X-linked alpha thalassemia mental retardation (ATR-X) syndrome is associated with profound developmental delay, facial dysmorphism, genital abnormalities, and alpha thalassemia. Patients with ATR-X syndrome frequently present with gastrointestinal problems, in particular feeding difficulties, regurgitation and vomiting, abdominal pain, distension, and chronic constipation. Parental reports of prolonged food refusal and distress in these children are common and although these episodes are suspected to be gastro-intestinal in origin they are rarely investigated. Death in early childhood from aspiration of vomitus or from pneumonia presumed to be secondary to aspiration has been recorded in a number of ATR-X cases. In this report we review the gastrointestinal phenotype of ATR-X syndrome in 128 cases. We also demonstrate that in two siblings, regurgitation was secondary to gastric pseudo-volvulus, a condition in which the stomach does not have a normal system of peritoneal ligaments and changes position with possible torsion around itself. Furthermore, ultra-short Hirschsprung disease with colonic hypoganglionosis was shown and this may contribute to the severe constipation affecting these children.
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PMID:Gastrointestinal phenotype of ATR-X syndrome. 1668 41

Sigmoid volvulus is a rare presentation of Hirschsprung's disease. A 38-year-old male presented with acute intestinal obstruction and a history of chronic constipation since childhood. Abdominal radiographs showed megarectum and megacolon with dissipated feces. Sigmoidoscopy revealed gangrenous bowel mucosa affecting the sigmoid colon. Emergency laparotomy revealed a grossly dilated bowel with concurrent gangrenous sigmoid volvulus. He was treated successfully with proctocolectomy with J-pouch-anal anastomosis and a defunctioning ileostomy. Histological analysis was consistent with short segment Hirschsprung's disease. Although uncommon, adult Hirschsprung's disease is a cause of chronic constipation and can present acutely with a sigmoid volvulus. Mortality in cases with sigmoid volvulus is greater than in cases without (15.4% vs. 0%). A better awareness of this condition will facilitate management.
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PMID:Adult Hirschsprung's disease presenting as sigmoid volvulus: a case report and review of literature. 1696 8

Patients with intractable chronic constipation should be evaluated with physiologic tests after structural disorders and extracolonic causes have been excluded. Conservative treatment options should be tried excessively. If surgery is indicated subtotal colectomy with IRA is the treatment method of choice. However, segmental resection may be a good option for isolated megasigmoid, sigmoidocele or recurrent sigmoid volvulus. In general patients with GID should not be offered any surgical options because of their anticipated poor results. Moreover, patients with psychiatric disorders should be actively discouraged from resection as they tend to have poorer prognosis. Patients must be counseled that preoperative pain and/or bloating will likely persist even if surgery normalizes bowel frequency. Patients with associated problems may be better served by having a stoma without resection as both a therapeutic maneuver and a diagnostic trial. Colectomy is no option to treat pain and/or abdominal bloating.
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PMID:Surgery for constipation. 1713 90

Observations about the natural history of aging in Cornelia de Lange syndrome (CdLS) are made, based on 49 patients from a multidisciplinary clinic for adolescents and adults. The mean age was 17 years. Although most patients remain small, obesity may develop. Gastroesophageal reflux persists or worsens, and there are early long-term sequelae, including Barrett esophagus in 10%; other gastrointestinal findings include risk for volvulus, rumination, and chronic constipation. Submucous cleft palate was found in 14%, most undetected before our evaluation. Chronic sinusitis was noted in 39%, often with nasal polyps. Blepharitis improves with age; cataracts and detached retina may occur. Decreased bone density is observed, with occasional fractures. One quarter have leg length discrepancy and 39% scoliosis. Most females have delayed or irregular menses but normal gynecologic exams and pap smears. Benign prostatic hypertrophy occurred in one male prior to 40 years. The phenotype is variable, but there is a distinct pattern of facial changes with aging. Premature gray hair is frequent; two patients had cutis verticis gyrata. Behavioral issues and specific psychiatric diagnoses, including self-injury, anxiety, attention-deficit disorder, autistic features, depression, and obsessive-compulsive behavior, often worsen with age. This work presents some evidence for accelerated aging in CdLS. Of 53% with mutation analysis, 55% demonstrate a detectable mutation in NIPBL or SMC1A. Although no specific genotype-phenotype correlations have been firmly established, individuals with missense mutations in NIPBL and SMC1A appear milder than those with other mutations. Based on these observations, recommendations for clinical management of adults with CdLS are made.
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PMID:Natural history of aging in Cornelia de Lange syndrome. 1764 42

While sigmoid volvulus is commonly seen in older patients, it is rarely encountered in children and younger adults. Consequently, heightened awareness of this entity is required to avoid a delay in diagnosis. Among the pediatric and adult cases of colonic volvulus previously reported in the English literature, 23 of the affected individuals have also been diagnosed with Hirschsprung disease (HD). This report describes a 12-year-old male with a history of chronic constipation who presented with vomiting and abdominal distension and was found to have sigmoid volvulus with previously unrecognized HD. The case presentation is followed by a review of the literature describing colonic volvulus secondary to HD in children.
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PMID:Hirschsprung disease presenting as sigmoid volvulus: a case report and review of the literature. 2333 23

We report the first case of sigmoid volvulus after laparoscopic surgery for sigmoid colon cancer. The patient is a 75-year-old man who presented with the sudden onset of severe abdominal pain. He had undergone laparoscopic sigmoidectomy for cancer 2 years before presentation. CT scan showed a distended sigmoid colon with a mesenteric twist, or "whirl sign." Colonoscopy showed a mucosal spiral and luminal stenosis with dilated sigmoid colon distally and ischemic mucosa. The diagnosis of ischemic colonic necrosis due to sigmoid volvulus was established. Resection of the necrotic sigmoid colon was performed and a descending colon stoma was created. A long remnant sigmoid colon and chronic constipation may contribute to the development of sigmoid volvulus after laparoscopic sigmoidectomy. Prompt diagnosis is essential for adequate treatment, and colonoscopy aids in the diagnosis of ischemic changes in patients without definitive findings of a gangrenous colon.
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PMID:Sigmoid volvulus after laparoscopic surgery for sigmoid colon cancer. 2387 14

Cecal volvulus is uncommon in pediatric patients and there are few reports of cecal volvulus with cerebral palsy. Here, we report the case of a 19-year-old male patient who presented with abdominal distension, a history of cerebral palsy, refractory epilepsy due to lissencephaly, and chronic constipation. An abdominal x-ray and computed tomography without contrast enhancement showed fixed dilated bowel intensity in the right lower abdomen. Despite decompression with gastric and rectal tube insertion, symptoms did not improve. The patient underwent an exploratory laparotomy that revealed cecal volvulus. Cecal volvulus usually occurs following intestinal malrotation or previous surgery. In this patient, however, intestinal distension accompanying mental disability and chronic constipation resulted in the development of cecal volvulus. We suggest that cecal and proximal large bowel volvulus should be considered in patients presenting with progressive abdominal distension combined with a history of neuro-developmental delay and constipation.
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PMID:A case of cecal volvulus presenting with chronic constipation in lissencephaly. 2401 Jan 18

We report a case of sigmoid volvulus post-stapled transanal rectal resection (STARR) for obstructed defecation. The patient, a 68-yearold woman with chronic constipation and dolichosigma, two days post-STARR presented severe abdominal pain. CT revealed sigmoid ischemia. The patient underwent resection of the sigmoid colon with end colostomy (Hartmann's procedure). Can STARR procedure produce a serious complication as sigmoid volvulus in patient with dolichosigma and obstructed defecation syndrome?
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PMID:Sigmoid volvulus: is it a possible complication after stapled transanal rectal resection (STARR)? 2409 Nov 79

Sigmoid volvulus, a condition generally seen in debilitated elderly patients, is extremely rare in the pediatric age group. Frequent predisposing conditions that accompany pediatric sigmoid volvulus include intestinal malrotation, omphalomesenteric abnormalities, Hirschsprung's disease, imperforate anus and chronic constipation. A 16-year-old previously healthy African American male presented with a 12 hour history of sudden onset abdominal pain and intractable vomiting. CT was consistent with sigmoid volvulus. A contrast enema did not reduce the volvulus, but it was colonoscopically reduced. Patient condition initially improved after colonoscopy, but he again became distended with abdominal pain, so he was taken to the operating room. On exploratory laparotomy, a band was discovered where the mesenteries of the sigmoid and small bowel adhered and created a narrow fixation point around which the sigmoid twisted. A sigmoidectomy with primary anastomosis was performed. The diagnosis of sigmoid volvulus may be more difficult in children, with barium enema being the most consistently helpful. Seventy percent of cases do not involve an associated congenital problem, suggesting that some pediatric patients may have congenital redundancy of the sigmoid colon and elongation of its mesentery. The congenital band found in our patient was another potential anatomic factor that led to sigmoid volvulus. Pediatric surgeons, accustomed to unusual problems in children, may thus encounter a condition generally found in the debilitated elderly patient.
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PMID:Sigmoid volvulus in 16-year-old boy with an associated anomalous congenital band. 2416 46


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