Sigmoid volvulus as a complication of imperforate anus: a case report

The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients. Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy. Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence. • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.

Rationale:Sigmoid volvulus (SV) is an exceptionally rare but potentially life-threatening condition in children.Chief complaint:Abdominal distention for 1 week.Diagnoses:Sigmoid volvulus.Patient concerns:We present a case of a 12-year-old boy with mechanical ileus who was finally confirmed to have SV with the combination of abdominal plain film, sonography, and computed tomography (CT) with the finding of mesenteric artery rotation.Interventions:Because bowel obstruction was suspected, abdominal plain film, sonography, and CT were performed. The abdominal CT demonstrated whirlpool sign with torsion of the sigmoid vessels. In addition, lower gastrointestinal filling study showed that the contrast medium could only reach the upper descending colon. Therefore, he received laparotomy with mesosigmoidoplasty for detorsion of the sigmoid.Outcomes:The postoperative recovery was smooth under empirical antibiotic treatment with cefazolin. A follow-up lower gastrointestinal series on the seventh day of admission showed no obstruction compared with the previous series. He was finally discharged in a stable condition 8 days after admission.Lessons:SV is a congenital anomaly and an uncommon diagnosis in children. Nevertheless, case series and case reports of SV are becoming more prevalent in the literature. Failure to recognize SV may result in life-threatening complications such as sigmoid gangrene/perforation, peritonitis, sepsis, and death. Thus, if the children have persistent and recurrent abdominal distention, abdominal pain, and vomiting, physicians should consider SV as a “do not miss diagnosis” in the differential diagnosis.

Sigmoid volvulus in children and adolescents

Sigmoid volvulus in children is a potentially disastrous situation, still remaining rare in terms of occurrence. We hereby present a case report of a 10-year-old male, having admitted in our department complaining about abdominal pain, who finally proved to suffer from sigmoid volvulus.

Sigmoid volvulus is a rare but life-threatening diagnosis in the paediatric population and has only been reported a handful of times in the literature. We describe the case of a 14-year-old boy with abdominal pain and diarrhoea who was diagnosed with a sigmoid volvulus after initially being managed for infectious gastroenteritis. The patient initially presented with a 5-day history of watery stool, 1-day history of profuse vomiting and colicky abdominal pain. Whilst admitted, the patient developed worsening abdominal pain, distention and hyperresonance to percussion. Computed tomography demonstrated a dilated sigmoid colon, with a mesenteric ‘whirl sign’ around the inferior mesenteric artery. The patient underwent a laparotomy, which confirmed a sigmoid volvulus, requiring an anterior resection. This case emphasises the importance for general surgeons to consider the rare diagnosis of sigmoid volvulus in children.

The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Colonic Volvulus and Acute Colonic Pseudo-Obstruction.

69-Year-Old Man With Abdominal Pain and Obstipation

Management of Sigmoid Volvulus Based on Ladd's Procedure: A Case Report

A 17-year-old Caucasian man with no significant past medical history or hospitalization presented to the ED complaining of abdominal pain, bloating, and diarrhea for five days. The patient said his pain was located in his upper abdomen; it was cramping, constant, and worse when he laid down, with a 7/10 intensity. He reported more than 10 episodes of watery, brown, non-bloody diarrhea daily since the onset of his symptoms. He said he had nausea and had one episode of non-bloody and non-bilious vomiting prior to arrival. He denied fever, chills, headache, sore throat, back pain, genital pain, weight loss, and rash. He recalled similar symptoms that occurred five months earlier that lasted for one week and resolved without intervention. He had no associated trauma, sick contacts, recent travel, or family history of abdominal problems. The patient's vital signs were stable with a temperature of 98°F, a pulse of 80 bpm, a respiratory rate of 16 bpm, a blood pressure of 130/66 mm Hg, and an oxygen saturation of 100 percent on room air. The patient appeared uncomfortable during the physical exam. Heart sounds were normal, and lung fields were clear bilaterally. No bowel sounds were heard. He had marked abdominal distention in the epigastric region, and the abdomen was diffusely tender, firm to palpation, and tympanic to percussion, with guarding. A fluid wave was not appreciated. His mucous membranes were moist, and his skin had good turgor.The remainder of the physical exam was normal. Intravenous fluid was started, labs were drawn, and x-rays of his abdomen with decubitus and erect views were ordered. The patient was given morphine for pain control and ondansetron (Zofran) for nausea. The laboratory results were unremarkable except for a mild elevation in total bilirubin of 1.4 mg/dL. The x-ray showed marked constipation with moderate large bowel predominant air dilation, with the absence of gas in the rectum. (Figure 1.) A CT abdomen and pelvis with oral and IV contrast was ordered. The CT revealed significant distention of the entire colon, with a sigmoid diameter of 10 cm. A transition was seen at the rectosigmoid junction, and no air fluid levels were present. A mesenteric twist was seen at the base of the sigmoid, consistent with a sigmoid volvulus. (Figure 2.)Figure 1: X-ray of the abdomen, supine view.Figure 2: CT of the abdomen and pelvis, coronal view.The patient was admitted to the pediatric in-patient service. IV piperacillin/tazobactam and metronidazole were started. A barium enema was performed, but the volvulus was unable to be reduced. (Figure 3.) A flexible sigmoidoscopy and rectal suction biopsy was performed, which was successful in decompressing the sigmoid colon. The next day, the patient had a large bowel movement, which caused significant improvement in his distention. Rectal biopsies were negative for Hirschsprung's disease. The patient's diet was slowly advanced, and the patient was discharged home after three days on a high-fiber diet to prevent constipation, which was believed to be the precipitating factor. Ultimately, the patient did not require further intervention.Figure 3: Barium enema.Sigmoid volvulus is a rare diagnosis in the pediatric population, with only 63 reported cases in patients under 18 years of age from 1940 to 2000. The median age of diagnosis in a previous review was found to be 7 years old, with a strong male predominance of 3.5:1. (J Am Coll Surg 2000;190[6]:717.) The etiology of pediatric sigmoid volvulus differs from that seen in the adult population. It is most commonly caused by constipation in adults, and has also been associated with a high-fiber diet in developing countries. It is believed to be caused by a redundancy of the sigmoid colon, a long mesentery with a narrow base, or a lack of fixation of the mesentery to a portion of the colon in children. Children with Hirschsprung's disease are also at risk. (J Pediatr Surg 2004;39[9]:1434.) Sigmoid volvulus most commonly presents with the symptoms of abdominal pain, bloating, and vomiting. Less common signs and symptoms include abdominal tenderness, diarrhea, nausea, and fever. (J Am Coll Surg 2000;190[6]:717.) A plain film will most commonly show dilated loops of colon, and may demonstrate the coffee bean sign, referring to the two distended sigmoid compartments with the central double walls of the colon. Plain films can make a diagnosis in 35–60 percent of cases. A CT is the preferred modality for diagnosis in the emergency department, nearing 100 percent accuracy, and can exclude other causes of abdominal pain. (J Emerg Med 2013;44[3]:611.) Managing sigmoid volvulus in children is controversial because of its rarity. Fluid resuscitation and antibiotics should be given because of its association with bowel ischemia, gangrene, and perforation. Early consultation to surgery and gastroenterology is recommended. Nonoperative management should be considered first. Barium enema can be diagnostic and therapeutic, and was successful in reducing the volvulus in 77 percent of patients in a previous study. (J Am Coll Surg 2000;190[6]:717.) A flexible sigmoidoscopy is also an option, with a success rate of 78 percent, but it has a 2.4 percent risk of traumatic perforation. Nonoperative treatment has a high recurrence rate of 35 percent in children and up to 70 percent in adults. Definitive treatment is sigmoidectomy, and should be considered in most patients to prevent recurrence. (World J Surg 2010;34:1923.) Sigmoid volvulus, though rare in children, should be considered in any patient presenting with symptoms of bowel obstruction. Early diagnosis and decompression is vital to prevent complications such as bowel ischemia, gangrene, or perforation.

Sigmoid volvulus is a rare cause of intestinal obstruction in the pediatric age group. Rotation of the redundant sigmoid colon about its narrow mesenteric base results in vascular compromise and large bowel obstruction. Predisposing factors for sigmoid volvulus are Hirschsprung’s disease, congenital anomalous fixation of the colon, and chronic constipation. Here, we report two cases of sigmoid volvulus in children with redundant sigmoid colon in the South Indian subcontinent. If it is not diagnosed in time, it may lead to serious complications such as gangrene, perforation, septic shock, and eventually death. Thus, the condition warrants prompt evaluation and treatment.

Sigmoid volvulus is very uncommon cause of intestinal obstruction in pediatrics population withhigh rate of mortality. To date, few cases of sigmoid volvulus in children and association with several condition has been reported in literature, of them very few cases are with mental disability. We report a challenged (mentally disabled) 14-year old adolescent boy presented asan emergency with feature of complete bowel obstruction. Abdominal X-rays shows dilated loop of large bowel with inverted U shaped. Volvulus of sigmoid colon was found during laparotomy and successfully managed with resection of a redundant colon with colocolic end to end anastomosis. Sigmoid volvulus is relatively uncommon in children as compared to adults. Surgeons should be attentive of this rare entity, cause of large bowel obstruction to allow for early diagnosis and to enable better patient outcomes by reducing the morbidity and mortality.

Background: Sigmoid volvulus in the neonate is an extremely rare condition needing emergency treatment. Sporadic cases of neonatal sigmoid volvulus and some in association with Hirschsprung’s disease have been documented. Sigmoid volvulus has been described only twice in the literature in association with anorectal malformation. However, newborns with anorectal malformations might be especially at risk for sigmoid volvulus due to increased intraluminal intestinal weight caused by bowel obstruction and its existence might be underreported. Case presentation: This is the case report of a full-term neonate with trisomy 21 who was diagnosed with anorectal malformation upon birth. An abdominal X-ray on his second day of life showed typical radiological findings of sigmoid volvulus as described by the radiologist. However, the findings were interpreted as a dilated rectosigmoid sling typical for anorectal malformation by the surgeon and a transverse loop colostomy was placed. On the fourth postoperative day sigmoid perforation occurred and led to meconium peritonitis and septic shock. An emergency laparotomy was performed and a perforation at the distal sigmoid colon was found. Conclusion: There is an urgent need to raise awareness as to the existence of sigmoid volvulus in the neonate with anorectal malformations. The distinct radiological findings of the “coffee bean sign”, the “northern exposure sign” and an empty rectum allow to distinguish the extremely rare sigmoid volvulus from the common finding of a dilated rectosigmoid in patients with anorectal malformations. Detorsion of sigmoid volvulus is vital to preventing sigmoid perforation.

A 12-year-old boy presented with large-bowel obstruction due to sigmoid volvulus. Temporary relief was achieved with rectal tube decompression. Elective laparoscopic-assisted sigmoid colectomy was performed. Post-operative recovery was uneventful. The patient remained well with no recurrence after 4 years of follow-up. Laparoscopic-assisted sigmoid colectomy may be the procedure of choice for selected children with sigmoid volvulus.

The flexible colonoscope has notable advantages over rigid instruments and can be offered as an alternative and (probably) preferable method for non-surgical reduction of colonic volvulus. When operative intervention is called for because of repeated bouts of sigmoid volvulus, colonoscopy offers a means of preoperative deflation of the twisted loop, allowing time to prepare the bowel and correct systemic disturbances such as electrolyte imbalance. The first successful management of a case of recurrent sigmoid volvulus using fiberoptic flexible colonoscope is presented. It is suggested that the fiberoptic colonoscope may have similar application for instances of volvulus occurring more proximal than in the sigmoid colon. Sigmoid volvulus in children even though rare might also be amenable to correction by colonoscopy.

Colonic volvulus accounts for 3 to 5% of bowel obstructions in the United States. Current data reveal sigmoid volvulus as the most common colonic volvulus, followed by volvulus of the cecum, transverse colon, and splenic fixture. Despite a low incidence in the United States, diagnosis, management, and patient outcome depend on an appropriate index of suspicion and adherence to the proposed algorithm highlighting the approach to the patient with colonic volvulus. This review outlines the definition, pathogenesis, and epidemiology of colonic volvulus, as well as its clinical evaluation and treatment. Tables review the demographics of colonic volvulus in the United States, the differential diagnosis of and risk factors for colonic volvulus, important radiographic findings in colonic volvulus, and nonoperative management of sigmoid volvulus. Figures show the types of ileosigmoid knot; plain radiographs of cecal, sigmoid, transverse, and splenic flexure volvulus; contrast enema of cecal, transverse, splenic flexure, and sigmoid volvulus; cross-sectional abdominal imaging of cecal, sigmoid, and transverse colon volvulus and ileosigmoid knot; endoscopic evaluation in sigmoid volvulus; use of an esophageal overtube for placement of a rectal tube; necrotic cecum and transverse colon volvulus in the operating suite; and sigmoid volvulus in an elderly gentleman. This review contains 14 figures, 13 tables, and 165 references Keywords: Colonic volvulus, colonic obstruction, endoscopy, surgical intervention, ileosigmoid knot, splenic flexure volvulus, sigmoid volvulus, transverse colon volvulus, cecal volvulus