Volvulus with intestinal malrotation hiding a near-total intestinal aganglionosis: Case report.

Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the pediatric surgeon. We report the case of a new born, who was operated at the age of three days for malrotation with volvulus, treated by Ladd procedure. Postoperatively, we noticed persistent abdominal distension and emission of a small amount of meconium every 4 to 5 days. The barium enema showed a non-functional microcolon. Surgical exploration on the 24th day found an ileo-ileal transition zone located 60 cm distal to the ligament of Treitz. Extemporaneous biopsies from the colon and mid-ileum confirmed the absence of ganglion cells. We performed an ileostomy at 50 cm from duodeno-jejunal flexure. Unfortunately, the patient succumbed to nosocomial infection at 33 days of age.This case was a challenging scenario for us where a diagnosis of complicated malrotation had obscured the Hirschsprung's disease.

Megacolon andino y vólvulo del sigmoides de la altura: Presentación de 418 casos entre 2008 - 2012 en el hospital C. Monge - Puno, Perú / Andean megacolon and sigmoid volvulus in the high altitude: Presentation of 418 cases between 2008 - 2012 at C. Monge Hospital, Puno, Peru

Objetivo: El presente estudio describe el manejo médico y quirúrgico del vólvulo de sigmoides debido a dolicomegacolon andino en un hospital a una altitud mayor a 3000 msnm. Material y métodos: Estudio descriptivo, observacional, transversal de 418 pacientes con diagnóstico de vólvulo de sigmoides; admitidos inicialmente por cuadros de obstrucción intestinal, en el Hospital de Juliaca Carlos Monge - Puno, Perú, durante el periodo 2008-2012. Los datos fueron procesados a través del programa SPSS versión 21. Resultados: Se registraron 418 pacientes, la media de edad fue de 60 años, rango 18-89 años, con una proporción hombre/mujer de 3,5/1. El manejo no quirúrgico se hizo en 64 (15,4%), el tratamiento empleado fue de enema salino 20 casos 31% y sonda rectal 44 (69%), se presentó recurrencia en 27 pacientes (45%), los cuales tuvieron cirugía con resección anastomosis primaria, de estos la mortalidad correspondió a 8 pacientes (30%). De los 354 pacientes sometidos a manejo quirúrgico de emergencia 325 fueron sometidos a sigmoidectomia con anastomosis primaria (92%), mientras 29 tuvieron colostomía a lo Hartmann (8%), la morbilidad para ambos procedimientos fue de 52 casos (14,7%), la mortalidad para ambos procedimientos fue de 45 casos (12,7%). Conclusiones: El vólvulo sigmoides debido a megacolon andino tuvo una edad media de 60 años. El 15,4% tuvo manejo no quirúrgico, la tasa de recurrencia fue de 45%, mortalidad de 30%. El 84,7% tuvo manejo quirúrgico; el 92% tuvo resección anastomosis primaria y 8% colostomía a lo Hartmann, la morbilidad fue de 14,7% y la mortalidad de 12,7%.

Objective: The present study describes the medical and surgical management of sigmoid volvulus due to Andean dolicomegacolon in a hospital at an altitude above 3000 m. Material and methods: A descriptive, observational, crosssectional study of 418 patients diagnosed with sigmoid volvulus; Admitted initially due to intestinal obstruction, in the Hospital of Juliaca Carlos Monge. Puno-Perú, during the period 2008-2012. The data were processed through the SPSS software version 21. Results: A total of 418 patients were enrolled, the mean age was 60 years, range 18-89 years, and the male/female ratio was 3.5/1. Nonsurgical management was done in 64 (15.4%), the treatment used was saline enema 20 cases (31%) and rectal catheter 44 (69%), recurrence was present in 27 patients (45%), who had surgery with primary anastomosis resection, of which the mortality corresponded to 8 patients (30%). Of the 354 patients undergoing emergency surgical management, 325 were submitted to sigmoidectomy with primary anastomosis (92%), while 29 had Hartmann's colostomy (8%), the morbidity for both procedures was 52 cases (14.7%), Mortality for both procedures was 45 cases (12.7%). Conclusions: In patients with sigmoid volvulus due to Andean megacolon the mean age was 60 years. The 15.4% had non-surgical management, the recurrence rate was 45%, and mortality 30%. Patients with surgical management was 84.7%, from this group; 92% had primary anastomosis resection and 8% Hartmann colostomy, morbidity was 14.7% and mortality was 12.7%.

Pediatric colonic volvulus: A single-institution experience and review.

BACKGROUND/PURPOSE: Pediatric colonic volvulus is both rare and underreported. Existing literature consists only of case reports and small series. We present an analysis of cases (n=11) over 15 years at a single institution, focusing on workup and diagnosis. METHODS: This was an institutional review board approved single-institution retrospective chart review of 11 cases of large bowel volvulus occurring over 15 years (2000-2015). RESULTS: In our series, the most common presenting symptoms were abdominal pain and distention. Afflicted patients often had prior abdominal surgery, a neurodevelopmental disorder or chronic constipation. Of the imaging modalities utilized in the 11 patients studied, colonic volvulus was correctly diagnosed by barium enema in 100% of both cases, CT in 55.6% of cases and by plain radiography of the abdomen in only 22.2%of cases. Colonic volvulus was confirmed by laparotomy in all cases. The cecum (n=5) was the most often affected colonic segment, followed by the sigmoid (n=3). Operative treatment mainly consisted of resection (63.6%) and ostomy creation (36.4%). Colopexy was performed in 18.2% of cases. CONCLUSIONS: Plain abdominal radiography may be performed as an initial diagnostic study, however, it should be followed CT or air or contrast enema in children where there is high clinical suspicion and who do not have indications for immediate laparotomy. CT may be the most specific and useful test in diagnosis of colonic volvulus and has the added advantage of detection of complications including bowel ischemia. We demonstrate a range of diagnostic and therapeutic modalities for pediatric colonic volvulus. This underscores the need for further study to draft standard best practices for this life-threatening condition. LEVEL OF EVIDENCE: Prognosis Study: Level IV. Study of a Diagnostic Test: Level III.

[Andean megacolon and sigmoid volvulus in the high altitude. Presentation of 418 cases between 2008 - 2012 at C. Monge Hospital, Puno, Peru]. / Megacolon andino y vólvulo del sigmoides de la altura. Presentación de 418 casos entre 2008 - 2012 en el hospital C. Monge Puno, Perú.

OBJECTIVE: The present study describes the medical and surgical management of sigmoid volvulus due to Andean dolicomegacolon in a hospital at an altitude above 3000 m. MATERIAL AND METHODS: A descriptive, observational, crosssectional study of 418 patients diagnosed with sigmoid volvulus; Admitted initially due to intestinal obstruction, in the Hospital of Juliaca Carlos Monge. Puno-Perú, during the period 2008-2012. The data were processed through the SPSS software version 21. RESULTS: A total of 418 patients were enrolled, the mean age was 60 years, range 18-89 years, and the male/female ratio was 3.5/1. Nonsurgical management was done in 64 (15.4%), the treatment used was saline enema 20 cases (31%) and rectal catheter 44 (69%), recurrence was present in 27 patients (45%), who had surgery with primary anastomosis resection, of which the mortality corresponded to 8 patients (30%). Of the 354 patients undergoing emergency surgical management, 325 were submitted to sigmoidectomy with primary anastomosis (92%), while 29 had Hartmann's colostomy (8%), the morbidity for both procedures was 52 cases (14.7%), Mortality for both procedures was 45 cases (12.7%). CONCLUSIONS: In patients with sigmoid volvulus due to Andean megacolon the mean age was 60 years. The 15.4% had non-surgical management, the recurrence rate was 45%, and mortality 30%. Patients with surgical management was 84.7%, from this group; 92% had primary anastomosis resection and 8% Hartmann colostomy, morbidity was 14.7% and mortality was 12.7%.

Neonatal sigmoid volvulus.

BACKGROUND: Neonatal sigmoid volvulus is a rare entity. It is associated with Hirschsprung's disease. Presentation is acute abdominal distention, vomiting and obstipation. Abdominal radiograph will show the "coffee bean" sign, but this is frequently missed and the diagnosis requires a high index of suspicion. Treatment options include contrast enema, colonoscopy or laparotomy, depending on the condition of the baby and local availability. POPULATION AND RESULTS: During the last 6years, 6 infants with sigmoid volvulus were treated in our department. Four presented during the first 48h since birth, and 2 presented at the age of 2 and 7weeks of age. One child was operated and 5 had primary contrast enema with radiologic de-volvulus. Rectal biopsy was performed in all cases; three children had Hirschsprung's disease. Those with normal biopsies responded well to rectal washouts. Two patients had early one stage transanal pullthrough and one had 2 further occasions of sigmoid volvulus prior to definitive surgery. All three recovered with an uneventful course. CONCLUSIONS: Neonatal sigmoid volvulus requires a high level of suspicion. Contrast enema is efficient for primary de-volvulus. Rectal biopsy should be performed and if positive for Hirschsprung's disease, surgery should be performed sooner rather than later.

Association of anorectal malformation and intestinal malrotation.

BACKGROUND: Patients born with anorectal malformations (ARM) frequently have other congenital anomalies that are well-defined; however, limited data exist examining the relationship of ARM with malrotation. METHODS: A 10-year retrospective review was performed to examine all patients treated at a regional children's medical center with a diagnosis of ARM. Data were collected to identify malrotation, vertebral, anorectal, cardiac, tracheo-esophageal fistula, renal, radial, limb (VACTERL) anomalies, the type of ARM, operative procedures performed, and long-term bowel management. RESULTS: One hundred forty-six patients were identified. Upper gastrointestinal evaluation was performed in 21 patients (14.4%), while contrast enemas were performed in 22 patients (15.1%). Seven patients were found to have malrotation (4.8%) and 6/7 of these patients had 2 or more VACTERL anomalies. CONCLUSIONS: Patients with ARM and 2 or more VACTERL anomalies should undergo screening for malrotation. Patients with intestinal malrotation, ARM, and poor potential for bowel control should have their appendix preserved during a Ladd's procedure.

Sigmoid volvulus in 16-year-old boy with an associated anomalous congenital band.

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.

Manufactured volvulus.

BACKGROUND/PURPOSE: Malrotation with a common mesentery is the classical pathology allowing midgut volvulus to occur. There are only a few reports of small bowel volvulus without malrotation or other pathology triggering volvulation. We describe three cases of small bowel volvulus in very premature newborns with a perfectly normal intra-abdominal anatomy and focus on the question, what might have set off volvulation. METHODS: In 2005 to 2008, three patients developed small bowel volvulus without any underlying pathology. Retrospective patient chart review was performed with special focus on clinical presentation, preoperative management, intraoperative findings, and potential causative explanations. Mean follow-up period was 46 months. RESULTS: All patients were born between 27 and 31 weeks (mean 28 weeks) with a birth weight between 800 and 1,000 g (mean 887 g). They presented with an almost identical pattern of symptoms including sudden abdominal distension, abdominal tenderness, erythema of the abdominal wall, high gastric residuals, and radiographic signs of ileus. All of them were treated with intensive abdominal massage or pelvic rotation to improve bowel movement before becoming symptomatic. CONCLUSIONS: Properistaltic maneuvers including abdominal massage and pelvic rotation may cause what we term a "manufactured" volvulus in very premature newborns. Thus, this practice was stopped.

Colonic volvulus in children with intestinal motility disorders.

BACKGROUND: Chronic intestinal pseudo-obstruction (CIP) is a condition characterized by symptoms of bowel obstruction in the absence of an anatomical cause. Patients with CIP and chronic intractable constipation (CIC) can also develop anatomical obstruction, and the presenting symptoms mimic those of underlying pseudo-obstruction. OBJECTIVES: Our objectives were to evaluate the incidence, clinical presentation, and diagnostic investigations of colonic volvulus in children with intestinal motility disorders and to differentiate these episodes of colonic volvulus from the underlying motility disorder based on clinical presentation and imaging techniques. MATERIALS AND METHODS: Patients records of children with colonic volvulus cared for at our institution over the previous 20 years were retrospectively reviewed. We identified 8 patients who were between 2 and 22 years of age at the time of diagnosis with colonic volvulus who also had CIP and CIC. RESULTS: The mean age +/- SD at presentation with colonic volvulus was 13.2 +/- 5.05 years. All patients presented with worsening of abdominal distension and pain. The mean duration of symptoms of colonic volvulus before seeking medical help was 4.2 days (range 1-7 days). Water-soluble contrast enema was the single most useful investigation for confirming the diagnosis. All patients required surgery. There was no mortality associated with colonic volvulus. CONCLUSIONS: Clinicians should be vigilant and include volvulus in the differential diagnosis of the acute onset of abdominal distension and pain in patients with CIP and CIC. Delay in diagnosis can result in bowel ischemia and perforation.

Malrotation with volvulus of the transverse colon and duodenal obstruction secondary to Ladd's bands.

Colonic volvulus is a relatively uncommon cause of large bowel obstruction, accounting for 10% of colonic obstructions. Volvulus of the transverse colon is quite rare, accounting for only 4-11% of all reported cases of colonic volvulus (Kerry RL, Ransom HK. Volvulus of the colon: etiology, diagnosis, and treatment. Arch Surg 1969;99:215-221). We report an unusual case of documented volvulus of the transverse colon in a young man with intestinal malrotation and concomitant duodenal obstruction by Ladd's bands.

[Chronic recurrent volvulus of the colonic splenic flexure associated with the eventration of left diaphragm].

The eventration of diaphragm is usually found incidentally on chest X-ray or sometimes presented as acute gastric volvulus. However, colonic volvulus on splenic flexure area complicated by diaphragmatic eventration is extremely rare. A 25 year old man complained of upper abdominal pain for three days. He had a history of brain injury during infant period, and had epilepsy and mental retardation. Plain chest X-ray showed left diaphragmatic eventration and marked dilatation of colon on splenic flexure area which had not been changed for last three years. Barium enema showed bird beak appearance on distal colon near the splenic flexure. Colonoscopic reduction failed. After decompression with rectal and nasogastric tubes, colonic volvulus was relieved. To prevent the recurrence of volvulus, we performed segmental resection of left colon including splenic flexure area and repaired the left diaphragmatic eventration. After the operation, the patient had no further recurrent episode of volvulus although ileus persisted.

Chronic Recurrent Volvulus of the Colonic Splenic Flexure Associated with the Eventration of Left Diaphragm / 대한소화기학회지

The eventration of diaphragm is usually found incidentally on chest X-ray or sometimes presented as acute gastric volvulus. However, colonic volvulus on splenic flexure area complicated by diaphragmatic eventration is extremely rare. A 25 year old man complained of upper abdominal pain for three days. He had a history of brain injury during infant period, and had epilepsy and mental retardation. Plain chest X-ray showed left diaphragmatic eventration and marked dilatation of colon on splenic flexure area which had not been changed for last three years. Barium enema showed bird beak appearance on distal colon near the splenic flexure. Colonoscopic reduction failed. After decompression with rectal and nasogastric tubes, colonic volvulus was relieved. To prevent the recurrence of volvulus, we performed segmental resection of left colon including splenic flexure area and repaired the left diaphragmatic eventration. After the operation, the patient had no further recurrent episode of volvulus although ileus persisted.

Splenic flexure volvulus in a child with chronic idiopathic intestinal pseudo-obstruction syndrome.

Splenic flexure volvulus in a child with chronic idiopathic intestinal pseudo-obstruction syndrome is extremely rare. Here we present a case report of this unusual condition in a 7-year-old girl. The splenic flexure volvulus was managed by pressure reduction from the cecal antegrade continence enema, after which elective resection of the splenic flexure and primary anastomosis were performed because she had similar attacks during 4 months after the first detorsion. Postoperatively she made an uneventful recovery. Possible factors of pathogenesis and therapeutic measures are discussed.

Small bowel obstruction due to a persistent omphalomesenteric duct.

We report a case of a Meckel diverticulum connected with the umbilicus through a fibrotic cord causing small bowel obstruction. On admission, the patient presented with an acute abdomen. A plain upright radiography of the abdomen, an ultrasonography of the abdomen, and an enema with gastrografin were performed, showing a small bowel obstruction at the level of the pre-terminal ileum, without revealing the cause. Urgent surgery followed, showing a persistent omphalomesenteric duct connected to the abdominal wall through a fibrotic cord, with a secondary volvulus of the small bowel. The remnant was resected and the volvulus reduced. The post-operative course was uneventful. Because of the serious complications and even possible mortality due to ischemic disease of the affected small bowel the possibility of a complicated persistent omphalomesenteric duct should be kept in mind, even if the preoperative work-up does not reveal a Meckel diverticulum.

Aspectos embriológicos, clínicos y radiológicos de la malrotación intestinal / Embryologic, clinical, and radiological aspects of intestinal malrotation

La malrotación intestinal con su propensión al vólvulo representa un desafío diagnóstico en pacientes pediátricos. El diagnóstico precoz es de suma importancia para conseguir un buen resultado. En este artículo se revisan e ilustran los hallazgos radiológicos de la malrotación y el vólvulo de intestino medio en la infancia y adolescencia, y se discuten la embriología, la presentación clínica, el manejo radiológico y la evolución de este proceso. El diagnóstico de malrotación y vólvulo debe tenerse en cuenta siempre que se evalúe a un lactante o niño con vómitos y dolor abdominal, particularmente si los vómitos son biliosos. Un tránsito baritado generalmente dará el diagnóstico. La ecografía es muy útil, si bien no es suficientemente segura para excluir el diagnóstico. Sin embargo, el «signo del remolino» es un signo altamente sensible para el diagnóstico del vólvulo de intestino medio. El radiólogo siempre debe tener presente que esta complicación puede ocurrir a cualquier edad

Intestinal malrotation with its propensity to volvulus represents a diagnostic challenge in pediatric patients. Early diagnosis is of extreme importance for achieving a good outcome. This article reviews and illustrates the radiologic findings for malrotation and volvulus of the small bowel in childhood and adolescence. The embryology, clinical presentation, radiologic management, and evolution of this process are discussed. The diagnosis of malrotation and volvulus should be considered whenever a nursing baby or child presents with vomiting and abdominal pain, especially if the vomit is bilious. A barium follow-through usually provides the diagnosis. Ultrasound is very useful, although it cannot safely rule out the diagnosis. However, the "whirlpool sign" is a highly sensitive sign for the diagnosis of small bowel volvulus. The radiologist needs to bear in mind that this complication can occur at any age

Cecal volvulus: a report of two cases occurring after the antegrade colonic enema procedure.

BACKGROUND: Many children with chronic constipation and fecal incontinence have benefited from the antegrade colonic enema (ACE) procedure. Routine antegrade colonic lavage often allows such children to avoid daytime soiling. This report describes 2 children in whom the ACE procedure was complicated by a cecal volvulus. METHODS: A retrospective review of 164 children with an ACE procedure was conducted. Two instances of cecal volvulus were identified. RESULTS: The first child presented with abdominal pain and difficulty intubating the ACE site. Over the subsequent day, his pain worsened, and radiographs depicted a colonic obstruction. At laparotomy, a cecal volvulus resulting in bowel necrosis was observed, and resection of the affected bowel and appendix (in the right lower quadrant) and end ileostomy was required. He subsequently had the stoma closed and a new ACE constructed with a colon flap. The second child presented with shock and evidence of an acute abdomen. At laparotomy, a cecal volvulus was noted, and ileocolic resection including the ACE stoma (located at the umbilicus) and an ileostomy and Hartmann pouch was performed. He had a protracted hospital course requiring ventilator and inotropic support. He currently is well and still has an ileostomy stoma. CONCLUSIONS: A high index of suspicion for a potentially life-threatening cecal volvulus should be maintained in children undergoing an ACE procedure who present with abdominal pain, evidence of bowel obstruction, or difficulty in advancing the ACE irrigation catheter.