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Hencke J, Loff S. Recurrent Sigmoid Volvulus in Children-Our Experience and Systematic Review of the Current Literature. CHILDREN (BASEL, SWITZERLAND) 2023; 10:1441. [PMID: 37761402 PMCID: PMC10528811 DOI: 10.3390/children10091441] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/17/2023] [Revised: 08/20/2023] [Accepted: 08/21/2023] [Indexed: 09/29/2023]
Abstract
Sigmoid volvulus (SV) occurs rarely in children. After encountering two cases of recurrent SV, we reviewed the literature to define the recurrence risk, identify outcome predictors and to give treatment recommendations. We found 256 cases reported in children (mean age 10.2 years, gender ratio (m:f) 2.3:1). Associations exist with Hirschsprung disease (HD) in 10%, neurodevelopmental disorders in 10.9% and chronic constipation in 10.2%. Common symptoms and clinical signs were abdominal pain (85%), distension (85%), tenderness (54%) and vomiting (59%). Signs of peritonitis were present in 14% and indicated a gangrenous sigmoid (X2 = 45.33; p < 0.001). A total of 183 had abdominal radiographs, and 65% showed a positive 'coffee-bean-sign'. Contrast enemas were positive in 90%. A total of 124 patients underwent laparotomy; in 41 cases, the sigmoid was gangrenous and associated with more complications (X2 = 15.68; p < 0.001). Non-operative treatment (NOT) like endoscopic, fluoroscopic or rectal tube decompression was performed in 135 patients and successful in 79% with a 38-57% recurrence rate. A total of 73 patients subjected to elective surgery: 50 underwent sigmoid resection; 17 had surgery for HD. Clinicians should consider SV in all children with abdominal pain, distension and vomiting. Gangrene carries a higher morbidity. After successful NOT we recommend counselling about the recurrence risk and definitive surgery should be advised. HD is frequent in newborns but sometimes found in older children.
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Affiliation(s)
- Jonathan Hencke
- Department of Pediatric Surgery, Olgahospital, Klinikum Stuttgart, 70174 Stuttgart, Germany
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2
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Khlevner J, Patel D, Rodriguez L. Pediatric Neurogastroenterology and Motility Disorders: What Role Does Endoscopy Play? Gastrointest Endosc Clin N Am 2023; 33:379-399. [PMID: 36948752 DOI: 10.1016/j.giec.2022.10.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/24/2023]
Abstract
Although pediatric neurogastroenterology and motility (PNGM) disorders are prevalent, often debilitating, and remain challenging to diagnose and treat, this field has made remarkable progress in the last decade. Diagnostic and therapeutic gastrointestinal endoscopy emerged as a valuable tool in the management of PNGM disorders. Novel modalities such as functional lumen imaging probe, per-oral endoscopic myotomy, gastric-POEM, and electrocautery incisional therapy have changed the diagnostic and therapeutic landscape of PNGM. In this review, the authors highlight the emerging role of therapeutic and diagnostic endoscopy in esophageal, gastric, small bowel, colonic, and anorectal disorders and disorders of gut and brain axis interaction.
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Affiliation(s)
- Julie Khlevner
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Columbia University Vagelos College of Physicians and Surgeons, Gastrointestinal Motility Center, NewYork Presbyterian Morgan Stanley Children's Hospital, 622 West 168th Street, PH 17, New York, NY 11032, USA.
| | - Dhiren Patel
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Saint Louis University School of Medicine, SSM Cardinal Glennon Children's Medical Center, 1465 South Grand Boulevard, St Louis, MO 63104, USA
| | - Leonel Rodriguez
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Yale New Haven Children's Hospital, Yale School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA
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Obstructing Sigmoid Volvulus: An Unusual Complication in a Pediatric Patient With Ulcerative Colitis. ACG Case Rep J 2023; 10:e00998. [PMID: 36876286 PMCID: PMC9981391 DOI: 10.14309/crj.0000000000000998] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2022] [Accepted: 01/30/2023] [Indexed: 03/05/2023] Open
Abstract
Acute colonic dilation in pediatric patients with ulcerative colitis (UC) raises a concern for toxic megacolon, but other rare conditions such as sigmoid volvulus may present in a similar manner. We report a rare case of a teenager with UC without prior surgery who developed an obstructing sigmoid volvulus managed with endoscopic detorsion and decompression. Colonic inflammation in patients with UC may result in a volvulus in the absence of other predisposing factors and should be considered in the differential diagnosis of patients with UC who present with obstructive symptoms with an atypical presentation.
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4
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Lofgran T, Koury R. A Twist on Adolescent Abdominal Pain in the Emergency Department. Cureus 2022; 14:e27371. [PMID: 36046319 PMCID: PMC9418664 DOI: 10.7759/cureus.27371] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/27/2022] [Indexed: 11/05/2022] Open
Abstract
Abdominal pain is a common complaint in pediatric patients in the emergency department (ED). Evolutions in clinical practice have shifted away from computed tomography (CT) to ultrasound (US) in assessing abdominal pain. However, ultrasound may not reliably rule out critical diagnoses. We present a 15-year-old male with intermittent suprapubic abdominal pain. Subsequent CT imaging showed swirling mesenteric vessels with a dilated sigmoid colon. In adolescent abdominal pain, sigmoid volvulus (SV), although rare, should be considered. Clinicians should avoid anchoring bias by maintaining a broad differential. Definitive care is surgical with resection to prevent recurrence.
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Emeka CK. Sigmoid volvulus in a teenager. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2021.102119] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
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Colonic Volvulus in Children: Surgical Management of a Challenging Condition. CHILDREN 2021; 8:children8110982. [PMID: 34828695 PMCID: PMC8625683 DOI: 10.3390/children8110982] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/19/2021] [Revised: 10/24/2021] [Accepted: 10/25/2021] [Indexed: 11/17/2022]
Abstract
Colonic volvulus (CV) is a rare but potentially life-threatening condition with unclear etiopathogenesis. To date, less than 80 pediatric cases have been described. Hirschsprung’s disease (HD) is associated with CV in 17% of cases, representing a significant risk factor. Non-HD CV is an even more complex entity. The aim of this study is to describe a series of patients with CV to accentuate some peculiar aspects of this disease. We performed a retrospective study (period: 2012–2021) collecting information of patients with CV. Data analyzed included: demographics, medical history, presenting symptoms and radiological and surgical details. Eleven patients (12.5 ± 2.8 years; 7F/4M) had CV (eight sigmoid, two transverse colon, one total colon). Five patients had associated anomalies and three had HD. A two-step approach with volvulus endoscopic/radiological detorsion followed by intestinal resection was attempted in eight cases (one endoscopic approach failed). Three patients required surgery at admission. At follow-up, two patients developed recurrent intestinal obstruction, one of whom also had anastomotic stenosis. Colonic volvulus is a challenging condition that requires prompt patient care. A missed diagnosis could lead to severe complications. The evaluation of the patient should include a careful histological examination (searching for HD and alpha-actin deficiency), immunologic and metabolic screening, neurological tests and detection of chronic intestinal pseudo-obstruction (CIPO). Lifelong follow-up is mandatory for the early recognition and treatment of progressive diseases involving the proximal gastrointestinal tract.
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7
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Parrado RH, Rubalcava NS, Davenport KP. From the Cecum to the Sigmoid: Twisted Colon in the Pediatric Population. Cureus 2021; 13:e17974. [PMID: 34660156 PMCID: PMC8516025 DOI: 10.7759/cureus.17974] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/14/2021] [Indexed: 11/05/2022] Open
Abstract
Colonic volvulus (CV) is the third leading cause of colonic obstruction in adults. In infants and children, this is exceedingly rare, with only sporadic cases reported so far. We present two cases of CV to highlight the differences in etiology, presentation, diagnosis, and treatment of this condition. The first patient is a 12-year-old boy with no previous surgeries who presented with four days of abdominal pain. Imaging showed a sigmoid volvulus that was decompressed endoscopically, and he was discharged. He had a contrast enema showing an abnormal rectosigmoid ratio. At the time of the rectal biopsy four weeks later, he was found to have a recurrence, at which point definitive operative treatment was pursued. The second patient is a 17-year-old boy who presented with five days of abdominal pain and CT findings concerning for ischemic volvulus. This prompted emergent operative intervention, where a cecal volvulus was discovered as the result of a congenital band. The band was divided without complication. Pediatric CV is a rare condition that might be severe in some cases. High suspicion, prompt diagnosis, and treatment are essential to prevent early and long-term morbidity.
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Affiliation(s)
- Raphael H Parrado
- Division of Pediatric Surgery, Medical University of South Carolina, Charleston, USA
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Gohsman Z, Chan A, Davis MK. Endoscopic Reduction of Sigmoid Volvulus in a 15-Year-Old Male. Glob Pediatr Health 2021; 8:2333794X211033438. [PMID: 34377744 PMCID: PMC8330459 DOI: 10.1177/2333794x211033438] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2021] [Accepted: 06/30/2021] [Indexed: 11/16/2022] Open
Abstract
Sigmoid volvulus is a well-recognized phenomenon in the elderly but rare in children. The proposed mechanism involves rotation of a redundant sigmoid loop around a narrow, elongated mesentery with subsequent vascular occlusion. The condition can be intermittent or may resolve spontaneously, complicating diagnosis. Early diagnosis is imperative to prevent ischemic complications including necrosis, perforation, and sepsis. Abdominal pain, abdominal distention, and vomiting are the most common presenting symptoms, however abdominal tenderness is uncommon. Colonic dilation is the most frequent finding on abdominal radiograph. Contrast enema reveals a "bird's beak" configuration of the twisted colon and moreover, is successful in reducing the majority of pediatric cases. If there is no evidence of bowel ischemia or perforation, endoscopic reduction has been proposed as first-line treatment for sigmoid volvulus, especially in children. We report the case of 15-year-old male in which endoscopic reduction of sigmoid volvulus was successful without complication.
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Abstract
Sigmoid volvulus in paediatric patients is a rare but potentially life-threatening condition. Since 1940, only 100 cases have been reported. There are no consensual guidelines for juvenile sigmoid volvulus unlike in adults, where the condition and the treatment is well described. We report a case of a 12-year-old patient, who presented with uncharacteristic symptoms of mild abdominal discomfort and lack of passage of stool. A CT-scan showed a sigmoid volvulus and emergency resection was performed with placement of a colostomy. With this case, we want to emphasise juvenile sigmoid volvulus as a probable differential diagnosis when symptoms of abdominal distress and constipation occur.
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Affiliation(s)
- Merete Berthu Damkjaer
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen Universiy Hospital, Copenhagen, Denmark
| | - Waqas Farooqui
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen Universiy Hospital, Copenhagen, Denmark
| | - Inge Ifaoui
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen Universiy Hospital, Copenhagen, Denmark
| | - Luit Penninga
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen Universiy Hospital, Copenhagen, Denmark
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Ibrahim H, Sabra TA, Maher A. Short bowel syndrome as a result of sigmoid volvulus in an 8-year-old child. The first reported case worldwide: A case report. Int J Surg Case Rep 2021; 81:105769. [PMID: 33820736 PMCID: PMC8073200 DOI: 10.1016/j.ijscr.2021.105769] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2021] [Revised: 03/10/2021] [Accepted: 03/10/2021] [Indexed: 11/18/2022] Open
Abstract
INTRODUCTION We present a case of a male child 8 years old presenting with a sigmoid volvulus causing ischemia of most of bowel loops resulting in a short bowel syndrome. It is the first reported case worldwide. PRESENTATION A male child presented with a picture of intestinal obstruction. After complete laboratory and radiological investigation, laparotomy was done revealing a sigmoid volvulus compessing most of the small bowel loops with gangrenous sigmoid colon for which sigmoidectomy with end colostomy, resection of gangrenous small bowel loops and primary anastomosis of the remaining healthy part. DISCUSSION There are few reported cases describing sigmoid volvulus in this age group. However, none of them resulted in short bowel syndrome. The median age was 7 years with a higher ratio in males than females (3.5:1). CONCLUSION Sigmoid volvulus is not a common problem in children and adolescents, and is rarely considered as a cause of intestinal obstruction and it was never reported as a cause of short bowel syndrome. Early diagnosis and prompt treatment confer an excellent prognosis.
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Affiliation(s)
- Hussein Ibrahim
- Pediatric Surgery Unit, Assiut University Children Hospital, Assiut, Egypt.
| | | | - Ahmed Maher
- Pediatric Surgery Unit, Assiut University Children Hospital, Assiut, Egypt
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11
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Gosaye AW, Nane TS, Negussie TM. A case report of Hirschsprung's disease presenting as sigmoid volvulus and literature review, Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia. BMC Surg 2021; 21:109. [PMID: 33657993 PMCID: PMC7931363 DOI: 10.1186/s12893-020-00938-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2020] [Accepted: 10/29/2020] [Indexed: 11/10/2022] Open
Abstract
Background Sigmoid volvulus is an uncommon problem in children and adolescents, and is rarely considered a diagnosis in this group. A high index of suspicion is necessary to reach a diagnosis and avoid morbidity and mortality. Sigmoid volvulus is a rare complication of Hirschsprung’s disease, which has been reported in neonates, children, and adults. Here we report a case of sigmoid volvulus accompanied by undiagnosed Hirschsprung's disease. Case presentation A 9 years old boy who presented with sudden onset of colicky abdominal pain of 4 h duration associated with gross abdominal distension and 2 episodes of non-bilious vomiting. A plain abdominal radiographs showed single hugely dilated bowel loops in the left lower quadrant with single air fluid level. Sigmoid volvulus was considered and rectal tube deflation was done and it was successful. Full thickness rectal biopsy was done and it was consistent with aganglionic megacolon. A primary trans-anal Soave endo-rectal pull through was done 3 weeks later, after biopsy result arrived, which yielded loss of symptoms and regular bowel movement. Conclusions Sigmoid volvulus should be considered in the differential for children presenting with acute onset of abdominal obstruction. It should be known that when its’s diagnosed in children, it is often associated with Hirschsprung's disease. Therefore, a proper diagnostic and treatment algorithm should be followed in order not to miss associated HD and to give optimum care to such patients.
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Affiliation(s)
- Abay Wondimu Gosaye
- Department of Surgery, Tikur Anbessa Specialized Hospital, Addis Ababa University, Zambia St., Addis Ababa, Ethiopia.
| | - Temesgen Setato Nane
- Department of Surgery, Tikur Anbessa Specialized Hospital, Addis Ababa University, Zambia St., Addis Ababa, Ethiopia
| | - Tihitena Mammo Negussie
- Department of Surgery, Tikur Anbessa Specialized Hospital, Addis Ababa University, Zambia St., Addis Ababa, Ethiopia
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12
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McRae JJ, Goodman LF, Radulescu A. Sigmoid volvulus in a teenager. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2020.101392] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022] Open
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13
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Naveed M, Jamil LH, Fujii-Lau LL, Al-Haddad M, Buxbaum JL, Fishman DS, Jue TL, Law JK, Lee JK, Qumseya BJ, Sawhney MS, Thosani N, Storm AC, Calderwood AH, Khashab MA, Wani SB. American Society for Gastrointestinal Endoscopy guideline on the role of endoscopy in the management of acute colonic pseudo-obstruction and colonic volvulus. Gastrointest Endosc 2020; 91:228-235. [PMID: 31791596 DOI: 10.1016/j.gie.2019.09.007] [Citation(s) in RCA: 63] [Impact Index Per Article: 12.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2019] [Accepted: 09/04/2019] [Indexed: 12/11/2022]
Abstract
Colonic volvulus and acute colonic pseudo-obstruction (ACPO) are 2 causes of benign large-bowel obstruction. Colonic volvulus occurs most commonly in the sigmoid colon as a result of bowel twisting along its mesenteric axis. In contrast, the exact pathophysiology of ACPO is poorly understood, with the prevailing hypothesis being altered regulation of colonic function by the autonomic nervous system resulting in colonic distention in the absence of mechanical blockage. Prompt diagnosis and intervention leads to improved outcomes for both diagnoses. Endoscopy may play a role in the evaluation and management of both entities. The purpose of this document from the American Society for Gastrointestinal Endoscopy's Standards of Practice Committee is to provide an update on the evaluation and endoscopic management of sigmoid volvulus and ACPO.
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Affiliation(s)
- Mariam Naveed
- Advent Health Medical Group, Gastroenterology/Hepatology, Advent Health Hospital Altamonte Springs, Altamonte Springs, Florida, USA
| | - Laith H Jamil
- Division of Gastroenterology and Hepatology, Beaumont, Royal Oak, Royal Oak, Michigan, USA
| | | | - Mohammad Al-Haddad
- Division of Gastroenterology/Hepatology, Indiana University School of Medicine, Indianapolis, Indiana, USA
| | - James L Buxbaum
- Division of Gastrointestinal and Liver Diseases, Keck School of Medicine, University of Southern California, Los Angeles, California, USA
| | - Douglas S Fishman
- Department of Gastroenterology, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA
| | - Terry L Jue
- The Permanente Medical Group, Kaiser Permanente San Francisco Medical Center, San Francisco, California, USA
| | - Joanna K Law
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, Washington, USA
| | - Jeffrey K Lee
- Department of Gastroenterology, Kaiser Permanente San Francisco Medical Center, San Francisco, California, USA
| | - Bashar J Qumseya
- Department of Gastroenterology, Archbold Medical Group, Thomasville, Georgia, USA
| | - Mandeep S Sawhney
- Division of Gastroenterology, Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, Massachusetts, USA
| | - Nirav Thosani
- Division of Gastroenterology, Hepatology and Nutrition, McGovern Medical School, UTHealth, Houston, Texas, USA
| | - Andrew C Storm
- Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Audrey H Calderwood
- Department of Gastroenterology and Hepatology, Dartmouth Hitchcock Medical Center, Geisel School of Medicine, Lebanon, New Hampshire, USA
| | - Mouen A Khashab
- Division of Gastroenterology and Hepatology, Johns Hopkins University, Baltimore, Maryland, USA
| | - Sachin B Wani
- Division of Gastroenterology and Hepatology, University of Colorado Anschutz Medical Center, Aurora, Colorado, USA
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Abstract
Sigmoid volvulus is an extremely rare cause of abdominal pain in children. More commonly seen in older adults, an SV occurs when a redundant loop of sigmoid wraps around its elongated, narrow mesentery causing obstruction and ischemia to the affected bowel segment. Children usually present with abdominal pain, nausea, and abdominal distension. Presentations may be acute or chronic with a history of episodic constipation or abdominal distension. The treatment plan includes an initial reduction of the volvulus via sigmoidoscopy with rectal biopsy to rule out Hirschsprung disease; however, operative management to remove the dilated sigmoid colon may be required in the setting of recurrence or confirmed Hirschsprung disease. Although rare, SV should be considered in a child presenting with abdominal pain as a missed diagnosis can have high potential morbidity and mortality.
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Bhandari TR, Shahi S. Volvulus of sigmoid colon in a challenged adolescent: An unusual case report. Ann Med Surg (Lond) 2019; 44:26-28. [PMID: 31297192 PMCID: PMC6598602 DOI: 10.1016/j.amsu.2019.06.009] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2019] [Revised: 06/03/2019] [Accepted: 06/11/2019] [Indexed: 12/29/2022] Open
Abstract
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.
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Affiliation(s)
- Tika Ram Bhandari
- Department of General Surgery, People's Dental College and Hospital, Kathmandu, Nepal.,Formerly Department of General Surgery, Universal College of Medical Sciences, Bhairahawa, Nepal
| | - Sudha Shahi
- Department of ENT Head and Neck Surgery, National Academy of Medical Sciences, Kathmandu, Nepal
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A rare cause of acute abdominal pain in a child: Giant sigmoid volvulus. JOURNAL OF SURGERY AND MEDICINE 2018. [DOI: 10.28982/josam.464087] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
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Brumbaugh D, Watne L, Gottrand F, Gulyas A, Kaul A, Larson J, Tomezsko J. Nutritional and Gastrointestinal Management of the Patient With Duchenne Muscular Dystrophy. Pediatrics 2018; 142:S53-S61. [PMID: 30275249 DOI: 10.1542/peds.2018-0333g] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/26/2018] [Indexed: 11/24/2022] Open
Abstract
Advances in treatment and multidisciplinary management have resulted in improved survival of individuals with Duchenne muscular dystrophy (DMD). Updated DMD treatment recommendations as found in the 2018 DMD Care Considerations are aimed to assist multidisciplinary care teams in providing standardized care to their patients, including attention to nutritional and gastrointestinal health. Challenges remain for care teams in accurately estimating height and nutritional status for individuals with DMD. It can be difficult for patients to maintain a healthy weight. Risk factors for obesity include glucocorticoid therapy and loss of ambulation. In contrast, in the later stages of the disease, swallowing dysfunction can lead to poor nutrition and consideration for gastrostomy tube placement. Constipation is highly prevalent, underrecognized, and undertreated in DMD. With this article, we address the assessment and management of gastrointestinal and nutritional issues, as well as clinical controversies.
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Affiliation(s)
| | - Laura Watne
- Children's Hospital Colorado, Aurora, Colorado
| | - Frederic Gottrand
- Jean de Flandre Hospital, Université de Lille, Centre Hospitalier Régional Universitaire de Lille, Lille, France
| | - Ann Gulyas
- Western Maryland Hospital Center, Hagerstown, Maryland
| | - Ajay Kaul
- Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
| | | | - Jean Tomezsko
- Medical Nutrition Consulting of Media LLC, Philadelphia, Pennsylvania
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Carmo L, Amaral M, Trindade E, Henriques-Coelho T, Pinho-Sousa J. Sigmoid Volvulus in Children: Diagnosis and Therapeutic Challenge. GE-PORTUGUESE JOURNAL OF GASTROENTEROLOGY 2018; 25:264-267. [PMID: 30320166 DOI: 10.1159/000486242] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/11/2017] [Revised: 12/11/2017] [Indexed: 11/19/2022]
Abstract
Background Sigmoid volvulus is a rare condition in children. Early diagnosis increases the success of nonsurgical treatment in the emergency department, but posterior elective surgery is important due to the risk of recurrence. Methods/Conclusions We present the case of a healthy 16-year-old girl with recurrent volvulus and successful endoscopic treatment followed by elective surgery.
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Affiliation(s)
- Leonor Carmo
- Department of Pediatric Surgery, Centro Hospitalar de Vila Nova de Gaia/Espinho, Porto, Portugal
| | - Marina Amaral
- Department of Pediatric Surgery, Centro Hospitalar de Vila Nova de Gaia/Espinho, Porto, Portugal
| | - Eunice Trindade
- Department of Pediatric Gastroenterology, Centro Hospitalar São João, Porto, Portugal
| | | | - José Pinho-Sousa
- Department of Pediatric Surgery, Centro Hospitalar de Vila Nova de Gaia/Espinho, Porto, Portugal
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Haider F, Al Asheeri N, Ayoub B, Abrar E, Khamis J, Isa H, Nasser H, Al Hashimi F. Sigmoid volvulus in children: a case report. J Med Case Rep 2017; 11:286. [PMID: 29110733 PMCID: PMC5674852 DOI: 10.1186/s13256-017-1440-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2016] [Accepted: 08/28/2017] [Indexed: 12/23/2022] Open
Abstract
BACKGROUND Sigmoid volvulus is frequently reported in the "volvulus belt" (Middle East, Africa, the Indian subcontinent, Turkey, and South America) and is the third leading cause of large bowel obstruction in North America. It is an uncommon problem in children and adolescents, and is rarely considered a diagnosis in this group. A high index of suspicion is necessary to diagnose sigmoid volvulus in children. CASE PRESENTATION We present a 13-year-old Arabian girl who came with features suggestive of intestinal obstruction. Plain abdominal film revealed classic omega (coffee bean) sign of sigmoid volvulus. The volvulus was successfully decompressed by means of a rectal tube in our emergency department. The next day during the same admission the volvulus recurred and was successfully decompressed by endoscopy. She was discharged home on her parents' request; she presented again 1 month later. This time the volvulus could not be decompressed non-operatively, so she underwent sigmoidectomy with primary anastomosis. Postoperatively she developed paralytic ileus that resolved after 10 days. Following that she did well and was discharged home. She is still free of symptoms 1 year after the resection. CONCLUSIONS Sigmoid volvulus is an uncommon problem in children and adolescents, and is rarely considered a diagnosis in this group as a cause of intestinal obstruction. Pediatric surgeons should maintain a high index of suspicion, in order not to miss this important diagnosis, as any delay in instituting treatment has a devastating effect on morbidity as well as mortality. Early diagnosis and prompt treatment confer an excellent prognosis.
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Affiliation(s)
- Fayza Haider
- Pediatric Surgery Unit-Department of Surgery, Salmaniya Medical Complex, P.O. Box 12, Manama, Bahrain. .,Salmaniya Medical Complex, 12, Manama, Bahrain.
| | - Nabeel Al Asheeri
- Pediatric Surgery Unit-Department of Surgery, Salmaniya Medical Complex, P.O. Box 12, Manama, Bahrain
| | - Barrak Ayoub
- Pediatric Surgery Unit-Department of Surgery, Salmaniya Medical Complex, P.O. Box 12, Manama, Bahrain
| | - Eizat Abrar
- Pediatric Surgery Unit-Department of Surgery, Salmaniya Medical Complex, P.O. Box 12, Manama, Bahrain
| | - Jawad Khamis
- Department of Medicine, Salmaniya Medical Complex, Manama, Bahrain
| | - Hasan Isa
- Department of Pediatrics, Salmaniya Medical Complex, Manama, Bahrain
| | - Husain Nasser
- Department of Radiology, Salmaniya Medical Complex, Manama, Bahrain
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Van Horebeek I, Hoffman I, Witters P. A coffee bean in an infant: Call the endoscopist. J Paediatr Child Health 2017; 53:516. [PMID: 28470801 DOI: 10.1111/jpc.13532] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/02/2016] [Revised: 11/25/2016] [Accepted: 12/04/2016] [Indexed: 12/22/2022]
Affiliation(s)
- Ilse Van Horebeek
- Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium
| | - Ilse Hoffman
- Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium
| | - Peter Witters
- Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium
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Khalayleh H, Koplewitz BZ, Kapuller V, Armon Y, Abu-Leil S, Arbell D. Neonatal sigmoid volvulus. J Pediatr Surg 2016; 51:1782-1785. [PMID: 27444245 DOI: 10.1016/j.jpedsurg.2016.06.017] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2016] [Revised: 05/26/2016] [Accepted: 06/25/2016] [Indexed: 11/26/2022]
Abstract
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.
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Affiliation(s)
- Harbi Khalayleh
- Department of General Surgery, Kaplan Medical Center, Rehovot, Israel
| | - Benjamin Z Koplewitz
- Department of Medical Imaging, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
| | - Vadim Kapuller
- Department of Pediatric Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
| | - Yaron Armon
- Department of Pediatric Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
| | - Sinan Abu-Leil
- Department of Neonatology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
| | - Dan Arbell
- Department of Pediatric Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
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Parolini F, Orizio P, Bulotta AL, Garcia Magne M, Boroni G, Cengia G, Torri F, Alberti D. Endoscopic management of sigmoid volvulus in children. World J Gastrointest Endosc 2016; 8:439-43. [PMID: 27358669 PMCID: PMC4919692 DOI: 10.4253/wjge.v8.i12.439] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2016] [Revised: 04/19/2016] [Accepted: 05/17/2016] [Indexed: 02/05/2023] Open
Abstract
Sigmoid volvulus (SV) is extremely uncommon in children and is usually associated with a long-standing history of constipation or pseudo-obstruction. An early diagnosis and management are crucial in order to prevent the appearance of hemorrhagic infarction of the twisted loop, avoiding further complications such as necrosis, perforation and sepsis. In patients with no evidence of peritonitis or ischemic bowel, treatment starts with resuscitation and detorsion of the SV, accomplished by means of sigmoidoscopy and concomitant rectal tube placement. The bowel is then prepared and surgery is undertaken electively during the same hospitalization. We report a detailed review of the literature focusing on technical details, risks and benefits of endoscopic management of SV in childhood.
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23
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Parolini F, Alberti D. Sigmoid volvulus in children. Surgery 2016; 161:562-563. [PMID: 26953115 DOI: 10.1016/j.surg.2016.01.020] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2015] [Revised: 01/04/2016] [Accepted: 01/30/2016] [Indexed: 01/27/2023]
Affiliation(s)
- Filippo Parolini
- Department of Paediatric Surgery, "Spedali Civili" Children's Hospital, Brescia, Italy.
| | - Daniele Alberti
- Department of Paediatric Surgery, "Spedali Civili" Children's Hospital, Brescia, Italy; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
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