The Surgical Treatment of Toxic Megacolon in Hirschsprung Disease

Khasanov, R. R.; Schaible, T; Wessel, Lucas; Hagl, Cornelia Irene

doi:10.1097/pec.0000000000000444

Cited by 5 publications

(9 citation statements)

References 12 publications

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“…[ 27 ] This severe condition can be the first manifestation in previously undiagnosed HD patients, and it usually caries high mortality rates. [ 26 ] Our patients also presented initial signs of toxic megacolon, such as fever, severely dilated colon, leukocytosis, and elevated inflammatory biomarkers, but fortunately the surgical intervention was performed soon enough in order to avoid a fatal outcome. The same authors underlined that in cases of toxic megacolon associated with HD, the medical treatment presents no benefits, and due to the high mortality rate of this condition, the patients should undergo a surgical resection of the massively distended colon even with ileostomy if needed in order to improve the survival rate.…”

Section: Discussionmentioning

confidence: 98%

“…HD-associated enterocolitis is the most severe complication of this condition that can lead in extreme cases to toxic megacolon. [ 11 ] Toxic megacolon is defined by total or segmental colonic dilation along with systemic toxicity, [ 26 ] and it can appear also associated with inflammatory bowel diseases. [ 27 ] This severe condition can be the first manifestation in previously undiagnosed HD patients, and it usually caries high mortality rates.…”

Section: Discussionmentioning

confidence: 99%

“…The same authors underlined that in cases of toxic megacolon associated with HD, the medical treatment presents no benefits, and due to the high mortality rate of this condition, the patients should undergo a surgical resection of the massively distended colon even with ileostomy if needed in order to improve the survival rate. [ 26 ] Therefore, the treatment of TCA consists in total colectomy with a bridging ileostomy followed by a surgical repair as a secondary intervention.…”

Section: Discussionmentioning

confidence: 99%

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Atypical onset of total colonic Hirschsprung disease in a small female infant

Mărginean

Meliţ²,

Gozar³

et al. 2018

Medicine

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Rationale:Hirschsprung disease (HD) or colonic aganglionosis is a congenital disorder, which results from the abnormal migration of neuronal cells of the neural crest leading to a disorder of the enteric nervous system consisting in the absence of ganglion cells within the submucosal and myenteric plexus.Patient concerns:We report the case of a 7-month-old female infant admitted in our clinic for constipation and failure to thrive. At the age of 6 months, she was examined in our clinic for the same reasons, and we recommended symptomatic treatment without improvements. The clinical examination revealed pallor of the skin and mucosa, distended abdomen, and abdominal tenderness at palpation.Diagnoses:The abdominal ultrasound showed abdominal bloating, and the barium enema was normal. The patient's evolution worsened progressively within the following 3 days after admission associating sings of toxic megacolon.Interventions:She underwent a surgical intervention with total colectomy and ileostomy, and the final diagnosis confirmed by the histological examination was of total colonic aganglionosis (TCA).Outcomes:The evolution immediately after the surgery and the follow-up examination after approximately 3 months pointed out normal weight gain and the laboratory tests were within normal limits.Lessons:TCA can also manifest in older infants. Barium enemas can guide the diagnosis in most cases of HD. Nevertheless, in patients with TCA, it can be normal. Moreover, it could represent a trigger for toxic megacolon.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Atypical onset of total colonic Hirschsprung disease in a small female infant

Mărginean

Meliţ²,

Gozar³

et al. 2018

Medicine

View full text Add to dashboard Cite

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“…[8] Toxic megacolon is quite rare in pediatric patients, but there were reported cases where toxic megacolon due to Hirshprung disease have been managed surgically. [9,10] These patients underwent emergency operation which involved extensive resection of the distended colon. Surgical colonic decompression with fecal diversion alone was reported to be associated with higher complication rate and mortality in treating toxic megacolon due to Hirshprung disease.…”

Section: Discussionmentioning

confidence: 99%

Communicating multiple tubular enteric duplication with toxic megacolon in an infant

Jang

Chung

2021

Medicine

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Rationale: Gastrointestinal tract duplication is a rare congenial anomaly which can be found anywhere along the gastrointestinal tract. While many patients are incidentally diagnosed during operation, in some cases it can present with severe gastrointestinal symptoms. In this case report, the patient presented with signs of toxic megacolon leading to rapid aggravation of inflammatory shock. Patient concerns: A 49-day old male infant presented with fever, poor feeding, and severe abdominal distension. Diagnosis: Abdominal ultrasonography was done. During the examination, a foley catheter was inserted through the anus to evaluate bowel patency and enable rectal decompression. The tip of the foley catheter was located in a separate narrower tubular lumen adjacent to the distended rectum. These findings suggested possibility of a tubular duplication cyst of the rectum as the culprit for the bowel obstruction. Interventions: The patient underwent emergency laparotomy. Findings showed multiple tubular intestinal duplications involving the ileum, appendix, cecum, descending colon, sigmoid colon and rectum. The true lumen of the rectosigmoid colon was completely collapsed while the adjacent tubular cyst remained severely distended and stool passage was not possible. Decompression of the sigmoid colon was done with loop colostomy with both the wall of the true bowel and enteric cyst forming the colostomy orifice. Outcomes: After 40 days of postoperative care, the patient was discharged with no immediate complications. Four months after the initial operation, colostomy take-down and transanal rectal common wall division was done. No complications were observed. Lessons: To our knowledge, this is the first case to be reported where a rare presentation of intestinal duplication resulted in an acute presentation toxic megacolon. Such emergency cases can be effectively treated with emergency surgical bowel decompression and elective common wall division.

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“…Although this is most frequently associated with ulcerative colitis, it may also occur in association with a variety of other conditions, including Crohn's disease, amebic colitis, pseudomembranous colitis, cholera, and bacillary dysentery. TM complicating Hirschsprung disease is quite rare [22].…”

Section: Discussionmentioning

confidence: 99%

Toxic Megacolon with Colonic Ischemia Masquerading as Diabetic Ketoacidosis: A Case Report

Mansouri¹

2020

SJMPS

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A previously healthy 12-year-old boy presented with abdominal pain and clinical and laboratory features highly suggestive of diabetic ketoacidosis. When his blood glucose plummeted and his urinary ketones disappeared within the first hour of insulin therapy, while his abdominal pain, acidosis and hemodynamic status failed to improve despite vigorous fluid resuscitation, the diagnosis of diabetic ketoacidosis was questioned. At laparotomy, gangrenous, hugely dilated large bowel was found, requiring a subtotal colectomy from the cecum to the sigmoid colon; leaving the patient with an ileostomy. The child survived a complicated postoperative course and is currently doing well.

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The Surgical Treatment of Toxic Megacolon in Hirschsprung Disease

Abstract: Because of the high mortality in patients with TM that were treated medically or with colonic decompression, a resection of massively distended part of the colon should be performed.

Cited by 5 publications

References 12 publications

Atypical onset of total colonic Hirschsprung disease in a small female infant

Atypical onset of total colonic Hirschsprung disease in a small female infant

Communicating multiple tubular enteric duplication with toxic megacolon in an infant

Toxic Megacolon with Colonic Ischemia Masquerading as Diabetic Ketoacidosis: A Case Report

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