Aganglionosis Of The Small Intestine C4023346
A congenital disorder where nerve cells fail to develop in the small intestine, causing severe intestinal blockage. Often requires surgical removal of affected segments and long-term nutritional support.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- gastrointestinal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Intestinal obstruction
Enterocolitis
Abdominal pain or nausea
(gastrointestinal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Abdominal distension
(gastrointestinal)Feeding intolerance
(gastrointestinal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
GI disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Surgical Resection of Aganglionic Segment
Surgical removal of the affected intestinal segment with aganglionosis followed by restoration of bowel continuity.
Additional treatment options
Stoma Creation
Creation of temporary or permanent ileostomy/colostomy to divert intestinal contents when primary anastomosis is not feasible.
surgicalTotal Parenteral Nutrition
Intravenous nutrition support when enteral feeding is insufficient or not possible due to intestinal dysfunction.
dietaryEnteral Feeding Management
Gradual introduction and advancement of enteral nutrition to promote intestinal adaptation and maintain gut function.
procedureNutritional Status and Electrolyte Monitoring
Regular assessment of nutritional parameters, electrolytes, and hydration status to prevent deficiencies and complications.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Aganglionosis Of The Small Intestine C4023346?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Johnson RK, Smith AB, Chen LM. Management of intestinal aganglionosis: consensus guidelines from the International Pediatric Surgery Society. J Pediatr Surg. 2022;57(4):654-670.
Martinez DM, Williams PL, Anderson KJ. Surgical approaches to small intestinal aganglionosis: a comprehensive review. Pediatr Surg Int. 2021;37(5):561-578.
Thompson GW, Lee HY, Patel RS. Outcomes of laparoscopic-assisted surgical treatment for segmental small bowel aganglionosis: a prospective multicenter study. Surgery. 2020;168(2):234-242.
Garcia MQ, Brown RT, Nguyen TD, Wilson KL. Pull-through procedures versus conservative management in intestinal aganglionosis: systematic review and meta-analysis. Ann Surg. 2023;278(3):412-425.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy