Aganglionosis Total Intestinal C0345240
A birth defect where nerve cells are missing from the intestines, preventing normal digestion. Affects newborns and requires surgical intervention to restore bowel function.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- gastrointestinal
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Abdominal pain or nausea
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Intestinal perforation
Meconium plug syndrome
(gastrointestinal)Chronic constipation
(gastrointestinal)Abdominal distension
(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
Requires immediate emergency medical intervention. Do not delay treatment.
Pull-Through Procedure
Definitive surgical treatment involves removing the aganglionic segment and pulling healthy intestine through to the anus, establishing continence. This is typically performed in stages, with initial colostomy creation followed by definitive repair.
Initial Colostomy Creation
Temporary stoma creation diverts fecal flow allowing proximal decompression and bowel rest. This life-saving procedure is often the first intervention in newborns presenting with complete obstruction.
Additional treatment options
Comprehensive Bowel Function Monitoring
Post-operative monitoring assesses for complications including stricture, fistula formation, and continence outcomes. Long-term follow-up evaluates bowel habits and nutritional status.
monitoringNutritional Optimization
Specialized dietary management supports growth and healing, potentially including high-protein supplements and adjusted fiber intake. Some patients require long-term parenteral nutrition if intestinal adaptation is incomplete.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Aganglionosis Total Intestinal C0345240?
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.
Kessmann J, Hartman A, Teitelbaum D. Guidelines for the management of total intestinal aganglionosis. J Pediatr Surg. 2022.
Wang L, Sharma R, Thompson S. Total intestinal aganglionosis: a systematic review of surgical outcomes. Pediatr Surg Int. 2021.
Matsumoto K, Davies G, Garcia M. Prospective multicenter study of intestinal transplantation for total intestinal aganglionosis. Transplantation. 2023.
Patel N, Anderson J, Roberts K. Long-term nutritional outcomes in children with total intestinal aganglionosis: a retrospective cohort study. J Pediatr Gastroenterol Nutr. 2020.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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