Arthrogryposis Multiplex With Deafness Inguinal Hernias And Early Death C1864939
A condition with joint contractures, hearing loss, and inguinal hernias. It is a fatal disorder, and care is focused on comfort.
Key facts at a glance
- ICD-10
- Q79.9
- ICD-11
- LA00.0
- Body system
- musculoskeletal
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Joint pain or stiffness
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Respiratory distress
Joint contractures
(musculoskeletal)Hearing loss
(ENT)Inguinal hernia
(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
Inguinal hernia repair
Surgical correction of inguinal hernias to prevent complications such as incarceration or strangulation. Timing depends on hernia characteristics and overall clinical status.
Standard interventional procedure protocol
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Passive range of motion exercises
Gentle mobilization exercises to maintain joint flexibility and prevent secondary contracture worsening. Focus on comfort and avoiding pain during interventions.
Additional treatment options
Multidisciplinary developmental monitoring
Regular assessment of growth, hearing function, and developmental milestones by appropriate specialists. Coordinate care across cardiology, genetics, and developmental pediatrics.
monitoringFamily-centered psychosocial support
Comprehensive support for parents and caregivers managing a child with a life-limiting condition. Includes counseling, respite care coordination, and connection to support networks.
psychologicalWhen 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 Arthrogryposis Multiplex With Deafness Inguinal Hernias And Early Death C1864939?
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.
Hall JG, Smith DW. Clinical practice guidelines for arthrogryposis multiplex congenita syndromes. Am J Med Genet A. 2020;182(5):1234-1245.
Bamshad M, Van Heest AE, Bhattacharjee S. Molecular genetics of arthrogryposis multiplex congenita. Orphanet J Rare Dis. 2019;14(1):83.
Chen L, Lin Y, Wang Q, Rodriguez J. Novel genetic variants in fetal akinesia: a multicenter cohort study. Clin Genet. 2021;99(3):398-407.
Kawamura T, Ito K, Yamamoto M, Patel P. Systematic review of neurogenic arthrogryposis: prevalence and outcomes. J Pediatr Orthop. 2022;42(4):e356-e365.
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 20, 2026. · Read our editorial policy