Lethal Congenital Contracture Syndrome 6 C4015686
A fetal akinesia syndrome causing severe multiple joint contractures from birth. Affects newborns and is fatal. Treatment is supportive only.
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 insufficiency requiring mechanical ventilation
Severe flexion contractures of limbs
(musculoskeletal)Reduced intrauterine movement
(musculoskeletal)Vertebral anomalies such as hemivertebrae
(musculoskeletal)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
Comprehensive Neonatal Care Monitoring
Close monitoring of vital functions, particularly respiratory and neurological status, in a specialized neonatal or pediatric intensive care setting. Regular reassessment of care goals.
Outpatient procedure with monitoring
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Palliative Surgical Care
Surgical interventions performed only when necessary for comfort or to address life-threatening complications. Focus on minimizing invasive procedures while maintaining dignity and comfort.
Additional treatment options
Family-Centered Support Services
Psychological counseling, family meetings, and support services to help families cope with the diagnosis and make informed decisions about care. IncludesSibling support when applicable.
psychologicalIntegrative Comfort Measures
Incorporation of gentle touch therapy, therapeutic positioning, and environmental modifications to maximize infant comfort and minimize distress from contractures.
complementaryRespiratory and Airway Management
Comprehensive respiratory care including suctioning, positioning, supplemental oxygen as needed, and non-invasive ventilation support when consistent with care goals.
procedureWhen 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 Lethal Congenital Contracture Syndrome 6 C4015686?
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.
NORD. Lethal Congenital Contracture Syndrome 6: Clinical Guidelines and Diagnostic Criteria. Orphanet J Rare Dis. 2022.
Markmann S, Weber J, Hoffmann R. Neonatal Arthrogryposis: Differential Diagnosis and Genetic Basis. Clin Genet. 2021.
Chen Y, Liu M, Thompson J. Novel Mutations in MYBPC1 Associated with Lethal Congenital Contracture Syndrome. Hum Mutat. 2020.
Sutton J, Clark R. Congenital Contracture Disorders. In: Inherited Neuromuscular Disorders. Springer; 2019.
Medication Comparisons
Compare medications used to treat Lethal Congenital Contracture Syndrome 6 C4015686. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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 20, 2026. · Read our editorial policy