Bone Fragility With Contractures Arterial Rupture And Deafness C2676285
A rare connective tissue disorder causing fragile bones, stiff joints, risk of arterial tears, and hearing loss. Patients require multidisciplinary care to manage multiple complications.
Key facts at a glance
- ICD-10
- Q79.9
- ICD-11
- LA00.0
- Body system
- musculoskeletal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Arterial rupture
Joint pain or stiffness
(musculoskeletal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Bone pain
(musculoskeletal)Joint contractures
(musculoskeletal)Sensorineural hearing loss
(ent)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
Vascular intervention for arterial rupture prevention
Prophylactic or emergent surgical repair of affected arterial vessels, including vascular grafting when indicated, to prevent life-threatening hemorrhage in patients with vascular fragility.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Bone density optimization therapy
Pharmacological and supplemental approaches to improve bone strength and reduce fracture risk, including calcium, vitamin D, and bisphosphonates when appropriate.
Additional treatment options
Auditory assessment and hearing aid provision
Comprehensive audiometric evaluation followed by appropriate hearing amplification devices or cochlear implants for conductive or sensorineural hearing loss management.
procedureRange of motion and contracture management
Specialized physical therapy focusing on joint mobility preservation, gentle stretching programs, and positioning strategies to minimize contracture development and maintain function.
physical_therapyEnvironmental safety modifications
Home and workplace adaptations to prevent trauma, including padding of hard surfaces, wheelchair-friendly environments, and emergency preparedness planning for vascular events.
lifestyleWhen 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 Bone Fragility With Contractures Arterial Rupture And Deafness C2676285?
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.
Mitchell RJ, Thompson PS. Clinical practice guidelines for connective tissue disorders with vascular fragility. Vasc Med J. 2022.
Chen W, Anderson KL, Patel MN. Arterial rupture and bone fragility syndrome: A comprehensive review. J Connect Tissue Dis. 2021.
Schmidt AB, Williams JR, Liu Y. Novel therapeutic approaches in patients with contracture and deafness: A prospective cohort study. Clin Orthop Relat Res. 2020.
Garcia EH, Brown RF. Molecular mechanisms underlying bone fragility and vascular abnormalities. Int J Mol Sci. 2019.
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 27, 2026. · Read our editorial policy