Ehlers Danlos Syndrome Kyphoscoliotic Type 1 C0268342
A genetic condition causing abnormal connective tissue development, leading to progressive spine curvature and joint hypermobility. Often appears in childhood. Treatment focuses on physical therapy, bracing, and orthopedic care to manage 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.
Congenital scoliosis
(musculoskeletal)Joint pain or stiffness
(musculoskeletal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Joint hypermobility
(musculoskeletal)Muscle hypotonia
(neurological)Scleral fragility
(ocular)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Spinal Stabilization and Postural Training
Specialized physical therapy focusing on spinalcore strength, postural awareness, and breathing exercises to support the kyphoscoliotic spine and prevent curve progression.
Scoliosis Curve Progression Assessment
Regular radiographic monitoring of spinal curvature, pulmonary function testing, and orthopedic evaluation to track disease progression and surgical timing.
Additional treatment options
Spinal Deformity Correction Surgery
Surgical intervention for severe scoliosis or kyphosis when curves progress beyond acceptable parameters, involving spinal fusion and instrumentation by experienced orthopedic surgeons.
surgicalBracing and Orthotic Support
Use of spinal bracing to provide external support and potentially slow curve progression, combined with adaptive equipment for daily activities.
lifestyleNutritional Support for Connective Tissue
Dietary optimization including adequate protein, vitamin C, zinc, and other nutrients essential for collagen synthesis and connective tissue health.
dietaryWhen 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 Ehlers Danlos Syndrome Kyphoscoliotic Type 1 C0268342?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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 20, 2026. · Read our editorial policy