Kaposiform Lymphangiomatosis C5681097
A rare condition involving abnormal growth of lymphatic vessels. It primarily affects children and often involves multiple organs. Treatment may include chemotherapy and other interventions.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- other
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Respiratory distress
Generalized symptoms
(other)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lymphatic malformations
(lymphatic)Cutaneous lesions
(integumentary)Peripheral edema
(cardiovascular)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
Sirolimus Therapy
mTOR inhibitor therapy with sirolimus addresses the proliferative and lymphatic components of kaposiform lymphangiomatosis, often improving symptoms and stabilizing disease progression.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Multidisciplinary Surveillance
Regular monitoring by a multidisciplinary team including cardiology, pulmonology, and hematology is essential given the multi-system nature of kaposiform lymphangiomatosis and its complications.
Additional treatment options
Pleurodesis
Chemical or mechanical pleurodesis can control recurrent pleural effusions causing respiratory compromise, offering palliative benefit when conservative measures fail.
procedureInterferon-alpha Therapy
Interferon-alpha provides antiangiogenic and immunomodulatory effects that may help control disease activity, particularly for cutaneous or soft tissue involvement.
procedureSupportive Care and Compression Therapy
Compression garments and careful skin care help manage lymphatic complications and reduce risk of infection in affected limbs, while maintaining activity and quality of life.
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 Kaposiform Lymphangiomatosis C5681097?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Kaposiform Lymphangiomatosis C5681097. 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