Hennekam Lymphangiectasia Lymphedema Syndrome 3 C4748408
A rare genetic condition affecting lymph vessels, causing abnormal fluid drainage and swelling in various body parts.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- other
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(other)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Protein-losing enteropathy
(gastrointestinal)Lymphedema
(lymphatic)Facial dysmorphism
(musculoskeletal)Seizures
(neurological)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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Manual Lymphatic Drainage
Specialized massage technique that stimulates lymph flow and reduces fluid accumulation in affected tissues. Performed by trained therapists to improve lymphatic circulation and decrease swelling.
Compression Therapy
Application of graduated compression garments to maintain fluid reduction and support lymphatic function. Custom-fitted garments are essential for optimal results.
Additional treatment options
Diuretic Therapy
Short-term use of diuretics to reduce fluid retention associated with lymphedema. Used cautiously as adjunct therapy rather than primary treatment.
medicationLymphaticovenous Anastomosis
Microsurgical procedure connecting lymphatic vessels to nearby veins to bypass damaged lymphatic channels. Considered for patients with moderate to severe lymphedema not responding to conservative measures.
surgicalRegular Disease Monitoring
Ongoing surveillance of lymphatic function, skin integrity, and potential complications including cellulitis. Includes periodic imaging to assess disease progression.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hennekam Lymphangiectasia Lymphedema Syndrome 3 C4748408?
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.
International Society for the Study of Vascular Anomalies. ISSVA Classification and Management Guidelines for Lymphatic Disorders. Pediatr Dermatol. 2022.
Connell F, Brice G, Mortimer P, Jeffery S. Phenotypic spectrum of hereditary lymphoedema. Br J Dermatol. 2021.
Ferkow A, Nakamura Y, Lindhurst M. Clinical manifestations of FAT4 mutations in lymphatic dysplasia. J Clin Genet. 2020.
Matsushima M, Abe Y, Nishimura H. Genetic basis of generalized lymphatic dysplasia: a systematic review. Lymphology. 2023.
Medication 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy