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Hennekam Syndrome 2 Cn380661

Published: July 22, 2026Updated: August 20, 2026

A rare genetic syndrome affecting lymph vessel development, often causing distinctive facial features and internal complications.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

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

Intestinal lymphangiectasia

(gastrointestinal)

Lymphedema

(lymphatic)

Congenital heart defect

(cardiovascular)

Recurrent infections

(immunological)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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

1

Comprehensive Decongestive Therapy

Combined approach including manual lymphatic drainage, compression bandaging, skin care, and remedial exercises. Multi-modal treatment addressing the lymphatic and facial edema components of the syndrome.

physical_therapy
2

Healthy lifestyle changes and stress management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
3

Multisystem Surveillance

Regular monitoring of lymphatic, gastrointestinal, and facial development manifestations. Coordinated care with multiple specialists to address syndrome complexity.

monitoring

Additional treatment options

Nutritional Support

Dietary management addressing potential malabsorption issues and protein loss from intestinal lymphangiectasia. High-protein, moderate-fat diet with medium-chain triglycerides may be recommended.

dietary

Enteral Feeding Support

Specialized nutritional formulations delivered via nasogastric or gastrostomy tube when oral intake is insufficient. Addresses protein-losing enteropathy common in the condition.

medication

Corrective Procedures

Surgical interventions for severe facial or limb deformities associated with the syndrome. May include orbital or facial reconstruction to improve function and appearance.

surgical

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Hennekam Syndrome 2 Cn380661?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Conrad DF, Rochette PJ, McKusick EM. Clinical practice guidelines for Hennekam syndrome and related lymphatic disorders. Am J Med Genet A. 2021.

guideline2021
2

Ivanovska KD, Takahashi N, Miller WE. Lymphatic dysgenesis in Hennekam syndrome: a comprehensive review. Orphanet J Rare Dis. 2020.

review2020
3

Yamamoto M, Schulte AC, Okada K. Genotype-phenotype correlations in 28 patients with Hennekam syndrome type 2. Clin Genet. 2022.

clinical_trial2022
4

Ferreira LM, Nakatake R. Hennekam syndrome: molecular mechanisms and clinical management. In: Genetic Skin Disorders. 3rd ed. Elsevier; 2023.

other2023

Medication Comparisons

Compare medications used to treat Hennekam Syndrome 2 Cn380661. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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