Cystic Hygroma (Cystic Lymphangioma)

Last updated 20.09.12

 

Features

-  Failure of lymphatics to connect to venous system

-  Can effect any anatomic site

-  75% head & neck, post. triangle most common

-  20% in axilla

-  Also mediastinum, groin, retroperitoneum

-  Left > right

-  1 per 6,000 – 16,000 live births

 

Causes

-  Congenital

-  50–60% present at birth

-  80–90% by 2 years

-  50% chromosomal: Turners, Downs, Trisomy 18, 13

-  Noonan, Klinefelter, Fryns syndromes, achondroplasia, multiple pterygium

-  Acquired: trauma, Sx, inflammation, lymph obstruction

 

Associated S/S

-  Soft, painless, compressible mass which transilluminates

-  Airway compromise: stridor, cyanosis, resp. distress

-  Faliure to thrive

-  Sudden increase in size: bleeding, infection

-  Complications

-  Pneumonia, bronchiextasis

-  Airway compromise

-  Infection --> abscess

-  Gorham-Stout syndrome: bone dissolution

 

 

DDx

- Neck Mass

 

Ix

-  CT: margin enhancing lesion, sharp demarcation

-  MRI: hyperintese lesion T2, hypointense T1

-  DDx: haemangiomas

-  US

-  Relationship to surrounding structures

-  In utero Dx by nuchal translucency

-  Visualised via transabdominal by 10 weeks (TV better quality)

-  Neck X-ray: airway compromise

 

Mx

-  Intubation, tracheostomy & NGT

-  ENT referral if effects neck

-  OK432 (Picibanil) Sclerosis: inactive bacteria --> inflammation & fibrosis

-  EtOH sclerosis

-  Doxycycline sclerosis

-  Surgery

-  Complete excision possible in < 40%

-  High recurrence, most in 1st year

 

 

 

References