cystic hygroma

[အာကာလြင္]

Background
First described by Wernher in 1843, cystic hygroma (CH) is a cystic
lymphatic lesion that can affect any anatomic subsite in the human
body. CH is synonymous with cystic lymphangioma, which is also known
as a macrocystic lymphatic malformation and was first described in
1828 by Redenbacker.
sites
CH usually affects the head and neck (approximately 75%), with a left-
sided predilection. Within the neck, the posterior triangle tends to
be most frequently affected. Approximately 20% of CHs occur in the
axilla; more infrequent subsites include the mediastinum, groin, and
retroperitoneum.
Differential Diagnoses
Teratomas and Other Germ Cell Tumors
Other Problems to Be Considered
Branchial cleft cyst
Thyroglossal duct cyst
Ranula
Goiter
Soft tissue tumors
Neck abscess

Laboratory Studies
Studies have suggested that fluorescent in situ hybridization (FISH)
can be used to evaluate for cystic hygroma.
Imaging Studies
MRI, CT scanning, and ultrasonography are all helpful in delineating
the nature of a cystic neck mass. CT scanning and MRI reveal ringlike
margin enhancement with sharp demarcation of cystic areas. The cystic
areas tend to appear circumscribed and discrete. A poorly defined
isodense mass that obscures muscle and fatty planes is more consistent
with a microcystic lymphatic malformation than a CH.
Treatment
Medical Care
Although some authors have reported watchful waiting of cystic hygroma
(CH), it should be considered only in patients who are asymptomatic.
The medical treatment of CH consists of the administration of
sclerosing agents. Sclerosing agents include OK-432 (an inactive
strain of group A Streptococcus pyogenes), bleomycin, pure ethanol,
bleomycin, sodium tetradecyl sulfate, and doxycycline.
Surgical Care
The mainstay of treatment is surgical excision. Although surgery is
the criterion standard for treatment, both the operating team and the
family of the patient should go forward with the knowledge that CH is
a benign lesion. If acute infection occurs prior to resection, surgery
should be delayed at least 3 months.
• The surgical team should attempt to completely remove the
lymphangioma or to remove as much as possible, sparing all vital
neurovascular structures. Complete excision has been estimated to be
possible in roughly 40% of cases.
• CHs are ideally removed in one procedure because secondary excisions
are complicated by fibrosis and distorted anatomical landmarks.
• Microcystic lesions are much more difficult to remove because of
their intimate association with nearby tissues. Laser therapy is a
recent advancement in the treatment of microcystic lesions.
Diet
No special dietary requirements are necessary for patients with CH.
Activity
Patients with CH should be directed to avoid direct trauma to the area
because intralesional bleeding or infection can be precipitated by
trauma.
Medication
Sclerosant therapy, as described in Treatment, is the only medical
therapy available to treat cystic hygroma.
Follow-up
Complications
• Complications include airway obstruction, hemorrhage, infection, and
deformation of surrounding bony structures or teeth if left untreated.
Complications from the surgical excision of a cystic hygroma

[အာကာလြင္]

[mr. pearl]

Dear dr,let me ask some questions...

• firstly,why does CH affect head and neck(75%),with a left-sided predilection?(embryology and pathogenesis)

• second,in case of diagnosis,can we do other easy methods like taking sample of liquid by syringe and lab examination for type of fluid?(supposing we are in a rural hospital)

• third,let me know the criteria for surgical treatment?(as complete excision can be expected only in 40% of cases and a lot of complications if secondary excisions require)

i would be grateful if u fulfil my curiosity,thank for imparting of knowledge

2009/11/30 အာကာလြင္ <kaungk...@gmail.com>

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[အာကာ လြင္]

1.There is no known cause for these lymphatic abnormalities.But assumed that CH affect head and neck (75%) is due to rich presence of vascular endothelial growth factor C (VEGF-C) in these region and its receptors may play an important role in the development of lymphatic malformations.
2.left-sided predilection is clear that lymphatic system involved ';
  1)left sided flow(75%)
  2)right sided flow(25%)
Area supplied by lymphatic system in left is increased than right.
left sided flow is carried lymphatic fluid from all lower limbs and left sided upper body.It acts to remove bacteria and certain proteins from the tissues, transport fat from the small intestine, and supply mature lymphocytes to the blood., returning to the venous bloodstream through the thoracic duct.
3.CH is highly associated with neurovascular malformation and cyst wall pentrated to nearby structure ,esp.. in head and neck ,so we can do other easy methods like taking sample of liquid by syringe but it is the most  dangerous  method and can cause complications ( eg, aneurysm , bleeding , nerve injury ) .If possible we can do ;

• MRI: MRI is the consensus study of choice. It provides the best soft tissue detail and can delineate the relationship of the lesion to underlying structures. Contrast can be used to differentiate hemangiomas from lymphangiomas. On MRI, CHs appear hyperintense on T2-weighted images and hypointense on T1-weighted images.
• CT scanning: CT scanning is faster and may be more readily available than MRI. CT scanning carries the risk of radiation exposure, and detail is lost if the CH is surrounded by tissue of similar attenuation. Contrast helps to enhance cyst wall visualization and the relationship to surrounding blood vessels. On CT scans, CHs appear isodense to cerebrospinal fluid (CSF).
• Ultrasonography: This is the least invasive study. It is very useful in demonstrating the relationship of CH to the surrounding structures. Ultrasonography has limited ability in assessing mediastinal and retropharyngeal structures. It can be used to detect CH in utero. Echographic visualization of multiple septae in fetal CH has been postulated to be a poor prognostic indicator.
• Plain radiography: With any large mass of the head and neck, airway radiography (high-kilovolt anteroposterior and lateral neck radiographs or airway fluoroscopy) can be helpful in delineating possible airway compromise. Plain radiography is a reasonable initial imaging modality in the evaluation of a neck mass with a potential airway manifestation.
• Lymphoscintigraphy: A case report highlighted the ability to visualize CH using lymphoscintigraphy.

4.criteria for surgical treatment is depending on the anatomical location,embryological defect and investigation result .It is referral to a surgeon or surgical specialist is appropriate. In patients with CH of the head and neck, referral to an otolaryngologist is appropriate.

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              ေလးစားစြာျဖင္႕
        အာကာ( Kaungkinsky)
   

[mr. pearl]

very thank for yr complete explanation
wishing u able to discuss more and make us widen our horizons!