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Cystic Hygroma

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By : Senty Annamalai

• Benign mal development of lymphatic-cavenous sacs with


sequestration of lymphatic tissue

• No longer communicates with the normal lymph system and


accumulation of fluid leads to cystic enlargement

• Appear early in life, generally posterior triangle (6% of all


benign lesions of infancy and childhood)
• are divided histologically into two major groups based on the
depth and the size of abnormal lymph vessels. The superficial
ones are called lymphangioma circumscriptum. The more deep
seated ones are cavernous lymphangioma or cystic hygroma
• Another classification is to divide them into three types as
capillary, cavernous and cystic .
• McGill and Mulliken proposed a classification of cystic
hygromas based on location, histology and CT findings .
• Type I malformations develop below the mylohyoid muscle and
are macrocystic involving the anterior and posterior triangles of
the neck.
• Type II malformations are found in the neck above the level of
the mylohyoid and are invasive and microcystic.
• Stage 1 : unilateral infrahyoid lesion
• Stage 2 : unilateral suprahyoid lesion
• Stage 3 : unilateral lesion etending both above and below the
hyoid
• Stage 4 bilateral infrahyoid lesion
• Stage 5 : bilateral lesion etending both above and below hyoid
Growth Pattern

• No skin involvement with cystic hygroma

• Grow along fascia planes and around neurovascular structures

• Largely asymptomatic (depending on size/location)


• Hemorrhage within the mass

• Infection

• Visual, auditory, oral or esophageal obstruction

• Airway obstruction
• Drainage

• Surgical Excision

• Sclerotherapy

• Radiofrequency ablation and laser excision (used for microcystic


lymphatic malformations)

• Combination therapy
• Needle aspiration alone is not indicated for definitive treatment

• Useful for emergent decompression of a cyst to relieve airway


obstruction or to facilitate delivery
• Long been the preferred method of treatment

• Downside is incomplete excision due to involvement of vital


structures

• CT or MRI is indicated to evaluate the full extent of the lesion


for surgical planning.
• Traditional approach is an incision overlying the mass

• Neck scars are very cosmetically unpleasing in various cultures.

• More recently, some have used an endoscopic approach


through the anterior chest. (Han, et al.)
• If the entire cyst can be resected, recurrence is practically zero.

• Subtotal resection leads to recurrence about 56%

• Partial resection has 86% recurrence

• Drainage only leads to nearly 100% recurrence.


• Agents include:
• OK – 432
• Bleomycin – toxicity limits use
• Doxycycline
• Ethanol – widely used in the US due to its availability
• Sodium Tetradeyl Sulfate
• Fibrin glue
• OK – 432 (Picibanil) : Freeze-dried, heat and penicillin-killed,
mixture of group A Streptococcus pyogenes

• Shown to cause either total or marked shrinkage of cystic


lesions without scarring of the overlying skin.

• Found to be superior to bleomycin therapy with less toxicity.

• Better for macrocystic lesions than for microcystic.


• Radiofrequency ablation involves either a high (deep) or low
(superficial) radiofrequency to destroy tissue.

• Temperatures reached are less than with traditional cautery so


surrounding tissue thermal damage is lessened.

• Mainly useful with microcystic lesions as macrocystic lesions are


too large to be dealt with in this way.
• A combination of cyst aspiration and injection of OK – 432 has
been shown to markedly reduce the mass size.

• Recurrence did not occur with any of the patients in the study

• Complications from combination aspiration and sclerotherapy


were minimal.
• UNLIKE IN HEMANGIOMAS, SPONTANEOUS RESOLUTION OF
CH IS UNCOMMON.
• RECURRENCE IS RARE WHEN ALL GROSS DISEASE IS
REMOVED.
• IF RESIDUAL TISSUE IS LEFT BEHIND, THE EXPECTED
RECURRENCE RATE IS APPROXIMATELY 15%.
• IN PRENATAL CH, DIAGNOSIS AFTER 30 WEEKS' GESTATION
IS CONSIDERED A POSITIVE PROGNOSTICATOR.
S. Ogita, T. Tsuto, K. Nakamura, E. Deguchi, N. Iwai, OK-432 Therapy in 64 Patients With
Lymphangioma, Journal of Pediatric Surgery. 29 (1994) 784-785.

N. Mitsukawa, K. Satoh, New Treatment for Cystic Lymphangiomas of the Face and Neck:
Cyst Wall Rupture and Cyst Aspiration Combined With Sclerotherapy, The Journal of
Craniofacial Surgery. 23 (2012) 1117-1119.

J. Oosthuizen, P. Burns, J. Russell Lymphatic malformations: A proposed management


algorithn, International Journal of Pediatric Otorhinolaryngology. 74 (2010) 398-403.

S. Kim, K. Kauvanough, D. Orbach, A. Alomari, J. Mulliken, R. Rahbar, Long-term


Outcomes of Radiofrequency Ablation for Intraoral Microcystic Lymphatic Malformation,
Archives of Otolaryngology Head and Neck Surgery. 137 (2011) 1247-1250.

L. Nielsen, B. Charabi, F. Jensen, G. Claesson, P. Bretlau, Cystic hygroma: OK-432 is superior to surgery,
International Congress Series. 1254 (2003) 519-522.

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