University Journal of and Surgical Specialties

ISSN 2455-2860 Volume 2 Issue 4 2016

GIANT CYSTIC HYGROMA OF FACE AND

KARPAGA VINAYAGAM NATARAJAN Department of Paediatric Surgery, MADURAI MEDICAL COLLEGE AND HOSPITAL

Abstract : Keyword :Cystic Hygroma, antenatal diag- A 5 days old male child presented with nosis, surgical treatment antenatally detected huge Cystic Hy- (fig:1) groma, with a characteristic heart shaped Clinical History: double headed appearance. Child pre- Five days old male child admitted with an- sented with respiratory distress and was tenatally detected cystic lesion of face in- managed with early surgical excision volving the entire right half of the face. At successfully. Early surgery was per- birth child had a heart shaped double formed because the Cystic Hygroma was headed appearance (fig:1), with the lesion compressing the structures in the face stretching the eye and pushing the eye ball and neck, besides causing severe disfig- outside. The child had respiratory distress, urement. The paper is being presented tachypnoea with stridor and difficulty in for the interesting appearance of the sucking and taking feeds. child at birth and the successful surgical On general examination, the child was ac- outcome. Residual disabilities were cor- tive with a good cry and hydration. No his- rected with Bleomycin. These combina- tory of seizure disorder. Local examination tion modalities resulted in an almost nor- revealed a soft cystic swelling over the face mal looking child. involving forehead, cheeks, maxillary and mandibular region, with extension into the neck. There was a bulge over the inner buccal mucosa and lateral oral cavity with thinning of the bones and stretching of the eye lids. The ear lobe on the whole was pushed back. It was a fluctuant and bril- liantly trans-illuminant swelling (fig: 2)

An Initiative of The Tamil Nadu Dr M.G.R. Medical University University Journal of Surgery and Surgical Specialities without any pulsation.

Discussion: The child was put on antibiotics, and sup- are localized, congeni- plemented with nasal oxygen and IV sup- tal malformations of the lymphatic sys- plements. and ENT opin- tem that involve the skin and subcutane- ions were obtained. The antenatal USG ous tissues. In 1843, Wernher gave the was suggestive of a giant cystic hygroma first case report of a cystic Hygroma. (fig: 3 & 4). The MRI revealed a huge cystic This term comes from the Greek word swelling involving right parietal, maxillary “hygroth” meaning fluid and “oma” mean- and mandibular region with slight extension ing tumor. Lymphangiomas are seques- into the neck. There was compression over tration of lymphatics that fail to communi- the maxilla and the mandible with thinning cate with the vascular tree1. Lymphan- of the bones and medial displacement with giomas are more common the left as the thinning of orbital wall and displacement of Thoracic duct joins the Brachio Cephalic eyeball (fig: 5). Vein here. Spillage or sequestrations at (fig:2) these junctures leads to Cystic Hygroma. There was no evidence of any intracranial They are multi lobular and multi locular extension or loculations or septations. It cystic lesions. The walls consist of was associated with well defined wall ( fig : single layer of flattened endothelium. 6 ) with mild displacement of the vessels 50% present at birth and up to 90% are ( fig : 7 ). The other investigations were evident by the end of second year of life. within normal limits and the child was taken They are found most commonly in the up for surgery and a near total excision of head and neck, with the posterior cervi- the cyst was done through a curvilinear inci- cal region the commonest site2 and also sion, leaving a part of the cyst wall adherent in the axilla and abdomen. Cervical facial to the vital structures ( fig : 9 & 10 ). The Lymphangiomas are relatively uncom- child was progressively improving in the mon, representing fewer than 6% of be- post operative period except for minimal nign tumors of childhood. Incidence has collections which were drained and supple- been reported to be 1.2 to 2.8 per thou- mented with 2 courses of Bleomycin intra sand populations. Most studies report an lesional injection. The child improved sig- equal incidence among males and fe- nificantly well (fig: 12) and is on regular fol- males. low up. The biopsy report was consistent with .

An Initiative of The Tamil Nadu Dr M.G.R. Medical University University Journal of Surgery and Surgical Specialities

The treatment modalities are surgical excision or partial excision and medical management - intra lesional bleomy- 4 cin . Excision is the only effective avail- 5 able treatment because of the risks of spontaneous infection, progressive growth, airway compression and ob-

struction or dysphagia is relieved by surgical excision. Post operative com- plications include infection, Horner’s syndrome, recurrent laryngeal nerve palsy, damage to spinal accessory and Lymphangiomas are associated with marginal mandibular Turner’s, Klinefelter's and Noonan’s syn- nerves. Histologically, these tumors are dromes and exposure to alcohol during preg- 1. composed of dilated lymphatic chan- nancy It is necessary to differentiate these nels. The large empty lymphatic chan- lesions from other cervical congenital nels that are lined by a flattened layer masses or malignancy. The cystic nature of endothelial cells, they contain pro- and the bluish hue frequently resemble char- teinaceous fluid. The intervening acteristicsof a . Lesions in the stroma consists of fat, fibrous tissue floor of the mouth, particularly when they and skeletal muscle. The dilated bulge in the sub mental region, may simulate spaces can vary from microscopic to a plunging . Otherdifferential consid- large and cystic. They vary in size de- erations in the head and neck include a pending on the location and surround- , a Thyro glossal duct ing tissues. Landing and Farber were cyst, and goiter. Investigations of choice are the first to divide these lesions into two a Prenatal USG and MRI to r/o airway com- major groups based on the size of pression or obstruction. If present EXIT Pro- 1. st these abnormal lymphatic vessels. cedures are considered If diagnosed in 1 When a lymphangioma is confined to trimester with a Karyo typing abnormality rather dense tissue like the it there is a 59% chance for poor outcome. If st presents as a micro cystic lesion, but diagnosed in 1 trimester without a Karyo generally when it develops in the rela- typing abnormality there is a 89% chance for tively loose fascia of the neck a macro viability and 20% would go for a dysmorphic sequelae. If diagnosed in 2nd and 3rd trimes- ter (i.e. <30 weeks) prognosis is guarded. If diagnosed in 3rd trimester (i.e. >30 weeks) –

An Initiative of The Tamil Nadu Dr M.G.R. Medical University University Journal of Surgery and Surgical Specialities

(fig:6) disease must be kept in mind and no un- OTHER MODES OF TREATMENT: CO2 duly aggressive surgery entailing perma- laser treatment for superficial lesions, radia- nent loss must not be performed at the tion, steroids, and intra le- other extreme. 4 sional injection of OK – 432 , bleomycin A5 and vincristine.Lymphangioma is not a fatal disease. There is a 3% mortality rate. These deaths are usually due to broncho- spasm, atelectasis, or airway compromise from . There is no risk of malignant transformation. The growth rate is variable but most lesions tend to progress slowly. Recurrence rates following complete exci- sion range from 0 to 27% in various studies, and after partial excision the recurrence rate is reported to be between 50 to 100%. Most recurrences occurs within the first year of resection, but delayed recurrences (fi:8) as long as 10 years after the initial opera- References : tion have been reported. 1. Robert Arensman, jayant Agarwal, M Statter. Vascular and Lymphatic anomalies. Paediatric Surgery, Diag- nosis and management 2009: Vol 2 (Sec : 6): 1104 - 1107.

2 Thomas L. Kennedy. Cystic Hy- goma- lymphangioma: A rare and still unclear entity. The Laryngoscope, Oct 1989: Vol 99 ( S1): 1 - 10.

3 Ronald L.Thomas. Prenatal diagno- sis of giant cystic hygroma: Prognosis, Conclusion: counselling and management; case Cystic Hygroma varies from a simple cyst to presentation and review of the recent life threatening conditions like CHAOS literature. Prenatal Diagnosis Nov 92; (Congenital High Airway Obstruction). vol 12 (11): 919-23. Treatment also follows suit from Observa- tion, Injections to extirpation of the tumour. 4 Nasir saleem Saddal, Ahmad Sharif, Here we have successfully, managed a Soofiq Ahmad, Farhat Mirza. Intrale- grossly disfiguring tumour which was life sional bleomycin injection: A primary threatening and also causing gross distor- for peripheral lymphangio- tion of the anatomy employing all the mo- mas. Pakistan journal of medical sci- dalities. Hence we advocate intervention ences Apr – jun 2007; vol 23 (part – when Compressive features, and complica- 1), No:2. tions like infection and bleeding supervene. The benign nature of the

An Initiative of The Tamil Nadu Dr M.G.R. Medical University University Journal of Surgery and Surgical Specialities

5 Onur Ozen, Serdar Moralioglu, Ramazan Karabukut. Surgical Treat- ment of Cervicofacial Cystic Hygro- mas in children. Gazi university Medi- cal Faculty 2005.

(fig:9) (fig:10)

An Initiative of The Tamil Nadu Dr M.G.R. Medical University University Journal of Surgery and Surgical Specialities