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Introduction
Lymphangiomas are rare, congenital malformations of the lymphatic system. They most commonly occur in the head and neck,1 with 90 per cent presenting before two years of age.1 Presentation in adulthood is rare, with approximately 100 cases having been identified internationally.2 Whilst surgical resection remains the recommended treatment, a variety of non-surgical therapies (aspiration, injection of sclerosing agents, diathermy, radiation and observation) have been attempted in children, with varying success rates.
First used in 1987,3 the intracystic injection of OK-432, a lyophilised mixture of a low-virulence strain of group A Streptococcus pyogenes incubated with benzylpenicillin, is the primary therapy for lymphangiomatous lesions in Japan. Studies from Japan4,5 and the United States6-8 support the effectiveness of this treatment in reducing the size of lymphangiomas, particularly of the macrocystic type. Whilst this therapy is used frequently in children, its effectiveness in adults is unknown, with only two reports of such use appearing in the medical literature.9,10
We report an adult case of cystic lymphangioma successfully treated with OK-432, and we review the literature on OK-432 therapy.
Case report
A 31-year-old woman presented to the ENT out-patient clinic with a four-week history of a left-sided neck lump. The lump had appeared spontaneously four weeks after the normal, vaginal delivery of her first child. The lump was not inflamed or painful and the patient was systemically well. She had no past medical history and was taking no medication. She had no known allergies.
On examination, the patient had an obvious cystic lesion, which transilluminated, in the left supraclavicular fossa, lying above the middle third of the clavicle. The lesion was non-pulsatile and had no apparent solid component, and all overlying structures were mobile. The ENT examination, including the thyroid gland, was unremarkable, and there was no cervical lymphadenopathy. Nasendoscopy was normal.
An ultrasound (US) scan demonstrated a simple cyst measuring 5 cm in diameter. Aspiration drained 40 ml of fluid. Cytological analysis demonstrated nuclear pleomorphism, raising the possibility of a lymphoproliferative disorder.
The patient was admitted for formal removal of the cystic lesion under general anaesthesia. The cyst was drained and partially excised. Histological analysis demonstrated a cystic lymphangioma.
The patient re-attended six weeks later,...