Cryosurgery: an easy and cheap therapy for facial angiofibromas in tuberous sclerosis

ARTICLE

Auteur(s) : Carmelo Schepis, Maddalena Siragusa

Oasi Institute (IRCCS), Unit of Dermatology, Via Conte Ruggero, 73, 94018 Troina, Italy

Tuberous Sclerosis Complex (TCS) is a neurocutaneous syndromic disorder which affects skin, the central nervous system, the heart, kidney, lung and other sites of the human body. It is an autosomal dominant genetic condition related to two tumour suppressor genes located on chromosome 9 or chromosome 16 [1].

Cutaneous manifestations of TSC are considered to be part of the major features required for its diagnosis [1]. Among skin features, facial angiofibromas (FA) develop at approximately the age of 5 and become more evident with age. At first, FA are small pink or red papules, mostly located on the cheeks, nasolabial folds, nose and chin. After puberty they grow, may bleed and often cause discomfort for patients and their parents. For this reason, various modalities of treatment have been adopted: laser [2] dermoabrasion, electrosurgery and, recently, application of a tincture of podophyllin [3], or photodynamic therapy followed by pulsed dye laser [4]. These treatments, if repeated, result in acceptable cosmetic results. All surgical methods need previous local anaesthesia consisting of regional nerve block or EMLA application under occlusion over the affected area. Sometimes, general anaesthesia has been required. Only cryotherapy among the different surgical techniques, can be used without previous anaesthesia.

For this reason we recently treated two women, affected by TSC and aged 35 and 17, with cryosurgery, using the liquid nitrogen spray gun CRY-AC. They came to our observation with a request for a cosmetic improvement of their facial appearance. Both presented a very mild mental disability and had pharmacologically controlled epileptic seizures.

For surgical treatment, the thinnest nozzles were used to emit an open spray of liquid nitrogen and the application was repeated after an interval of some minutes. Both patients accepted easily the interventions and only referred to a mild burning sensation during the application and for some minutes after, together with a mild reddish color of the treated areas, which lasted for less than one hour. Both patients improved after a series of repeated treatments and reported feeling satisfied with the results of the therapy. The older patient, treated every 45 days, from autumn to spring for two years, obtained a dramatic improvement after ten applications (figures 1A, B). The younger had a less important, but good, benefit too after four applications.

Cryotherapy is anecdotally cited in the treatment of FA in patients with TSC; however, this method is barely documented for FA [5]. On the other hand, it is widely used in dermatology to destroy several types of benign lesion [6]. In our recent experience, we found it easy to apply and not requiring local anaesthesia with EMLA or regional nerve blocks because its administration produces only an initial mild pain that disappears in a few minutes. Thus, in selected patients, like the two treated by us, such a method does not need hospitalization or day surgery. In our experience, it is both effective and efficient, very cheap, easy to administer, convenient for the patients and repeatable from time to time, according to the clinical evolution. The side effects are minimal and predictable; moreover it can be used in parallel to other treatments currently available, such as lasers, in case of failure. However, additional experience is needed to reach a definitive opinion.

Acknowledgements

References

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3 Türkmen M, Ertam I, Ünal I, Dereli T. Facial angiofibromas of Tuberous Sclerosis: successful tretment with podophyllin. JEADV 2009; 23: 713-4.

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5 Duir E, Hirshowitz B. The use of cryosurgery in treating the fibrous papules of Tuberous Sclerosis. Ann Plast Surg 1980; 4: 158-60.

6 Thai K-E, Sinclair RD. Cryosurgery of benign skin lesions. Australas J Dermatol 1999; 40: 175-86.