Tuberous sclerosis can manifest itself by multiple facial nodules affecting primarily the nose, cheeks, chin, and the nasolabial folds. A simple tangential (shave) excision of these facial tumors is believed to be adequate treatment for some patients

Brown, M R

Parsa, F. Don

eVols at University of Hawaii at Manoa

Shave Excision of Facial Tumors inTUberous SclerosisMichael R. Brown MD, PhD,* F. Don Parsa MD**Tuberous sclerosis can manifest itself by multiple facial nodulesaffecting primarily the nose, cheeks, chin, and the nasolabial folds.A simple tangential (shave) excision of these facial tumors isbelieved to be adequate treatment for some patients.IntroductionTuberous sclerosis is one of the large groups of neurocutaneousdiseases seen by plastic surgeons, dermatologists, and other practitioners because oftheir cutaneous presentations. In 1880, Bournevilleassociated these cerebral lesions with those of the face, and in 1890Pringle described the skin lesions of tuberous sclerosis in detail.Tuberous sclerosis is a systemic disorder characterized by a triad offacial lesions, epilepsy, and mental retardation. There is no sexualpredeliction and the inheritance pattern is probably autosomaldominant with variable penetrance. The incidence of tuberoussclerosis is one in 20,000. In the past, the facial lesions have beeninaccurately called adenoma sebaceum; more recent studies haveclassified these nodules as angiofibromas that microscopicallyshow interlacing strands of fibroblasts and collagen with numeroussmall blood vessels. Histopathologically, tuberous sclerosis manifests itself by hyperplasia of ectodermal cells of the skin, brain,heart, kidney, and retina.The facial distribution of these nodules is quite characteristic andpresents in childhood over the cheeks, nasolabial folds, and chin, aswell as over the nose and forehead. The most common locations‘General surgery resident in trainingDepartment of SurgeryJohn A. Bums School of MedicineUniversity of HawaiiHonolulu, Hawaii“Professor of Surgery (Plastics)Department of SurgeryJohn A. Bums School of MedicineUniversity of HawaiiHonolulu, HawaiiCorrespondence and request for reprints:Michael R. Brown MDDepartment of SurgeryUniversity ot Hawaii1356 Lusitana Street, Sixth FloorHonolulu, HI 96813Office: (808) 586-2924Fax: (808) 537-7827overlie the nasolabial folds and the chin. Other skin manifestationsinclude hypomelanotic macules, subungual fibromas, and shagreenpatches.Alvarez first reported surgical excision and grafting of a massivetumor involving the right cheek in 1957, Divir and Hirshowitzdescribe cryosurgery ofthe facial lesions in 1980, and Mulliken firstsuccessfully treated these tumors with dermabrasian and limitedexcision in 1977.A review of the literature over the past 15 years reveals a paucityof case reports describing specific treatments. Two cases of faciallesions as they occur in tuberous sclerosis are presented, and we willdescribe and discuss the rationale for our mode of therapy.Case Report ILK, a 19-year-old Caucasian man, was referred for treatment ofmultiple facial tumors. The facial lesions consisted of smooth-surfaced pink to red nodules of various sizes localized to thenasolabial folds, cheeks, chin, and nose. The lesions had beenpresent since the patient was five years old and had enlarged slowlyand progressively. The patient also had a history of generalizedseizures with mild mental retardation. A cerebral hamartoma wasremoved at age 15 and a left frontal cyst was drained a few monthslater. The patient developed hydrocephalus and required a ventricular shunt at 16. Physical examination revealed a well-developedman with mild mental retardation.Multiple nodules, measuring from 1 mm to 8 mm in diameter,were present on the face (Fig 1). These were particularly abundantover both nasolabial folds and the chin but also involved the noseand the upper lip, with several small nodules present in the leftretroauricular region. The patient underwent tangential excisionwith Nos 10 and 15 blades. The wounds were covered with Xeroformgauze. Within 10 days the skin was healed, allowing easy removalofthe gauze. The patient’s postoperative appearance two years aftersurgery is seen in Figure 2.Case Report 2SW, an 18-year-old Caucasian man, was admitted for control ofseizures and management of skin tumors. The patient had a historyof mental retardation and a history of generalized seizure disordersince the age of three months. The facial lesions had been presentsince he was four. Physical examination showed a well-developedyoung adult with obvious mild mental retardation. There weremultiple nodules present over the nasolabial folds, cheeks, and chin(Fig 3).This patient underwent tangential excision under general anesthesia, utilizing Nos 10 and 15 scalpel blades. The lesions were excisedto the level of adjacent healthy skin. The postoperative treatmentwas identical to case I and good healing took place within 12 dayswithout any complications. Figure 4 is the 12-month follow-upphotograph.HAWAII MEDICAL JOURNAL, VOL 55, JANUARY 199612Fig 3—Preoperative photograph of Case 2. Fig 4.—Postoperative photograph of Case 2.DiscussionThe postoperative results were satisfactory in both cases, andthere has been only minimal recurrence of the nodules in a one-yearfollow-up. We believe this conservative approach is adequatedespite the fact that we do not totally eradicate the skin disease. Theangiofibromas of tuberous sclerosis involve the full thickness of theskin. In these two cases with extensive involvement, total andcomplete removal would have required split thickness skin graftingfor resurfacing. Such an approach is unwarranted in this clinicalsetting. These patients do benefit from the aesthetic improvement ofsubtotal removal, and since these lesions grow slowly, the likelihood that they will require further surgery is small.In severe cases of tuberous sclerosis, approximately 30% of thepatients die before the age of five years and 50% to 75% die beforereaching adulthood. Those patients with lesser signs and symptomscan be expected to live longer, but might require further surgery forrecurrent skin tumors—a reasonable approach, considering theminimal morbidity of the procedure recommended.References1 Alvarez E. Unusual cutaneous manifestations of tuberous sclerosis: case report. Plast ReconstrSurg.1967; 40:153-154.2. DMr E, Hirshowltz B. The use of cryosurgery in treating the fibrous papules of tuberous sclerosis. AnnPlast Surg. 1980; 4:158-160.3. Drake DB, Morgan RF, Cooper PH. Shave excision and dermabrasion for facial angiofibroma intuberous sclerosis. Ann Plast Surg. 1992, 28:377-380.4. Fitzpatnck TB, Amdf K, Clark W, Eisen A, van Scott B, Vaughan JH. Dermatology in General Medicine.New York, NY: McGraw-Hill.5. Morgan JE, MullikenJB, Dermabrasion andlinIited excision of the fibrous papules of tuberous sclerosis.Plast Reconsfr Surg. 1977; 59:124-127.Fig 1.—Preoperative photograph of Case 1. Fig 2.—Postoperative photograph of Case 1.HAWAII MEDICAL JOUttNAL, VOL 55, JANUARY199613

Shave excision of facial tumors in tuberous sclerosis.

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Shave excision of facial tumors in tuberous sclerosis.

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