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Enamel Pitting A Common Sign of Tuberous Sclerosis GREG MLYNARCZYK 1100 Sorlotrrrr Aul’rlrlr’ Ko.cr1, crllifol-tz~~r 95405
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SELECTION OF PATIENTS Fifty patients with tuberous sclerosis (TSC), 3-44 years of age, who presented with varying degrees of disease involvement. were evaluated. Twenty-four patients (48%) were male and 26 (52%) were female. The diagnosis of each patient was made by a qualified medical specialist. Each patient had at least three of the following clinical features of TSC: facial angiofibroma, hypomelanotic macules of the skin, intracranial calcifications, retinal hamartomas, shagreen patch, cardiac rhabdomyoma, renal angiomyolipoma, cortical tubers, ungual fibroma. seizures, or an immediate relative with TSC.’,’
SELECTION OF CONTROLS The study evaluated 250 patients not known to have TSC. They were chosen at random a s a control group from a general dentistry practice. The patients ranged in age from 3-75 years. the majority being greater than I 1 years and returning for routine dental checkups. Ofthese patients, 105 (42%))were male and 145 (58%) were female (TABLE I).
STUDY METHODS AND MATERIALS As a standardized technique in examination of patients for enamel pitting was lacking, a protocol was developed and used for each patient in this study. I t was one that could easily be duplicated. The technique is based on the use of dental plaque, disclosing stain that provides a remarkable color contrast, allowing the detection of many small and subtle enamel pits (FIG.1 ). Examination requires surgical gloves, cotton rolls, disclosing solution (TRACE, Lorvic Corp.; a liquid dental disclosing dye containing F D & C Red #3), 6-inch cotton swabs, and a sterile dental explorer (Premier pigtail f 1 2 ) . Examinations can be performed by nondental personnel and are noninvasive.
SUMMARY Dental enamel pitting as a diagnostic sign of tuberous sclerosis (TSC) is explored and a protocol for the oral examination of patients and individuals at risk is described.’-h In the study, 50 patients with TSC and 250 control patients were 367
ANNALS NEW YORK ACADEMY OF SCIENCES
368
Enamel Pits in 50 Patients with TSC and 250 Control Subjects before and after Age 1 1 Years (Mlynarczyk, 1989)
TABLE 1.
TSC Controls
1612 I 4/20
76 20
29/29 171230
I00
7
45150 2 I1250
90 9
FIGURE 1. Multiple red-stained pits on normal-looking enamel.
examined for dental enamel pitting. A simple clinical protocol was established for examination using a dental disclosing solution swabbed on dry teeth. The incidence of enamel pitting in the adult dentition of patients with TSC was 10096, whereas that in the adult dentition of the control group was 7%. The simplicity of the test and the high probability of occurrence in ‘TSC make it a n important tool in the diagnosis of this serious genetic disease. REFERENCES GOMEZ,M. R., ed. 1988. Tuberous Sclerosis. 2nd ed.: 1-20, 179-189, 213-231. 257259. Raven Press. New York. 2. FI E U R Y , J. E., I). DEBOETS,N . MAFFRE.c. AUCLAIR-ASSAAD, c. BROCHEKIOU & A . SOULIE. 1984. Rev. Stomatol. Chir. Masillofac. 85: 375-382. 3. HOFF, M . , M . F. VAN GRUNSVEN, W . L. TONGLEBLOED & E. J . GRAVENMADE. 1975. Enamel defects associated with tuberous sclerosis. Oral Surg. Oral Med. Oral Pathol. 40: 261-269. I.
MLYNARCZYK: ENAMEL PI'ITING IN TSC
369
4. WEITS-BINNERTS, J. J., M. HOFF& M. F. VAN GRUNSVEN. 1982. Dental pits in deciduous teeth, an early sign in tuberous sclerosis. Lancet 11: 1344-1345. 5 . CABALLERO, L. R., J. L. DE ROBLES,C. R. CABALLERO, S . E. Yus & H. B. MORO. 1987. Tooth pits: An early sign of tuberous sclerosis. Acta Derm. Venereol. (Stockh.) 67: 457-459. 6 . LYGIDAKIS, N. & D. LINDENBAUM. 1987. Pitted enamel hypoplasia in tuberous sclerosis patients and first-degree relatives. Clin. Genet. 3 2 216-221.
Enamel Pitting A Common Sign of Tuberous Sclerosis GREG MLYNARCZYK 1100 Sorlotrrrr Aul’rlrlr’ Ko.cr1, crllifol-tz~~r 95405
Srrrlrrl
SELECTION OF PATIENTS Fifty patients with tuberous sclerosis (TSC), 3-44 years of age, who presented with varying degrees of disease involvement. were evaluated. Twenty-four patients (48%) were male and 26 (52%) were female. The diagnosis of each patient was made by a qualified medical specialist. Each patient had at least three of the following clinical features of TSC: facial angiofibroma, hypomelanotic macules of the skin, intracranial calcifications, retinal hamartomas, shagreen patch, cardiac rhabdomyoma, renal angiomyolipoma, cortical tubers, ungual fibroma. seizures, or an immediate relative with TSC.’,’
SELECTION OF CONTROLS The study evaluated 250 patients not known to have TSC. They were chosen at random a s a control group from a general dentistry practice. The patients ranged in age from 3-75 years. the majority being greater than I 1 years and returning for routine dental checkups. Ofthese patients, 105 (42%))were male and 145 (58%) were female (TABLE I).
STUDY METHODS AND MATERIALS As a standardized technique in examination of patients for enamel pitting was lacking, a protocol was developed and used for each patient in this study. I t was one that could easily be duplicated. The technique is based on the use of dental plaque, disclosing stain that provides a remarkable color contrast, allowing the detection of many small and subtle enamel pits (FIG.1 ). Examination requires surgical gloves, cotton rolls, disclosing solution (TRACE, Lorvic Corp.; a liquid dental disclosing dye containing F D & C Red #3), 6-inch cotton swabs, and a sterile dental explorer (Premier pigtail f 1 2 ) . Examinations can be performed by nondental personnel and are noninvasive.
SUMMARY Dental enamel pitting as a diagnostic sign of tuberous sclerosis (TSC) is explored and a protocol for the oral examination of patients and individuals at risk is described.’-h In the study, 50 patients with TSC and 250 control patients were 367
ANNALS NEW YORK ACADEMY OF SCIENCES
368
Enamel Pits in 50 Patients with TSC and 250 Control Subjects before and after Age 1 1 Years (Mlynarczyk, 1989)
TABLE 1.
TSC Controls
1612 I 4/20
76 20
29/29 171230
I00
7
45150 2 I1250
90 9
FIGURE 1. Multiple red-stained pits on normal-looking enamel.
examined for dental enamel pitting. A simple clinical protocol was established for examination using a dental disclosing solution swabbed on dry teeth. The incidence of enamel pitting in the adult dentition of patients with TSC was 10096, whereas that in the adult dentition of the control group was 7%. The simplicity of the test and the high probability of occurrence in ‘TSC make it a n important tool in the diagnosis of this serious genetic disease. REFERENCES GOMEZ,M. R., ed. 1988. Tuberous Sclerosis. 2nd ed.: 1-20, 179-189, 213-231. 257259. Raven Press. New York. 2. FI E U R Y , J. E., I). DEBOETS,N . MAFFRE.c. AUCLAIR-ASSAAD, c. BROCHEKIOU & A . SOULIE. 1984. Rev. Stomatol. Chir. Masillofac. 85: 375-382. 3. HOFF, M . , M . F. VAN GRUNSVEN, W . L. TONGLEBLOED & E. J . GRAVENMADE. 1975. Enamel defects associated with tuberous sclerosis. Oral Surg. Oral Med. Oral Pathol. 40: 261-269. I.
MLYNARCZYK: ENAMEL PI'ITING IN TSC
369
4. WEITS-BINNERTS, J. J., M. HOFF& M. F. VAN GRUNSVEN. 1982. Dental pits in deciduous teeth, an early sign in tuberous sclerosis. Lancet 11: 1344-1345. 5 . CABALLERO, L. R., J. L. DE ROBLES,C. R. CABALLERO, S . E. Yus & H. B. MORO. 1987. Tooth pits: An early sign of tuberous sclerosis. Acta Derm. Venereol. (Stockh.) 67: 457-459. 6 . LYGIDAKIS, N. & D. LINDENBAUM. 1987. Pitted enamel hypoplasia in tuberous sclerosis patients and first-degree relatives. Clin. Genet. 3 2 216-221.
