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Basal cell nevus syndrome
Int. J. Oral Surg. 1979: 8: 63-66 (Key words: keralocysl; nevus; surgery, oral; basal cell nevlls)
Basal cell nevus syndrome A case report T. NAKAJIMA, T. YOKOBAYASHI AND M. ONISHI First and Second Departments of Oral Surgery, School of Dentistry, Niigata University, Niigata City, Japan
An ll-year-old boy with multiple dentigerous cysts in the maxilla and mandible is described. Other findings seen in the face, plantar skin, skeletal system and oral cavity indicated the lesions to be due to the basal cell nevus syndrome. This was further confirmed by the presence of similar abnormalities in his father and brother. ABSTRACT -
(Received fol' publication 24 October 1977, accepted 17 February 1978)
A panoramic radiograph showed many cystic lesions developing in the maxilla and mandible (Fig. 2). Frontal and parietal bossing and a mild mandibular protrusion were revealed in the cranial radiographs. A chest X-ray showed presence of a rudimentary sixth cervical rib and flaring of the left third rib at its tip. The canthal index calculated from the ratio of the distance of the medial to that of the lateral canthi was 41, which was larger than that of the normal value of 38. The plantar skin showed many small depressions which were histologically diagnosed as pitted keratolysis. Two nevoid lesions in the abdominal skin were lentigines with no carcinomatous change. Family history showed that his brother had three cysts, one in the mandible treated in 1972 (Fig. 3) and the other two which developed later on the right side of the maxilla and the mandible. Examination of his head and chest showed brachcephaly with frontal and parietal bossing and a bifid fifth rib on the right side. Cranial and panoramic radiographs of his father also disclosed bossing of the skull, calcification of the falx cerebri and
The main features of the basal cell nevus syndrome include nevoid basal cell carcinomas of the skin, multiple dentigerous cysts of the jaws and other skeletal anomalies. The syndrome is also often associated with a familial history. It is not uncommOn among Caucasians, but it appears to occur less frequently in the black race2 • Well-documented reports of the syndrome are also rare in Japan5 •
Case report An ll-year-old boy visited us on September 18, 1975 because of a swelling on the right side of his face (Fig. 1). External palpation showed an expansive swelling of the underlying bone. Intraorally, there were two swellings of bone on the buccal aspect of the right maxilla and mandible. The occlusion was an edge to edge bite. Other interesting findings were bifid uvula and a high arch of the palate. 0300-9785/79/010063-04$02.50/0
@
1979 Munksgaard, Copenhagen
64
NAKAJIMA, YOKOBAYASHI AND ONISHI
Fig. 1. Facial appearance of the patient at ini-
tial examination showing swelling on right side of face associated with broad nasal root and wide-set eyes.
three eytic lesions in the mandible (Fig. 4). He had a palate with a very high arch. According to his statement, his ,father, already deceased, had a large skull with frontal and parietal bossing.
Fig. 2. Panoramic radiograph showing many
cystic lesions in maxilla and mandible with impacted and displaced teeth.
Fig. 3. Postanterior radiograph of patient's
brother with large cystic lesion in mandible.
In consideration of the young age of the patient and multiple large cysts with impacted and displaced teeth, the lesions were treated by marsupialization. Walls of the maxillary cysts were excised on the buccal aspect to allow communication with the oral cavity in October 1975. The contents of the cysts were greasy, cheeselike materials as seen in kerato· cysts. The histologic diagnosis of each specimen was dentigerous keratocyst. The second operation for marsupialization of the mandibular cysts was performed in March 1976 by removing the buccal bone of the right canine region. The cysts were similar to those of the maxilla. Further marsupialization of the remaining cysts (cysts 3 and 6) and remarsupialization of the other cysts were performed twice in 1976. Obviously, all the cysts have shown a definite tendency to resolution after treatment, even though the untreated ones grew rapidly (Fig. 5). Orthodontic treatment is now being done to help eruption and alignment of the impacted and displaced teeth.
BASAL CELL NEVUS SYNDROME
65
Fig. 5. Panoramic radiograph of patient taken Fig. 4. Postanterior radiograph of patient's father showing three cystic lesions in mandible, calcification of falx cerebri and parietal bossing.
Discussion In the current case there were no carcinomatous sites to indicate the basal cell nevus syndrome, but support for it was offered by the characteristic appearance of the face and the bone survey which revealed multiple dentigerous cysts and skeletal defects such as the rudimentary sixth cervical rib, flaring of the third costal rib and bossing of the skull. The cysts of the jaws are not distinguishable from the keratocysts not related to the syndrome, except that they usually occur as multiple lesions. at an early stage of life 4 • Consequently, extensive displacement of many developing teeth is seen in the majority of cases. Another important aspect of the cysts is the high re-
in October 1976 (A) and in February 1977 (B) showing enlargement of untreated cysts (cysts 3 and 6) and resolution or reduction in size of cysts after treatment.
currence rate. Therefore, complete removal with care not to leave any component of the cyst wall has been recommended for its treatment. BROWNE! reported, however, that no difference was found in the ratio of recurrence following marsupialization, enucleation and primary closure, and enucleation and packing. As is often the case with other jaw cysts, simple marsupialization, though time consuming, may lead to gradual decrease of cystic lesions and spontaneous eruption of the impacted teeth. Orthodontic guidance is helpful in shortening the time required for their eruption and to obtain final proper ~clusion. A case was reported3 of the syndrome in which conservative treatment of two cysts in the maxilla and mandible followed by enucleation when they reached an operable size
66
NAKAJIMA, YOKOBAYASHI AND ONISHI
showed an excellent result. A conservative approach by marsupialization in our case had also produced a rapid reduction in the size of the radiolucent lesions accompanied by shifting of the positions of the impacted teeth toward the openings. Family history is often necessary to offer an additional support to establish a diagnosis of the syndrome. Cystic lesions were present in both the patient's brother and father. Examination of their faces and skeletal system also disclosed some of the characteristic abnormalities associated with the syndrome. Thus, we believe that the lesions found in the members of the family are due to the basal cell nevus syndrome with hereditary disorder even though the full symptoms are not present. Acknowledgments - We are grateful to Professor T. ISHIKI and other members of the Department of Oral Pathology, School of Dentistry, Niigata University, for histologic study of the specimens. Our thanks are also due to Professor N. TOKIWA of the First Department of Oral Surgery and Professor Y. OHASHI of the Second Department of Oral Surgery for their criticism.
References 1. BROWNE, R. M.: The odontogenic keratocyst. Clinical aspects. Br. Dent. I. 1970: 128: 225-231. 2. GIANSANTI, J. S. & BAKER, G. 0.: Nevoid
basal cell carcinoma syndrome in Negroes: report of five cases. I. Oral Surg. 1974: 32:
3.
138-144.
HICKORY,
1. E., GILliLAND, R. F.,
WADE,
W. M. & TAYLOR, C. G.: Conservative treatment of cysts of the jaws in nevoid basal cell carcinoma syndrome; report of case.
J. Oral Surg. 1975: 33: 693-697. 4. HoWELL, 1. B., UMAR BYRD, D., McCLENDON, J. L. & ANDERSON, D. E.: Identification and treatment of jaw cysts in the ne· void basal cell carcinoma syndrome. J. Oral Surg. 1967: 25: 129-138. 5. SUGAr, T., SHOJI, A. & YAMAMOTO, T.: Two cases of nevoid basal cell epithelioma syndrome in father and son suspected as multiple trichoepithelioma. lap. I. Derm. 1972: 82: 947.
Address: T. Nakajima First Department of Oral Surgery School of Dentistry, Niigata University 2·5274, Gakkocho, Niigata City Japan
Basal cell nevus syndrome A case report T. NAKAJIMA, T. YOKOBAYASHI AND M. ONISHI First and Second Departments of Oral Surgery, School of Dentistry, Niigata University, Niigata City, Japan
An ll-year-old boy with multiple dentigerous cysts in the maxilla and mandible is described. Other findings seen in the face, plantar skin, skeletal system and oral cavity indicated the lesions to be due to the basal cell nevus syndrome. This was further confirmed by the presence of similar abnormalities in his father and brother. ABSTRACT -
(Received fol' publication 24 October 1977, accepted 17 February 1978)
A panoramic radiograph showed many cystic lesions developing in the maxilla and mandible (Fig. 2). Frontal and parietal bossing and a mild mandibular protrusion were revealed in the cranial radiographs. A chest X-ray showed presence of a rudimentary sixth cervical rib and flaring of the left third rib at its tip. The canthal index calculated from the ratio of the distance of the medial to that of the lateral canthi was 41, which was larger than that of the normal value of 38. The plantar skin showed many small depressions which were histologically diagnosed as pitted keratolysis. Two nevoid lesions in the abdominal skin were lentigines with no carcinomatous change. Family history showed that his brother had three cysts, one in the mandible treated in 1972 (Fig. 3) and the other two which developed later on the right side of the maxilla and the mandible. Examination of his head and chest showed brachcephaly with frontal and parietal bossing and a bifid fifth rib on the right side. Cranial and panoramic radiographs of his father also disclosed bossing of the skull, calcification of the falx cerebri and
The main features of the basal cell nevus syndrome include nevoid basal cell carcinomas of the skin, multiple dentigerous cysts of the jaws and other skeletal anomalies. The syndrome is also often associated with a familial history. It is not uncommOn among Caucasians, but it appears to occur less frequently in the black race2 • Well-documented reports of the syndrome are also rare in Japan5 •
Case report An ll-year-old boy visited us on September 18, 1975 because of a swelling on the right side of his face (Fig. 1). External palpation showed an expansive swelling of the underlying bone. Intraorally, there were two swellings of bone on the buccal aspect of the right maxilla and mandible. The occlusion was an edge to edge bite. Other interesting findings were bifid uvula and a high arch of the palate. 0300-9785/79/010063-04$02.50/0
@
1979 Munksgaard, Copenhagen
64
NAKAJIMA, YOKOBAYASHI AND ONISHI
Fig. 1. Facial appearance of the patient at ini-
tial examination showing swelling on right side of face associated with broad nasal root and wide-set eyes.
three eytic lesions in the mandible (Fig. 4). He had a palate with a very high arch. According to his statement, his ,father, already deceased, had a large skull with frontal and parietal bossing.
Fig. 2. Panoramic radiograph showing many
cystic lesions in maxilla and mandible with impacted and displaced teeth.
Fig. 3. Postanterior radiograph of patient's
brother with large cystic lesion in mandible.
In consideration of the young age of the patient and multiple large cysts with impacted and displaced teeth, the lesions were treated by marsupialization. Walls of the maxillary cysts were excised on the buccal aspect to allow communication with the oral cavity in October 1975. The contents of the cysts were greasy, cheeselike materials as seen in kerato· cysts. The histologic diagnosis of each specimen was dentigerous keratocyst. The second operation for marsupialization of the mandibular cysts was performed in March 1976 by removing the buccal bone of the right canine region. The cysts were similar to those of the maxilla. Further marsupialization of the remaining cysts (cysts 3 and 6) and remarsupialization of the other cysts were performed twice in 1976. Obviously, all the cysts have shown a definite tendency to resolution after treatment, even though the untreated ones grew rapidly (Fig. 5). Orthodontic treatment is now being done to help eruption and alignment of the impacted and displaced teeth.
BASAL CELL NEVUS SYNDROME
65
Fig. 5. Panoramic radiograph of patient taken Fig. 4. Postanterior radiograph of patient's father showing three cystic lesions in mandible, calcification of falx cerebri and parietal bossing.
Discussion In the current case there were no carcinomatous sites to indicate the basal cell nevus syndrome, but support for it was offered by the characteristic appearance of the face and the bone survey which revealed multiple dentigerous cysts and skeletal defects such as the rudimentary sixth cervical rib, flaring of the third costal rib and bossing of the skull. The cysts of the jaws are not distinguishable from the keratocysts not related to the syndrome, except that they usually occur as multiple lesions. at an early stage of life 4 • Consequently, extensive displacement of many developing teeth is seen in the majority of cases. Another important aspect of the cysts is the high re-
in October 1976 (A) and in February 1977 (B) showing enlargement of untreated cysts (cysts 3 and 6) and resolution or reduction in size of cysts after treatment.
currence rate. Therefore, complete removal with care not to leave any component of the cyst wall has been recommended for its treatment. BROWNE! reported, however, that no difference was found in the ratio of recurrence following marsupialization, enucleation and primary closure, and enucleation and packing. As is often the case with other jaw cysts, simple marsupialization, though time consuming, may lead to gradual decrease of cystic lesions and spontaneous eruption of the impacted teeth. Orthodontic guidance is helpful in shortening the time required for their eruption and to obtain final proper ~clusion. A case was reported3 of the syndrome in which conservative treatment of two cysts in the maxilla and mandible followed by enucleation when they reached an operable size
66
NAKAJIMA, YOKOBAYASHI AND ONISHI
showed an excellent result. A conservative approach by marsupialization in our case had also produced a rapid reduction in the size of the radiolucent lesions accompanied by shifting of the positions of the impacted teeth toward the openings. Family history is often necessary to offer an additional support to establish a diagnosis of the syndrome. Cystic lesions were present in both the patient's brother and father. Examination of their faces and skeletal system also disclosed some of the characteristic abnormalities associated with the syndrome. Thus, we believe that the lesions found in the members of the family are due to the basal cell nevus syndrome with hereditary disorder even though the full symptoms are not present. Acknowledgments - We are grateful to Professor T. ISHIKI and other members of the Department of Oral Pathology, School of Dentistry, Niigata University, for histologic study of the specimens. Our thanks are also due to Professor N. TOKIWA of the First Department of Oral Surgery and Professor Y. OHASHI of the Second Department of Oral Surgery for their criticism.
References 1. BROWNE, R. M.: The odontogenic keratocyst. Clinical aspects. Br. Dent. I. 1970: 128: 225-231. 2. GIANSANTI, J. S. & BAKER, G. 0.: Nevoid
basal cell carcinoma syndrome in Negroes: report of five cases. I. Oral Surg. 1974: 32:
3.
138-144.
HICKORY,
1. E., GILliLAND, R. F.,
WADE,
W. M. & TAYLOR, C. G.: Conservative treatment of cysts of the jaws in nevoid basal cell carcinoma syndrome; report of case.
J. Oral Surg. 1975: 33: 693-697. 4. HoWELL, 1. B., UMAR BYRD, D., McCLENDON, J. L. & ANDERSON, D. E.: Identification and treatment of jaw cysts in the ne· void basal cell carcinoma syndrome. J. Oral Surg. 1967: 25: 129-138. 5. SUGAr, T., SHOJI, A. & YAMAMOTO, T.: Two cases of nevoid basal cell epithelioma syndrome in father and son suspected as multiple trichoepithelioma. lap. I. Derm. 1972: 82: 947.
Address: T. Nakajima First Department of Oral Surgery School of Dentistry, Niigata University 2·5274, Gakkocho, Niigata City Japan