Examine Your Orofacial Cleft Patients for Gorlin-Goltz Syndrome

Abstract

The Gorlin-Goltz syndrome is characterized by tour primary symptoms: multiple nevold basal cell epitheliomas that usually undergo malignant transformation; jaw keratocysts that show constant growth; skeletal anomalies; and intracranial calcifications. A myriad of additional findings may also be noted. Among the most frequent are: palmar and plantar pits, a characteristic flattened facles and broad nasal root, frontal and parietal bossing, mandibular prognathia, hypertelorism, strabismus, dystrophia of the canthi, and clefts of the lip, alveolus, and/or palate. In this study, we review the literature and our 25 cases of Gorlin-Goltz syndrome patients, questioning their incidence of cleft formations (8.5%) as compared to the general population (0.1%). It is our contention that all patients who present with an orofacial cleft warrant deeper investigation as to the presence of additional signs indicative of Gorlin-Goltz syndrome. The nevi turn malignant with time, and thus, early diagnosis, follow-up, and treatment are imperative.

Keywords

cleft formations epitheliomas Gorlin-Goltz syndrome nevoid basal cell carcinomas

Get full access to this article

View all access options for this article.

References

Anderson

D.E.

McClendon

J.L.

Howell

JB.

Genetics and skin tumors with special reference to basal cell nevi. In: Anderson Hospital and Tumor Institute. Tumors of the skin. Chicago: Yearbook Medical Publishers, 1964: 92–127.

Becker

M.H.

Kopf

A.W.

Lande

Basal cell nevus syndrome, its roentgenologic significance.

Am J Roentgenol 1967; 99: 817–825.

Becker

Lambrecht

J.T.

Sterry

Extreme Verläufe beim Gorlin-Goltz-Syndrom und Vorschläge zur Vorsorge.

Dtsch Zahnarztl Z 1985; 40: 531–532.

Binkley

G.W.

Johnson

H.H.

Epithelioma adenoides cysticum: basal cell nevi, agenesis of corpus callosum and dental cysts.

Arch Dermatol Syph 1951; 63: 73–84.

Block

J.B.

Clendenning WE. Parathyroid hormone hyporesponsiveness in patients with basal-cell nevi and bone defects.

N Engl J Med 1963; 268: 1157–1162.

Bopp

Bakos L, Erling H. Sindrome nevo baso cellular.

An Bras Dermatol 1968; 43: 131–162.

Cesteleyn

Wierich

Das Gorlin-Goltz Syndrom.

Z Stomatol 1984; 81: 149–154.

Clarkson

Wilson

Two cases of basal cell congenital naevus.

Proc R Soc Med 1960; 53: 295–296.

Clendenning

W.E.

Block

J.B.

Radde

I.C.

Basal cell nevus syndrome.

Arch Dermatol 1964; 90: 38–53.

10.

Compton

J.G.

Goldstein

A.M.

Turner

. Fine mapping of the locus for nevoid basal cell carcinoma syndrome on chromosome 9q. J Invest Dermatol 1994; 103: 178–181.

11.

Cotton

S.T.

Super ST, Sunderraj M, Chaudhry A. Multiple nevoid basal cell carcinoma syndrome.

J Oral Med 1982; 37: 69–73.

12.

de Jong

Bistervels

Cosgrove

. Medullated nerve fibers. A sign of multiple basal cell nevi (Gorlin's) syndrome. Arch Ophthalmol 1985: 103; 1833–1836.

13.

de Kersaint-Gilly

Hofmann

Delaire

. A neurological study of the naevoid basal cell carcinoma syndrome. Eighteen cases. J Neuroradiol 1985; 12: 200–211.

14.

Delaire

Billet

Traitement chirurgical conservateur des kystes öpider-moides de la naevomatose baso-cellulaire.

Rev Stomatol Chir Maxillofac 1980; 81: 18–28.

15.

de León

Zakri

Donner

Karmazin

Cerebral rhinocele, hydrocephalus, and cleft lip and palate in infants with cardiac fibroma.

J Neurol Sci 1990; 99: 27–36.

16.

van Dijk

Sandbrink

J.F.H.

Basal cell naevus syndrome.

Dermatologica 1967; 134: 101–106.

17.

van Dijk

. Nerring H. The association of cleft lip and palate with basal cell nevus syndrome. Oral Surg 1980; 50: 214–216.

18.

Evans

D.R.G.

Ladusans EJ, Rimmer S, et al. Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

J Med Genel 1993; 30: 460–464.

19.

Fidler

R.S.

Kissane

R.W.

Koons

R.A.

Primary fibrosarcoma of the heart.

Am Heart J 1937; 13: 736–740.

20.

Fitzpatrick

P.J.

Thompson

G.A.

Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.

Can Med Assoc J 1982; 127: 465–470.

21.

Formas

Naevobasaliom, Z Haut Geschlechtskr 1967; 42: 131–140.

22.

Forssell

The primordial cyst: a clinical and radiographic study.

Proc Finn Dent Soc 1980; 76: 129–174.

23.

Gesterkamp

Lippen-Kiefer-Gaumenspalten bei Patienten mit Gorlin-Goltz-Syndrom. Dental medicine dissertation, Christian-Albrechts-University, Kiel. Germany, 1993.

24.

Gorlin

R.J.

Nevoid basal-cell carcinoma syndrome.

Medicine 1987; 66: 98–113.

25.

Gorlin

R.J.

Nevoid basal cell carcinoma syndrome.

Dermalol Clin 1995; 13: 113–125.

26.

Gorlin

R.J.

Goltz

R.W.

Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib: a syndrome.

N Engl J Med 1960; 18: 908–912.

27.

Gorlin

R.J.

Sedano

H.O.

Multiple nevoid basal cell carcinoma syndrome. In: Vinken

P.J.

Bruyn

G.W.

, eds. Handbook of clinical neurology. Vol. 14: The phakomatoses. Amsterdam: North Holland Publishing, 1972: 455–473.

28.

Gorlin

R.J.

Vickers

R.A.

Kelln

Williamson

J.J.

The multiple basal-cellnevi syndrome.

Cancer 1965; 8: 89–104.

29.

Gundlach

K.K.H.

Kiehn

Multiple basal cell carcinomas and kerato-cysts–the Gorlin and Goltz syndrome.

J Maxillofac Surg 1979; 7: 299–307.

30.

Herzberg

J.J.

Wiskemann

Die fünfte Phakomatose, Basalzellnaevus mit familiärer Belastung und Medulloblastom.

Dermatologica 1963. 126: 106–123.

31.

Howell

J.B.

Caro

M.R.

The basal-cell nevus.

Arch Dermatol 1959; 79: 67–80.

32.

Jackson

Gardere

Nevoid basal cell carcinoma syndrome.

Can Med Assoc J 1971; 105: 850–862.

33.

Jarisch

, Zur Lehre von den Hautgeschwülsten. Arch Dermatol Syph 1894; 28: 163–222.

34.

Jones

K.L.

Wolf

P.L.

Jensen

. The Gorlin syndrome, a genetically determined disorder associated with cardiac tumor. Am Heart J 1986; 111: 1013–1015.

35.

Kamiya

Narita

Yamamoto T, et al. Familial odontogenic keratocysts.

Int J Oral Surg 1985; 14: 73–80.

36.

Kirsch

Pathogenetische Beziehungen zwischen Kieferzysten und Hautveränderungen unter besonderer Berücksichtigung der Hautkarzinomatose.

Schweiz Monatsschr Zahnmed 1956; 66: 687–701.

37.

Koutnik

A.W.

Kolodny

S.C.

Hooker

S.P.

Roche

W.C.

Multiple nevoid basal epithelioma, cysts of the jaw, and bifid rib syndrome. Report of a case.

J Oral Surg 1975; 33: 686–689.

38.

Lahti

Rintala

Salo

Polycystic jaws in patients with cleft lip and palate.

Ann Chir Gynaecol 1973; 62: 161–165.

39.

Lambrecht

J.T.

Sojka-Raytscheff

Brix

Computertomographische Befunde des Hirmschädels bei Patienten mit Gorlin-Golt/Syndrom.

Dtsch Zahnarztl Z 1985; 40: 529–530.

40.

Lindeberg

Halaburt

Larsen

P.O.

The nevoid basal cell carcinoma syndrome.

J Maxillofac Surg 1983; 10: 246–249.

41.

McEvoy

B.F.

, Multiple nevoid basal cell carcinoma syndrome: radiological manifestations. Br J Radiol 1969; 42: 24–28.

42.

Nakajima

Yokobayashi

Onishi

Basal cell nevus syndrome. A case report.

Int J Oral Surg 1979; 8: 63–66.

43.

Nisbet

T.W.

Multiple basal cell epitheliomas originating from congenital pigmented basal cell nevi.

Arch Dermatol Syph 1943; 47: 373–381.

44.

Nomland

Multiple basal cell epitheliomas originating from congenital pigmented basal cell nevi.

Arch Dermatol Syph 1932; 25: 1002–1008.

45.

Olson

R.A.J.

Stroncek

G.G.

Scully

J.R.

Govin L. Nevoid basal cell carcinoma syndrome: review of the literature and report of a case.

J Oral Surg 1981; 39: 308–312.

46.

Planté

Beauvais

Djian

Marie

M-P

. Un nouveau cas de naevomatose baso-cellulaire. Rev Stomatol 1978; 79: 411–418.

47.

Pollard

J.J.

New

P.F.J.

Hereditary cutaneomandibular polyoncosis.

Radiology 1964; 82: 840–849.

48.

Pollitzer

Eine eigentümliche Karzinose der Haut.

Arch Dermatol Syph 1905; 76: 323–345.

49.

Pritchard

L.J.

Delfino

J.J.

Ivey

D.M.

. Variable expressivity of the multiple nevoid basal cell carcinoma syndrome. J Oral Maxillofac Surg 1982; 40: 261–269.

50.

Ramsden

R.T.

Barrett

Gorlin's syndrome.

J Laryngol Otol 1978; 9: 615–621.

51.

Rater

C.J.

Sflke

A.C.

van Epps

E.F.

Basal cell nevus syndrome.

Am J Roentgenol 1968; 103: 589–594.

52.

Rayner

C.R.W.

Towers

J.F.

Wilson

J.S.P.

What is Gorlin's syndrome?

The diagnosis and management of basal cell naevus syndrome based on a study of thirty-seven patients. Br J Plast Surg 1977; 30: 62–67.

53.

Ruprecht

Austermann

K-H

Umstadt

Cleft lip and palate, seldom seen features of the Gorlin-Goltz syndrome.

Dentomaxillofac Radiol 1987; 16: 99–103.

54.

Shanley

Ratcliffe

Hockey

et al. Nevoid basal cell carcinoma syndrome: review of 118 affected individuals.

Am J Med Genet 1994; 50: 282–290.

55.

Shroff

F.R.

Smith

J.L.

Multiple cysts of the jaw, basal cell carcinomas and bifid ribs.

N Z Dent J 1962; 62: 37–38.

56.

Soekarman

Fryns

J.P.

Casaer

Van Den Berghe

Increased head circumference and facial cleft as presenting signs of the nevoid basal-cell carcinoma syndrome.

Genet Couns 1991; 2: 157–162.

57.

Southwick

G.J.

Schwartz

R.A.

The basal cell nevus syndrome.

Cancer 1979; 44: 2294–2305.

58.

Stahle

I.O.

Multiple basal cell carcinotnata: Gorlin's syndrome.

Australas J Dermatol 1969; 10: 197–198.

59.

Stoelinga

P.J.W.

Cohen

M.M.

Morgan

A.F.

The origin of keratocysts in the basal cell nevus syndrome.

J Oral Surg 1975; 33: 659–663.

60.

Storck

Schwarz

Cavegn

. Naevoid Basaliome mit Kieferzysten (Ward-Syndrom). Dermatologica 1966; 133: 145–150.

61.

Straith

F.E.

Hereditary epidermoid cysts of the jaws.

Am J Orthod 1939; 25: 673–691.

62.

Summerhill

Hutton

J.G.

Multiple benign cystic epithelioma.

Arch Dermatol Syph 1932; 26: 854–864.

63.

Summerly

Hale AJ. Basal cell naevus syndrome.

Trans St John's Hosp Dermatol Soc 1965; 51: 77–79.

64.

Taichfr

Shteyer

The basal cell nevus syndrome associated with cleft lip and palate. Report of case.

J Oral Surg 1978; 36: 799–802.

65.

Tasanen

Lamberg

M.A.

Nordling

Skeletal anomalies and keratocysts in the basal cell naevus syndrome, Int J Oral Surg 1975; 4: 225–235.

66.

Taylor

W.B.

Anderson DE, Howell JB, Thurston CS. The nevoid basal cell carcinoma syndrome.

Arch Dermatol 1968; 98: 612–614.

67.

Tobias

C.S.

Zum Basalzellnaevus-Kiefercysten-Syndrom (Ward-Syndrom) mit familiärem Auftreten.

Schweiz Med Wochenschr 1967; 97: 949–953.

68.

Totten

J.R.

Multiple basal cell naevi syndrome; management of the young patient.

Br J Oral Surg 1980; 17: 147–156.

69.

Veltmann

Adari

Die fünfte Phakomatose.

Z Haut Geschtechtskr 1971; 46: 221–240.

70.

Watt

Bragg

F.E.

, Additional observations on the nevoid basal cell epithelioma syndrome, a case report. 1 Maine Med Assoc 1973; 64: 268–269.

71.

Wiedemann

H-R

Kunze

Dibbern

Atlas der klinischen Syndrome. Stuttgart, New York: Schatlauer, 1989.

72.

Zenk

Schumann

Schelhorn

Mueller

E-I

. Hauiveraendemngen und Kieferzysten - ein Beitrag zum Basalzell-Naevus-Syndrom. Zahn Mund Kieferheilkd 1984; 72: 559–565.