CASE REPORT

Gorlin Goltz Syndrome

Chandra Shekar. L1, Sathish. R2, Beena. S3, Sachin Ganeshan1

1 Department of Oral medicine,

Sri Siddhartha Dental College
ABSTRACT
Agalkote, Tumkur 572 107
Karnataka, India Jaw cyst-Basal cell nevus-Bifid rib syndrome or Gorlin-Goltz syndrome involves multiple organ
2 Department of oral surgery, system. The most common findings include multiple odontogenic keratocysts in the jaws and basal
Sri Siddhartha Cental College, cell nevus on the skin that have an early age onset. These multiple odontogenic keratocysts warrant
Agalkote, Tumkur 572 107
Karnataka, India aggressive treatment at the earliest because of the damage and possible complications associated
3 Department of Endodontics, with them. Recurrence in these lesions is the most characteristic feature that has to be taken in
Sri Siddhartha Cental College consideration while explaining the prognosis to the patient. A case report of an adolescent affected
Agalkote, Tumkur 572 107 with Gorlin-Goltz syndrome is presented here.
Karnataka, India
Keywords: Gorlin-Goltz syndrome, Odontogenic keratocyst, Bifidrib.
Journal of Dental Sciences and Research
Vol. 2, Issue 2, Pages 1-5

INTRODUCTION normal except undergone extraction of 24, 25 due to

Jaw cyst-Basal cell nevus-Bifid rib syndrome or Gorlin-
Goltz syndrome is a hereditary condition and it is
transmitted as an autosomal dominant trait with high or
almost 100% penetrance and varying expressivity. In
1960, Gorlin and Goltz studied the main features of this
syndrome. This syndrome affects multiple organ
systems, which include skeletal, skin, eye, reproductive,
and neural system, although all the features are rarely
observed in a single patient. Often these patients first
visit a dental hospital with the chief complaint of jaw
swelling where the diagnosis of this syndrome is
generally made. Hence, a dental clinician should always
be open to the possibility of encountering multiple lytic
lesions of the jaws. We report a case with similar features.
CASE REPORT
A 14-year-old female patient came to the department of
oral medicine & radiology with a chief complaint of
intraoral firm painless mass on the upper left back tooth
region. The duration of the swelling was 8 months and the
growth was slow in nature. There was no discharge of any
type and she never had any abnormal sensation on the
affected side. Her medical and past dental history was
Address for correspondence:
Dr Beena. S
E-mail: beenahari.ganesh@gmail.com
Access this article online
Website: http://www.ssdctumkur.org/jdsr.php.
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dental caries, On examination, wide nasal bridge and
mild prognathism was observed extra orally and intra
orally the swelling was firm and nontender, it extended
from the region of 25 to 27. (fig-1,2,3)and missing 13was
seen.
The orthopantomogram showed multiple lytic lesions in
the upper and lower jaws associated with unerupted
permanent teeth (fig-4). The chest radiograph showed
second and third bifid ribs on the right side (fig-5).intra
oral maxillary occlusal radiograph showing cortical
expansion, and radiolucent lesion on the right side (fig-
6). Based on the clinical and radiological findings, a
provisional diagnosis of Gorlin-Goltz syndrome was
considered. No skin lesions in the form of basal cell
nevus, palmer or plantar pits or keratosis were present.
The parents and siblings of the patient were also
examined clinically and radiographically; however,
none of them showed any characteristics of this
condition.
DISCUSSION
Gorlin-Goltz syndrome often presents itself in an early
age. Multiple basal cell carcinomas (usually on the face,
beginning early in life) and multiple odontogenic
kerotocysts(OKC) (also beginning early in life) are the
main hallmarks of this syndrome; however, there are
other manifestations that are grouped into the following
five categories[1]. (A) Cutaneous anomalies: Basal cell
nevus, other benign dermal cysts and tumors, palmer
 Vol. 2, Issue 2, September 2011

Fig-1 Broad Nasal Bridge Fig-2 Proganthism

Fig-3 Intra Oral Swelling Fig-4 OPG showing Multiple Radiolucent Lesions in Maxilla
and Mandible

Fig-5 Chest Radiograph showing Bifid Rib Fig-6 Maxillary Occlusal Radiographs showing Radiolucent
Lesions
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Journal of Dental Sciences and Research

pitting, palmer and planter keratosis and dermal
calcinosis. (B) Dental and osseous anomalies: multiple
odontogenic keratocyst, mild mandibular prognathism,
frontal and temporoparietal bossing, kyphoscoliosis or
other vertebral defects, bifurcated ribs, spina bifida and
brachymetacarpalism. (C) Ophthalmic anomalies:
hypertelorism, wide nasal bridge, dystopia canthorum,
congenital blindness and internal strabismus. (D)
Neurological anomalies: mental retardation, dural
calcification, bridging of sella, agenesis of corpus
callosum, congenital hydrocephalus, occurrence of
medulloblastoma. (E) Sexual anomalies:
hypogonadism, ovarian tumor-like fibrosarcoma.
Less than 10% of patients with multiple OKCs have other
manifestations of this syndrome; however, it has been
suggested that multiple OKCs alone maybe the
confirmatory of the syndrome. Two types of keratocysts
have been distinguished based on differences in the
histology and behavior: the more common parakeratotic
odontogenic keratocyst (P-OKC) and the less common
orthokeratotic odontogenic keratocyst (O-OKC). First,
the P-OKC has a more aggressive growth potential and a
higher recurrence rate than the O-OKC and other
odontogenic cysts. Second, in a minority of patients
(particularly, young patients with multiple cysts), the P-
OKC is a part of the Jaw cyst-Basal cell nevus-Bifid rib
syndrome. Although benign, the recurrence rate of P-
OKC is high, ranging from 12% to 62.5% and multiple
recurrences are not unusual[2]. Some investigators have
suggested that P-OKC should be regarded as a benign
neoplasm rather than a nonneoplastic cyst. Ameloblastic
transformation or malignant changes are other rarely
encountered complications, which are described in the
literature [3,4].
The management of thes e les ions varies in
aggressiveness from simple enucleation or curettage to
ostectomy with curettage of the adjacent bone. In
addition, the term "peripheral ostectomy" has been used
to describe an adjunctive surgical procedure following
enucleation or curettage, in which the osseous walls of
the defect are abraded with coarse surgical burs in order
to ensure that any residual peripheral neoplastic tissue is
removed[5]. The obvious advantage of a conservative
surgical choice is the preservation of the adjacent bone,
soft tissue and dental structures. Reduced morbidity as
well as a shorter hospital stay generally follows this
course of therapy. Some large, destructive cases require
segmental resection of the involved jawbone with
immediate or delayed reconstruction. All the cases
warrant periodic clinical and radiological follow up to
check any early signs of recurrence.
findings of basal cell nevus on the skin and multiple
Odontogenic keratocyst in the jaws show early onset and
have to be treated at the earliest with the possible
complications explained to the patient. Most of the times,
the patients visit a dental hospital with a chief complaint
of jaw swelling. Hence, the responsibility of proper
diagnosis and further treatment plan lies on the dental
team. Various treatment modalities have been proposed
for Odontogenic keratocyst considering its recurrent
nature with variable results. There should be a periodic
follow-up at regular intervals of 6 months till 5 years
followed by once annually for the entire life.
REFERENCES
1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell
epithelioma, jaw cysts and bifid rib. a syndrome. N
Engl J Med 1960;262:908
2. Oikarinen VJ. Keratocyst recurrences at intervals of
more than 10 years: Case reports. Br J Oral
Maxillofac Surg 1990;28:47
3. Brannon RB. The odontogenic keratocyst: A
clinicopathological study of 312 cases. Part II.
Histological features. Oral Surg Oral Med Oral
Pathol 1977;43:223
4. Foley WL, Terry BC, Jacoway JR. Malignant
transformation of an odontogenic keratocyst. J Oral
Maxillofac Surg 1991;49:768
5. Zachariades N, Papanicolaou S, Triantafyllou D.
Odontogenic keratocysts: Review of the literature
and report of 16 cases. J Oral Maxillofac Surg
1985;43:117
6. Voorsmit RA, Stollinga PT, Van Hallst VJ. The
management of keratocysts. J Maxillofac Surg
1981;9:228

A patient of Gorlin-Goltz syndrome can have multiple

findings, as described in the literature. The most common

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