Year : 2007 | Volume
: 25 | Issue : 3 | Page : 137--139
Jaw cyst-Basal cell nevus-Bifid rib syndrome: A case report
S Rai1, K Gauba2,
1 Department of Oral and Maxillofacial Surgery, Institute of Dental Sciences and Hospital, Punjab University, Chandigarh - 160 012, India
2 Department of Pedodontics, Institute of Dental Sciences and Hospital, Punjab University, Chandigarh - 160 012, India
Department of Oral and Maxillofacial Surgery, Institute of Dental Sciences and Hospital, Punjab University, Chandigarh -160 012
Jaw cyst-Basal cell nevus-Bifid rib syndrome or Gorlin-Goltz syndrome involves multiple organ system. The most common findings include multiple odontogenic keratocysts in the jaws and basal cell nevus on the skin that have an early age onset. These multiple odontogenic keratocysts warrant aggressive treatment at the earliest because of the damage and possible complications associated with them. Recurrence in these lesions is the most characteristic feature that has to be taken in consideration while explaining the prognosis to the patient. A case report of a child affected with Gorlin-Goltz syndrome diagnosed, treated and followed at this hospital is presented here.
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Rai S, Gauba K. Jaw cyst-Basal cell nevus-Bifid rib syndrome: A case report.J Indian Soc Pedod Prev Dent 2007;25:137-139
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Rai S, Gauba K. Jaw cyst-Basal cell nevus-Bifid rib syndrome: A case report. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2021 Dec 8 ];25:137-139
Available from: https://www.jisppd.com/text.asp?2007/25/3/137/36565
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. In this study, a similar case of Gorlin-Goltz syndrome was diagnosed, treated and followed at this hospital.
An 11-year-old male child attended the department of pediatric dentistry with a chief complaint of intraoral firm painless mass on the left side of the body of mandible. The duration of the swelling was 8 months and the growth was slow in nature. There was no discharge of any type and he never had any abnormal sensation on the affected side. On examination, the swelling was firm and nontender. It extended from the region of 32 to 37. The general examination revealed mild prognathism, frontal bossing and hypertelorism. [Figure 1]a, b. The gait was altered with limping and he also exhibited polydactyly [Figure 1]c. Further examination revealed that child was unable to bend both the thumbs.
The orthopantomogram showed multiple lytic lesions in the upper and lower jaws associated with unerupted permanent teeth displaced from their normal positions [Figure 2]a. The chest radiograph showed fourth and fifth bifid ribs on the right side [Figure 2]b. Coronal and axial computerized tomographic scan showed anteroposterior extension of the maxillary cysts and also the calcification of falx cerebri and tentorium cerebelli [Figure 3]. The spine X-ray showed spina bifida and the hand X-ray showed a shortened fourth metacarpal bilaterally. The clinical and radiological findings were confirmatory for Gorlin-Goltz syndrome. 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.
The enucleation of all the odontogenic keratocyst cysts followed by chemical cauterization with Carnoy's solution was planned for the patient. High recurrence rate of odontogenic keratocyst was explained to the parents. Under general anesthesia, two cysts from the mandible and two from the maxilla were enucleated. The contents of the cysts were found to be thick and cheesy. All the loose deciduous teeth and displaced permanent teeth were removed. Peripheral ostectomy was performed with a surgical bur followed by the application of Carnoy's solution for 5 min in each cavity followed by primary closure. The cystic lining was sent for histopathological examination and the report confirmed the diagnosis of parakeratinized odontogenic keratocyst (P-OKC). The removable type of prosthetic rehabilitation was done with removable partial denture and the patient has been on regular follow-up since then and has been asked to continue his visit once in every 6 months after 1 year.
Gorlin-Goltz syndrome often presents itself in an early age. Multiple basal cell carcinomas (usually on the face, beginning early in life) and multiple P-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.  (A) Cutaneous anomalies: Basal cell nevus, other benign dermal cysts and tumors, palmer 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.  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. ,
The management of these lesions 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.  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.
Chemical cauterization is a proved adjunctive technique in case of odontogenic keratocysts and is useful to prevent recurrence by fixing the daughter cysts or remnants of epithelial lining that are not removed during the enucleation procedure. Carnoy's solution is a phenolic compound with tissue fixative properties. Voorsmit has demonstrated that Carnoy's solution penetrates the bone to a depth of 1.54 mm following a 5-min application without any damage to the inferior alveolar nerve. 
A patient of Gorlin-Goltz syndrome can have multiple findings, as described in the literature. The most common 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 keratocysts 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.
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