The role of the orthodontist in the diagnosis of Gorlin’s syndrome

doi:10.1016/S0889-5406(99)70321-5

American Journal of Orthodontics and Dentofacial Orthopedics

Volume 115, Issue 1, January 1999, Pages 89-98

https://doi.org/10.1016/S0889-5406(99)70321-5 Get rights and content

Abstract

Gorlin’s syndrome is a relatively rare generalized disorder. Its diagnosis in childhood is usually through oral abnormalities. Some of the most frequent clinical features of this syndrome are discovered through radiographs commonly used in orthodontia. Thus, the orthodontist may be able to contribute to its diagnosis. The article shows three clinical cases that illustrate the role that the orthodontist may play in diagnosis of this syndrome. (Am J Orthod Dentofacial Orthop 1999;115:89-98)

Section snippets

Skin Abnormalities

•
Basal cell carcinomas appear as papules whose color is between pink and dark gray and are 1 to 10 mm in diameter. They are more frequent after the second decade of life and affect (in decreasing order) face, neck, back, thorax, abdomen, and limbs.
•
Pits of palms and soles
•
Milia
•
Cutaneous epidermoid cysts, especially on the extremities

Skeletal Abnormalities

•
Widened or forked ribs
•
Fused or missing ribs
•
Rudimentary cervical ribs
•
Vertebral fusions
•
Bifid spine
•
Dysplastic scapula
•
Pectus excavatum
•
Hand abnormalities (polydactyly,

ORAL ABNORMALITIES

Oral abnormalities, which in childhood and adolescence are of fundamental importance (these are the most important signs for diagnosis), have been described extensively in the medical literature.9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28 It can be summed up in this way.

DENTAL ABNORMALITIES

The most important of dental abnormalities are:

•
Number anomalies
•
Position anomalies (ectopic eruption and frequent diastemas)
•
Form anomalies

Teeth of these patients resemble lobodontia or carnivorous teeth with sharp canines and premolars.

Among oral abnormalities, cleft lip and palate are more frequent in patients with Gorlin’s syndrome than in the general population. The incidence in patients with Gorlin’s syndrome is between 4% and 8%.4, 29, 30, 31

We describe three clinical cases (two children

CLINICAL CASE 1

An 11-year-old child was admitted to surgery for orthodontic treatment. The frontal view shows some cranial enlargement with temporoparietal bossing and decreased facial height (Fig 1).

. Frontal view.

In a profile view we observe a Class III profile that was corroborated by later cephalometry. Prognathic jaw and hypoplastic maxilla were found (Figs 2, 3, 4, and 5).

. Profile view.

. Steiner’s cephalometry.

. Ricketts cephalometry.

. Witts appraisal.

In the intraoral views, we noted agenesis of upper

CLINICAL CASE 3

An 8-year-old child came to surgery because of anterior open bite (Fig 22).

. Anterior open bite.

He presented a somewhat concave profile (Fig 23).

. Profile view.

Cephalometry confirmed a Class III relationship (Figs 24 and 25).

. Ricketts cephalometry.

. Witts appraisal

A panoramic radiograph revealed agenesis of some premolars and a mandibular cyst (Fig 26).

. Cyst and agenesis.

A wrist radiograph shows agenesis of a phalanx of the fourth finger (Fig 27).

. Phalanx agenesis.

Because of previous experience

DISCUSSION

Gorlin’s syndrome has autosomal dominant inheritance. There is high penetrance and variable expressivity.³² In Case 1, transmission is clear; the mother of the patient (case 2) exhibited multiple maxillary and mandibular cysts. She lost all of her teeth. There was no diagnosis made at that time.

In Case 3, there was no family history, but one cannot rule out that some members of the family may have slight manifestations.

In the cases we studied, we found signs through the radiographs used in

CONCLUSIONS

Gorlin’s syndrome is a process in which orthodontic treatment is almost impossible because of the continuous recurrences of maxillary and mandibular cysts that cause multiple loss of teeth. Orthodontists can contribute to diagnose this condition because the signs of the syndrome appear in the films they use daily. Moreover, these signs are the most important to diagnosis during childhood and adolescence.

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The role of the orthodontist in the diagnosis of Gorlin’s syndrome☆,☆☆

Abstract

Section snippets

Skin Abnormalities

Skeletal Abnormalities

ORAL ABNORMALITIES

DENTAL ABNORMALITIES

CLINICAL CASE 1

CLINICAL CASE 3

DISCUSSION

CONCLUSIONS

Br J Oral Surg

J Am Dent Assoc

J Oral Maxillofac Surg

J Am Acad Dermatol

Oral Surg

Am J Orthodont

Int J Oral Surg

Birth defects encyclopedia. Center for birth defects information services

Multiple nevoid basal cell epithelioma, jaw cysts and bifid rib syndrome

N Engl J Med

Síndromes de la cabeza y del cuello. Ediciones Toray

Nevoid basal cell carcinoma syndrome

Medicine

Recognizable patterns of human malformation

Syndrome basocellulaire

Dermatologica

The basal cell nevus syndrome

Br J Hosp Med

Disorders involving medicine and dentistry: the basal cell nevus syndrome

Quintessence Int

Bilateral cysts of the jaw occurring with multiple skin lesions

J Am Dent Assoc

A propósito de un caso de Síndrome de Gorlin

Advances en Odontoestomatología