Bifid Ribs Research Articles

Multiple odontogenic keratocysts associated with Gorlin-Goltz syndrome

Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogenic keratocysts were causing disfigurement of the lower jaw as well as displacement and malocclusion of the lower teeth.

Multiple nevoid basal cell carcinoma syndrome: radiological manifestations

The nevoid basal cell carcinoma syndrome consists of multiple basal cell carcinoma of the skin appearing at an early age, multiple skeletal and soft tissue abnormalities, typical facies, and frequently endocrine abnormalities. It is thought that it is inherited as an autosomal dominant and it seems likely that the 150 cases in the literature represent only a part of the total number of affected individuals. Seventeen patients with the nevoid basal cell carcinoma were examined radiologically. A systematic radiological survey of a series of this kind has not previously been made. The commonest abnormalities discovered radiologically included: calcification of the falx, rib deformities (bifid rib, splayed rib or pseudarthrosis of ribs), scoliosis, spina bifida, cysts of the jaws and bossing of the skull. Knowledge of the radiological manifestations of the syndrome may allow the radiologist to diagnose this entity.

Gorlin's syndrome. A radiological and cytogenetic study of nine cases.

Abstract Gorlin's syndrome is a condition, often familial, showing multiple basal cell epitheliomata, jaw cysts and a variety of other abnormalities such as bifid ribs, scoliosis, calcification of the falx and mesenteric cysts. Nine cases are presented with their radiological and cytogenetic findings. Two of these cases possessed a marker chromosome in their karyotypes; the remainder were cytogenetically normal. It is thought that this condition may be commoner than formerly supposed.

Multiple cysts of the jaw, basal cell nævi, and bifid ribs

Multiple cysts of the jaw, basal cell nævi, and bifid ribs

Multiple cysts of the jaw, basal-cell epithelioma of the skin, and bifid ribs

Two cases, involving a brother and sister suffering from an uncommon syndrome that involved multiple cysts of the jaw, basal-cell trichoepithelioma of the skin, and bifid ribs, have been presented.

Multiple primordial cysts associated with bifid rib and ocular defects

Multiple primordial cysts associated with bifid rib and ocular defects

Multiple Nevoid Basal Cell Epitheliomas, Jaw Cysts, and Skeletal Defects

A syndrome characterized by multiple nevoid basal cell epitheliomas of the skin, cysts of the jaw, and congenital skeletal deformities has been studied and a series of new cases reported. Manifestations of the syndrome occur early in life, often in childhood, and there are definite familial or hereditary tendencies but no sex predilection. Some of the multiple basal cell tumors, which show areas of aggressive change and ulceration and demonstrate a wide range of histopathological basal cell patterns, could not be distinguished histologically from basal cell epitheliomas in patients without the syndrome. The cysts of the jaws are grossly destructive of bone and have not been found in any bones except the maxilla and mandible. The skeletal deformities include bifid and anomalous ribs, dorsal scoliosis, and frontal bosses.

Chromosomal study in patients with cysts of the jaw, multiple nevoid basal cell carcinomata and bifid rib syndrome.

A chromosomal abnormality in a patient with cysts of the jaw, recurrent nevoid basal cell carcinomata and bifid ribs is described. The patient presented a normal female complement, except for the unusual length of one arm of one of the chromosomes No. 1. Her mother, two sisters, and a male child of one of these two sisters presented a similar chromosomal anomaly with no expression of the syndrome. Several possible explanations are discussed.