The "Gorlin-Goltz syndrome" has also been called "nevoid basal-cell carcinoma syndrome" or more descriptively "multiple basal epithelioma, jaw cysts, and bifid rib
syndrome" [8, 9].
The structural abnormalities were bifid rib
in 44(6.7%) and fused type in 17(2.6%) cases.
Goltz, "Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib
. A syndrome," The New England journal of medicine, vol.
or bifurcated rib or forked rib is an anatomical variant where the sternal end of the rib is cleaved into two.
A bifid rib
, or sternum bifidum, is a congenital abnormality of the anterior chest wall, with the sternal end of the rib cleft into two.
A bifid rib
or sternum bifidum is a congenital abnormality of the anterior chest wall, with the sternal end of the rib cloven into two.
The syndrome consists of multiple BCCs, odontogenic keratocysts of jaws, palmer, or planter pits, anomalies of the ribs and spine (bifid ribs
, spina bifida) and calcification of the falx cerebri.
Other findings include basal cell carcinoma (young age at presentation and usually in non-sun-exposed areas), palmar and plantar pits, skeletal anomalies (e.g., bifid ribs
, calcified falx cerebri), hypertelorism, epidermal inclusion cysts, and even medulloblastoma or ovarian fibromas.
In our case the patient had absent & bifid ribs
, hemi & butterfly vertebrae, scoliosis of vertebral column & spina bifida, left lumbar hernia and tethered cord which is a disorder of primary neurulation.
These features include the development of multiple BCCs at a relatively young age, macrocephaly, frontal bossing, hypertelorism, bifid ribs
, palmar and plantar pitting, and bone cysts in the mandible.
He discovered Oliver had a kidney missing and also had bifid ribs
- a condition where the rib bones split instead of running parallel.
The infant exhibited the following manifestations of NBCCS: broad nasal bridge, bifid ribs
, palmar and plantar pits, hypertelorism, iris coloboma, macrocephaly, and cleft lip and palate.