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Delayed Eruption In Cleidocranial Dysplasia.

Sanaa Ahmed¹, Maria Naz Shaheen¹, Faisal Hameed¹

¹Department of Oral Medicine, Sindh Institute of Oral Health Sciences, Karachi, Pakistan.

Journal of Ayub Medical College, Abbottabad : JAMC

|August 24, 2020

Summary

This summary is machine-generated.

Cleidocranial dysplasia, a rare genetic bone disorder, affects skull and teeth development. This report details two sibling cases, highlighting familial inheritance patterns of this condition.

Keywords:

Cleidocranial Dysplasia; Supernumerary Tooth Pseudarthrosis of Clavicle; Congenital

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Area of Science:

Genetics
Orthopedics
Developmental Biology

Background:

Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal disorder.
It primarily impacts bones formed through intramembranous and endochondral ossification.
Clinical manifestations include characteristic facial and skeletal abnormalities.

Observation:

The study presents two sibling cases of cleidocranial dysplasia.
This highlights a familial occurrence of the disorder.
CCD is typically inherited in an autosomal dominant pattern, with sporadic cases being less common.

Findings:

Cleidocranial dysplasia presents with a distinct set of clinical features.
These include a brachycephalic skull, frontal bossing, depressed nasal bridge, hypertelorism, delayed fontanelle closure, delayed permanent tooth eruption, absent clavicles, and a wide pelvis.
The condition affects both intramembranous and endochondral ossification processes.

Implications:

Understanding familial patterns is crucial for genetic counseling in cleidocranial dysplasia.
Early diagnosis and management can address dental and skeletal issues.
Further research into the genetic basis and ossification defects can improve treatment strategies.