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Pathophysiology of cleft lip muscle
S A Schendel1, R M Pearl, S J De'Armond
1Division of Plastic Surgery, Stanford University Medical School, Calif.
Plastic and Reconstructive Surgery
|May 1, 1989
Summary
Facial muscles in infants with cleft lip show signs of mitochondrial myopathy and delayed maturation, not normal development. This finding suggests a potential cause for cleft lip deformities related to impaired facial tissue development.
Area of Science:
- Craniofacial biology
- Muscle pathology
- Developmental biology
Background:
- Cleft lip and palate are common congenital conditions with largely unknown causes.
- Previous research has primarily focused on surgical correction rather than underlying etiology.
- The muscular basis of cleft lip deformities has not been thoroughly investigated.
Purpose of the Study:
- To investigate the histological and ultrastructural characteristics of muscles in infants with cleft lip.
- To determine if the muscles in cleft lip infants exhibit abnormalities.
- To explore the potential role of muscle development in the etiology of cleft lip.
Main Methods:
- Acquisition of 66 muscle biopsy specimens from infants undergoing primary cleft lip repair.
- Application of histochemical stains (modified Gomori trichrome) and histographic analysis.
- Utilizing electron microscopy to examine muscle fiber ultrastructure.
Main Results:
- Observed non-neurogenic muscle atrophy, most severe near the cleft, with disorganized muscle fibers.
- Identified "ragged red" fibers indicative of mitochondrial myopathy.
- Electron microscopy revealed abnormal mitochondrial accumulation, enlarged size, and dense cristae.
Conclusions:
- Muscles in cleft lip infants are abnormal, exhibiting signs of myopathy or delayed maturation.
- Abnormalities suggest a potential mitochondrial myopathy affecting facial mesenchymal development.
- Hypothesizes that impaired muscle development may lead to insufficient mesenchymal reinforcement during facial development, contributing to cleft lip formation.