Surgical Correction of the Craniofacial Anomalies in Apert Syndrome

AbstractApert syndrome (AS) is an autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and symmetrical syndactyly of hands and feet. Ten percent children with AS can have associated congenital cardiac anomalies. Association of complex cyanotic heart disease with craniosynostosis is very rare. So far, only one case has been reported in the literature. The craniosynostosis corrective surgery is associated with the risk of massive bleeding or venous air embolism, which can cause paradoxical air embolism and precipitate cyanotic spell, which makes the anesthesia more challenging. In this report, we present the anesthetic challenges of an 8-month-old infant with AS and tetralogy of Fallot for craniosynostosis correction.

Download Full-text

Surgical Correction of the Craniofacial Dysmorphology of Apert Syndrome

Clinics in Plastic Surgery ◽

10.1016/s0094-1298(20)30821-x ◽

1991 ◽

Vol 18 (2) ◽

pp. 251-275 ◽

Cited By ~ 11

Author(s):

Jeffrey L. Marsh ◽

Miroslav Galic ◽

Michael W. Vannier

Keyword(s):

Surgical Correction ◽

Apert Syndrome ◽

Craniofacial Dysmorphology

Download Full-text

The Kleeblattschädel Anomaly in Apert Syndrome: Intracranial Anatomy, Surgical Correction, and Subsequent Cranial Vault Development

Plastic & Reconstructive Surgery ◽

10.1097/00006534-199712000-00024 ◽

1997 ◽

Vol 100 (7) ◽

pp. 1796-1802 ◽

Cited By ~ 9

Author(s):

Arun K. Gosain ◽

David C. Hemmy

Keyword(s):

Surgical Correction ◽

Cranial Vault ◽

Apert Syndrome

Download Full-text

Distraction Osteogenesis in the Treatment of Craniofacial Anomalies: Applications and Outcomes

Perspectives of the ASHA Special Interest Groups ◽

10.1044/2020_persp-20-00055 ◽

2020 ◽

Vol 5 (6) ◽

pp. 1469-1481 ◽

Cited By ~ 1

Author(s):

Joseph A. Napoli ◽

Carrie E. Zimmerman ◽

Linda D. Vallino

Keyword(s):

Distraction Osteogenesis ◽

Bone Growth ◽

Upper Airway ◽

Craniofacial Anomalies ◽

Craniofacial Skeleton ◽

Facial Skeleton ◽

Psychosocial Impairment ◽

And Function ◽

Correct Structure

Purpose Craniofacial anomalies (CFA) often result in growth abnormalities of the facial skeleton adversely affecting function and appearance. The functional problems caused by the structural anomalies include upper airway obstruction, speech abnormalities, feeding difficulty, hearing deficits, dental/occlusal defects, and cognitive and psychosocial impairment. Managing disorders of the craniofacial skeleton has been improved by the technique known as distraction osteogenesis (DO). In DO, new bone growth is stimulated allowing bones to be lengthened without need for bone graft. The purpose of this clinical focus article is to describe the technique and clinical applications and outcomes of DO in CFA. Conclusion Distraction can be applied to various regions of the craniofacial skeleton to correct structure and function. The benefits of this procedure include improved airway, feeding, occlusion, speech, and appearance, resulting in a better quality of life for patients with CFA.

Download Full-text