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Two cases of single-stage lip and nostril reconstruction in holoprosencephaly
N Morita1, Y Morita, Y Taenaka
1Department of Oral and Maxillofacial Surgery, Wakayama Medical University, Wakayama, Japan. n-morita@wakayama-med.ac.jp
Insights
Holoprosencephaly, a rare birth defect, involves midline facial clefts. This study presents simultaneous columella reconstruction with cheiloplasty for improved aesthetic outcomes in affected infants.
Area of Science:
- Craniofacial surgery
- Pediatric surgery
- Developmental biology
Background:
- Holoprosencephaly is a rare congenital disorder characterized by incomplete separation of the forebrain.
- It affects facial midline development, often resulting in cleft lip and palate.
- Historically, severe cases had poor prognosis, limiting surgical intervention.
Observation:
- Improved perinatal care has increased survival rates for infants with holoprosencephaly.
- Parents frequently desire complete lip repair, including columella reconstruction, for aesthetic reasons.
- Two cases of simultaneous columella reconstruction during cheiloplasty are presented.
Findings:
- Simultaneous columella reconstruction with cheiloplasty is a feasible surgical approach.
- This combined procedure addresses both functional and aesthetic concerns in holoprosencephaly.
- The technique aims to enhance the overall cosmetic result for affected children.
Implications:
- This surgical approach may improve patient and family satisfaction.
- It highlights the evolving management of holoprosencephaly, focusing on quality of life.
- Further research can explore long-term outcomes of this combined surgical technique.
Abstract:
Holoprosencephaly is a rare disorder of embryological development that presents with midline clefting of the lip. The incidence has been estimated at approximately 1 in 15,000. Previously, infants with severe holoprosencephaly were thought to die within 1-2 years of birth and seldom to benefit from surgery. Survival has increased with improved perinatal care and support services. Parents often request complete cheiloplasty, because the presence of a columella greatly influences aesthetic outcome. The authors report two cases of simultaneous columella reconstruction with cheiloplasty.