Unilateral Craniofacial Microsomia with Severe Tessier No. 7 Cleft and Mandibular Agenesis: A Rare Case Report

Craniofacial microsomia (CFM) presents with variable underdevelopment of craniofacial structures. The clinical severity varies widely and may lead to airway compromise and feeding difficulties in neonates. Herein, we describe a rare case of unilateral CFM with a severe Tessier number 7 cleft, complete mandibular agenesis, ear deformities, and extensive intraoral adhesions. Particularly, adhesions between the tongue and oral mucosa induced a deficiency of reconstructable soft tissue, making the release of the adhesion challenging and complicating the timing of surgical intervention. The patient required tracheostomy for respiratory distress and was fed via a nasogastric tube due to severe Tessier number 7 cleft, which caused significant difficulty with oral feeding. Considering the need for oral feeding and the anatomical challenges, surgical intervention was performed on day 70. The procedure involved removal of intraoral adhesions, separation of the oral cavity from the external face to restore functional integrity, and reconstruction of the oral commissure. This allowed initiation of oral feeding and facilitated aesthetic improvement without complications. This case illustrates a severe form of CFM and highlights the importance of timely, growth-adapted interventions to restore essential functions.

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Publication Details

Journal
The Cleft Palate-Craniofacial Journal
Published
2025-11-12
DOI
https://doi.org/10.1177/10556656251395572
Primary Topic
Craniofacial Disorders and Treatments
Type
article
Field-Weighted Citation Impact
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article

Unilateral Craniofacial Microsomia with Severe Tessier No. 7 Cleft and Mandibular Agenesis: A Rare Case Report

Yan Jin, Hyun Seung Lee, Kyung Hee Park, Chi Hyun Lee et al.
The Cleft Palate-Craniofacial Journal
Craniofacial Disorders and Treatments
article

Unilateral Craniofacial Microsomia with Severe Tessier No. 7 Cleft and Mandibular Agenesis: A Rare Case Report

Yan Jin, Hyun Seung Lee, Kyung Hee Park, Chi Hyun Lee, Su Jeong Park, Yong Chan Bae
article en

Abstract

Craniofacial microsomia (CFM) presents with variable underdevelopment of craniofacial structures. The clinical severity varies widely and may lead to airway compromise and feeding difficulties in neonates. Herein, we describe a rare case of unilateral CFM with a severe Tessier number 7 cleft, complete mandibular agenesis, ear deformities, and extensive intraoral adhesions. Particularly, adhesions between the tongue and oral mucosa induced a deficiency of reconstructable soft tissue, making the release of the adhesion challenging and complicating the timing of surgical intervention. The patient required tracheostomy for respiratory distress and was fed via a nasogastric tube due to severe Tessier number 7 cleft, which caused significant difficulty with oral feeding. Considering the need for oral feeding and the anatomical challenges, surgical intervention was performed on day 70. The procedure involved removal of intraoral adhesions, separation of the oral cavity from the external face to restore functional integrity, and reconstruction of the oral commissure. This allowed initiation of oral feeding and facilitated aesthetic improvement without complications. This case illustrates a severe form of CFM and highlights the importance of timely, growth-adapted interventions to restore essential functions.

The Cleft Palate-Craniofacial Journal
Pusan National University Hospital (KR), Pusan National University (KR)
Openalex Percentile: Top 64%
Craniofacial Disorders and Treatments
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