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Frontal Encephalocele Associated With a Bilateral Tessier Number Three Cleft and Fraser Syndrome
Benjamin C Wood1, Sojung Yi, Albert K Oh
1*Division of Plastic Surgery †Division of Neurosurgery, Children's National Medical Center, Washington, DC.
Insights
Rare craniofacial clefts combined with encephaloceles pose surgical challenges. This case highlights a male infant with frontoencephalocele and bilateral Tessier number 3 clefts, discussing management and surgical techniques.
Area of Science:
- Craniofacial surgery
- Pediatric neurosurgery
- Congenital anomalies
Background:
- Oblique craniofacial clefts and encephaloceles are rare congenital conditions.
- Combined occurrence is exceptionally infrequent, presenting significant reconstructive challenges.
- Literature review focuses on perioperative management and surgical techniques for these combined anomalies.
Observation:
- A male infant presented with a large right frontoencephalocele.
- The infant also exhibited bilateral Tessier number 3 clefts.
- This combination represents a rare presentation of craniofacial and neural tube defects.
Findings:
- The case involved a complex interplay of craniofacial and central nervous system malformations.
- Surgical intervention requires careful planning due to the extent of the defects.
- Successful management necessitates a multidisciplinary approach.
Implications:
- This case contributes to understanding the surgical management of rare combined craniofacial clefts and encephaloceles.
- Highlights the importance of tailored surgical strategies for complex congenital anomalies.
- Informs future reconstructive procedures and perioperative care protocols for similar rare conditions.
Abstract:
Oblique craniofacial clefts and encephaloceles are each rare conditions, and only a few instances of these findings in combination have been described. Each pathologic entity presents a unique reconstructive challenge. The authors report the case of a male infant who presented with a large right frontoencephalocele and bilateral Tessier number 3 clefts. A review of the pertinent literature, including specific considerations related to perioperative management and technical aspects of the surgical approach, is discussed.
