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Plastic surgery considerations for holoprosencephaly patients
Jennifer M Hendi1, Robert Nemerofsky, Cynthia Stolman
1Division of Plastic Surgery, Department of Surgery, New Jersey Medical School-University of Medicine and Dentistry of New Jersey, Newark, New Jersey, USA.
Insights
Holoprosencephaly (HPE) is a common brain and face abnormality. This study proposes surgical guidelines for consistent patient care, recommending early intervention for better outcomes.
Area of Science:
- Craniofacial Surgery
- Pediatric Neurosurgery
- Developmental Biology
Background:
- Holoprosencephaly (HPE) is the most common congenital anomaly of the brain and face.
- HPE presents with a spectrum of craniofacial malformations, including mid-line facial clefts, cyclopia, and nasal anomalies.
- Current management for HPE is primarily symptomatic and supportive, lacking a standardized treatment protocol.
Observation:
- The spectrum of HPE and its associated craniofacial anomalies are discussed.
- Surgical guidelines are proposed to standardize care for HPE patients.
- A case of a one-year-old female with HPE undergoing median lip repair and anterior palate repair is detailed.
Findings:
- Surgical intervention for HPE requires careful assessment of the brain abnormality to determine the extent and benefit.
- Repair of the lip and palate can be performed in young patients.
- The risks and benefits of surgical intervention were reviewed in the presented case.
Implications:
- Implementing standardized surgical guidelines can lead to more uniform and appropriate care for HPE patients.
- Early surgical intervention, guided by beneficence, may improve patient outcomes.
- Further research into surgical techniques and timing for HPE is warranted to optimize treatment strategies.
Abstract:
Holoprosencephaly (HPE) is considered the leading abnormality of the brain and face in humans and is frequently associated with a wide spectrum of specific craniofacial anomalies including mid-line facial clefts, cyclopia and nasal irregularities. A standard course of treatment has not been developed and management is symptomatic and supportive. In this work, the authors discuss the wide-ranging spectrum of HPE and propose surgical guidelines to provide more uniform and appropriate care to patients suffering from holoprosencephaly. Assessment of the patient's brain abnormality is essential in determining the extent and benefit of surgical intervention. The authors discuss a median straight-line repair of the lip and repair of the anterior palate in a one-year old female and review the risks and benefits of surgery. Consistent with the ethical approach of surgical beneficence, the authors recommend intervention at the earliest possible time.
