Abstract
The authors provide an in-depth review of cleft lip and plate from developmental pathophysiology through the surgical correction. Commonly associated syndromes are discussed in addition to the relevant anesthetic concerns present in this population.
A 12-month-old male presents for a primary cleft palate repair. The patient was recently adopted, and no significant medical history has been documented.
In the preoperative holding area, the patient is being held and appears calm. The patient’s preoperative vital signs are: weight 10 kg, T 36.7°C, pulse 110 bpm, BP 90/54, RR 28, SpO2 99% on room air. On physical exam, the patient is noted to have a right-sided cleft palate. The patient appears to have slight micrognathia. The remainder of the physical exam is within normal limits.
When Does Development of the Face and Lip Occur? When Does Development of the Palate Occur?
The development of the lip occurs during weeks four to eight of gestation. The primary palate (palate that is anterior to the incisive foramen) forms during week six of gestation with the secondary palate formed during weeks six to twelve. Cleft lip and alveolus results from failure of fusion of the nasal and maxillary prominences, whereas cleft palate (secondary palate) results from failure of fusion of the palatal shelves.
What Are the Predisposing Risk Factors Related to Cleft Lip and Palate?
Causes of cleft lip and palate are multifactorial and reported etiologies include genetics, maternal exposures and risk factors (smoking, alcohol, gestational diabetes, folate deficiency), as well as teratogens (retinoic acid, valproic acid, phenytoin).
What Is the Incidence of Cleft Lip and Palate?
The incidence of orofacial clefts is 1 in 700 live births. Cleft lip with or without cleft palate is more common in males (2:1) and certain ethnic populations (Asian, Native American). Nonsyndromic forms of cleft lip with or without cleft palate account for 70% of all cases.
How Are Different Types of Cleft Lip and Palate Classified?
The classification of craniofacial clefts was described by Dr. Tessier in 1976. Orofacial clefts are the most common craniofacial cleft deformity, and multiple classification systems for orofacial clefts exist. Cleft lip and palate can occur in combination or in isolation. Cleft lip is commonly described as unilateral or bilateral, and complete or incomplete. The clinical severity of cleft palate ranges from a submucosal, often undetectable, cleft to a complete bilateral cleft of the primary and secondary palate.
What Physiological Problems Are Common for Patients with Cleft Palate?
In cleft palate patients, improper closure between the palate and nasopharynx can lead to physiological problems of feeding (unable to generate negative pressure for swallowing and sucking) and difficulties with speech and language development. Eustachian tube dysfunction and middle ear disease related to abnormal insertion of the tensor veli palatini can result in conductive hearing loss. Inability to oppose maxillary and mandibular teeth leads to the inability to properly chew food.
When Is a Cleft Lip and/or Palate Typically Repaired?
Primary cleft lip repair is typically performed at around age 10–12 weeks. Historically, the “rule of 10s” described the preoperative parameters of weight over 10 lbs, hemoglobin over 10 g/dL, and age over 10 weeks. Primary cleft palate repair is typically performed around age 9–12 months in order to optimize speech and language development.
What Other Procedures Are Common for These Patients?
Eustachian tube dysfunction, middle ear disease, and conductive hearing loss may be associated with cleft palate; these patients often require placement of myringotomy tubes. In the long term, these patients may require multiple procedures including lip and nasal revisions, additional palate surgery for velopharyngeal insufficiency, alveolar bone grafting, dental procedures, maxillary advancement, and cosmetic septorhinoplasty. Orthodontic intervention is usually required following palate repair to improve maxillary hypoplasia and crowding of the teeth.
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