Questions and Answers
What is the prevalence of cleft palate alone in the US?
1 in 1500
Which ethnic group has the highest prevalence of cleft lip and palate based on the given information?
Asian
What is the main embryological process involved in the formation of cleft lip during the fourth week of embryogenesis?
Failure of fusion between maxillary and medial nasal processes
What is the difference in syndromic presentation between cleft lip and palate, and cleft palate alone?
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What is a characteristic feature associated with Van der Woude syndrome?
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What is a common characteristic associated with Charge syndrome?
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Which muscle helps to open the eustachian tubes and is often malpositioned in cleft palate patients, leading to middle ear effusions?
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What is the repair sequence for addressing issues in cleft palate patients?
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What procedure is performed for unilateral cleft lip patients to reposition alveolar segments and reshape nasal cartilage?
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What is the classification for a complete unilateral cleft lip and palate including the alveolus?
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Which syndrome is characterized by hypogonadotropic hypogonadism, anosmia, renal agenesis, and dental defects?
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What is the recommended treatment for a sagittal closure pattern in Velopharyngeal Insufficiency (VPI)?
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Study Notes
- The Loop Podcast episode discusses cleft lip and palate, a complex topic with varying presentations and causes.
- There are two main groups: cleft lip with or without cleft palate, and cleft palate alone.
- Prevalence in the US is one in seven hundred for cleft lip and palate, one in fifteen hundred for cleft palate alone.
- Male predilection for cleft lip and palate, female predilection for cleft palate alone.
- Ethnic distribution: Asia to Caucasian to African, 4:2:1 for cleft lip and palate, no difference for cleft palate alone.
- Cleft lip and palate is less syndromic than cleft palate alone (15% versus 50%).
- Embryology: During the fourth week of embryogenesis, five processes are formed, including the frontonasal process.
- Failure of fusion during this time leads to cleft lip (median, unilateral) and cleft palate (primary).
- Risk of having a child with cleft lip and palate: 2.5% for isolated sibling, 4.2% for sibling with cleft lip, 10% for two siblings, 17% for parent and sibling.
- Two most common syndromes associated with cleft lip and palate: Van der Woude syndrome (autosomal dominant inheritance, irf6 mutation, lip pits, small teeth) and Charge syndrome (mutation in chd7 gene, colobomas, heart defects, atresia of nasal coana, retardation of growth, genital and urinary abnormalities, ear deafness).
- Other syndromes: Coleman syndrome (hypogonadotropic hypogonadism, anosmia, renal and genesis or aplasia, dental defects), velocardiofacial syndrome (22q 11.2 deletion, long face, wide nose, small ears, decreased muscle tone, worse speech outcomes, VPI secondary to hypotonia), trisomy 13 or Patau syndrome (cleft palate, CNS disorders, microcephaly, polydactyly, rocker bottom feet, urogenital defects, cardiac anomalies).
- VO Classification for cleft palates: Class I (incomplete cleft of the soft palate), Class II (hard and soft palate but secondary palate only), Class III (complete unilateral cleft lip and palate including the alveolus), Class IV (complete bilateral cleft lip and palate).
- Muscles involved: Levator vel palatini (elevates the soft palate), Tensor palatini (helps open the eustachian tubes, malpositioned in cleft palate patients leading to middle ear effusions).
- Repair sequence: Address speech issues, then follow ideal sequence (cleft lip repair at 3-4 months, closure of anterior nasal floor and primary tip rhinoplasty if needed, cleft palate close at 6-12 months, secondary rhinoplasty and revisionary at 5-6 years, definitive surgery at 12-14 years or if maxillary mandibular orthognathics are needed).
- Common stigmata of different repair types: Short lip for rotational advancements, elongated lip for triangular or Tennison flaps, blunted cupid's box for bilateral repairs.
- Evaluation of closure for cleft palate: Nasoendoscopy and video-assisted fluoroscopy to assess velopharyngeal competency and palatal motion.
- Velopharyngeal insufficiency (VPI) treatment: Sphincter pharyngioplasty for coronal closure pattern, pharyngeal flap for sagittal closure pattern, paladoplasty for persistent issues after tonsillectomy and adenectomy.
- Screening for obstructive sleep apnea (OSA) in patients with VPI and orthognathic surgery.
- Repositioning and approximating alveolar segments and reshaping nasal cartilage with the Nam procedure for unilateral cleft lip patients.
- Common dental anomalies: Tooth agenesis (lateral incisor on cleft side), supernumerary teeth.
- Hypoplastic maxilla and five millimeters of alveolar cleft bone stock: Sufficient for implant or conventional LeFort one surgery with two-piece LeFort one and closing the alveolar cleft and correcting occlusion.
- Changes in mid-face with LeFort one advancement surgery: Increased Smith facial projection and fullness, decreased upper lip height, decreased depth of nasolabial folds, increased tooth show in repose and smile, increased alert base width, increased nasolabial angle.
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