Cleft Lip & Palate I

Complete bilateral CLP involves full separation on both sides through lip, alveolus, and palate, creating a premaxillary segment that's completely separated. Includes bilateral nasal deformity and protruding premaxilla.

Veau Type I is isolated soft palate cleft. This system includes: Type I (soft palate), Type II (hard and soft palate), Type III (complete unilateral), Type IV (complete bilateral). Understanding this classification helps in treatment planning

Simonart's band is a tissue bridge crossing the cleft at the nasal floor in incomplete clefts. Its presence can affect surgical planning and technique selection. May provide better columellar support.

In LAHSHAL classification, the first 'L' represents the right lip. This striped classification system describes: Lip, Alveolus, Hard palate, Soft palate (and reverse). Capital letters indicate complete clefts, lowercase incomplete.

Extends through lip, alveolus, and entire palate on one side. Creates complete separation from oral to nasal cavity, involving both primary and secondary palate. Results in significant nasal asymmetry.

A microform cleft shows a subtle notching of the vermillion border with minimal muscle disruption. May include slight nasal deformity. Also called mini-microform or forme fruste cleft. It's the mildest form of cleft lip.

Submucous clefts are characterized by the following classic triad (known as Calnan's triad):

• Bifid uvula: A split or forked uvula is often visible.
• Zona pellucida: A thin, bluish, translucent zone in the midline of the soft palate, caused by a lack of muscle tissue.
• Notching of the posterior hard palate: Palpable or visible notch at the posterior edge of the hard palate due to incomplete fusion.

Types

Complete SMCP: Involves both the soft and hard palate.
Incomplete SMCP: Limited to the soft palate.

Clinical Manifestations

Feeding difficulties in infants.
Speech problems, including hypernasality and nasal air escape.
Eustachian tube dysfunction: Recurrent otitis media due to poor function of the tensor veli palatini muscle.
Sometimes asymptomatic, identified incidentally during evaluation for other issues.

Diagnosis

Physical Examination: Look for Calnan’s triad.
Nasopharyngoscopy: To assess velopharyngeal insufficiency (VPI).
Imaging: Rarely needed, but CT or MRI can reveal the defect in more detail.

Management

Conservative:

Mild cases may not require intervention if asymptomatic.
Speech therapy for minor speech issues

Surgical (for significant VPI or other functional issues):

Palatoplasty: Repair of soft palate muscles to improve function.
Pharyngoplasty: May be needed in severe VPI cases.

An incomplete unilateral cleft lip is a congenital anomaly where the cleft extends partially through the upper lip but does not reach the nasal floor. This type of cleft may involve the muscle (orbicularis oris) and mucosa to varying degrees, resulting in functional and aesthetic concerns depending on the severity.

It is distinct from a microform cleft, which is a mild form of clefting that typically affects only the vermilion border and may appear as a subtle notch or indentation without significant disruption to the deeper muscular or mucosal structures.

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Secondary palate fusion occurs between weeks 8-12. The palatal shelves elevate and fuse medially during this period. This later timing explains why palatal clefts can occur independently of lip clefts.

Females are more commonly affected by isolated cleft palate (2:1 ratio). However, males are more commonly affected by cleft lip with or without cleft palate.

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The Van der Woude syndrome presents with cleft lip and/or palate in 88% of cases, along with characteristic lower lip pits. Key syndrome features:

Van der Woude Syndrome:

• Autosomal dominant
• Lower lip pits (pathognomonic)
• CL/P in 88% of cases
• IRF6 gene mutation
• High penetrance

About 90% of children with cleft palate develop middle ear problems due to Eustachian tube dysfunction. This high percentage necessitates early ENT involvement and regular monitoring.

Ruegg, T. A., Cooper, M. E., Leslie, E. J., Ford, M. D., Wehby, G. L., Frederic, … Weinberg, S. M. (2015). Ear Infection in Isolated Cleft Lip: Etiological Implications. The Cleft Palate-Craniofacial Journal, 54(2), 189–192. https://doi.org/10.1597/15-010

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Approximately 70% of cleft lip cases also have cleft palate involvement. This high association rate influences treatment planning and timing of interventions. Understanding this helps in comprehensive care planning.

The recurrence risk is approximately 4% for parents with one affected child. This risk increases if multiple family members are affected or if the cleft is syndromic. Important for genetic counseling.

D. Grosen, Chevrier, C., A. Skytthe, Bille, C., K. Molsted, Sivertsen, A., … Christensen, K. (2009). A cohort study of recurrence patterns among more than 54 000 relatives of oral cleft cases in Denmark: support for the multifactorial threshold model of inheritance. Journal of Medical Genetics, 47(3), 162–168. https://doi.org/10.1136/jmg.2009.069385

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While Down syndrome (DS), or Trisomy 21, is the most common chromosomal abnormality in newborns, it's rarely associated with cleft lip and palate; all other listed syndromes are associated with CL/P.

Note: Pierre Robin Sequence (micrognathia, glossoptosis, and cleft palate) is most commonly associated with isolated cleft palate.

Primary palate (lip and premaxilla) forms between weeks 4-7. Specifically, fusion of medial nasal and maxillary processes occurs during this period. This timing explains why early pregnancy factors affect lip formation.

Geneser, M. K., & Veerasathpurush Allareddy. (2019). Cleft Lip and Palate. Elsevier EBooks, 77-87.e2. https://doi.org/10.1016/b978-0-323-60826-8.00005-5

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Left-sided clefts occur approximately 2:1 compared to right-sided clefts. This left-side predominance is consistent across populations and may relate to developmental blood supply patterns.

Note:  There is a 2:1 male:female ratio and a similar 2:1 left-side:right-side ratio for unilateral clefts.

LaRossa, D., Randall, P., Cohen, M. A., & Afifi, G. Y. (2010). Cleft Palate. Elsevier EBooks, 151–156. https://doi.org/10.1016/b978-0-323-03470-8.00023-5

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Approximately ~ 30% of cleft cases are syndromic, while over 50% are isolated or non-syndromic, and another ~20% are associated with non-recognized syndromes. Syndromic cases often involve additional congenital anomalies and may require more complex management.

Martinelli, M., Palmieri, A., Carinci, F., & Luca Scapoli. (2020). Non-syndromic Cleft Palate: An Overview on Human Genetic and Environmental Risk Factors. Frontiers in Cell and Developmental Biology, 8. https://doi.org/10.3389/fcell.2020.592271

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The worldwide incidence is approximately 1:700 live births, with variation among ethnic groups. Asian populations show higher rates (1:500) while African populations show lower rates (1:1200).

Maternal smoking during pregnancy shows the strongest environmental association, increasing risk by 2-3 times. Other factors include folate deficiency, alcohol use, and certain medications, but smoking shows most consistent correlation.

More: "Also maternal tobacco smoking has been deeply investigated as an NSCPO risk factor. The studies were recently considered in two meta-analyses, which concluded by supporting a role for both maternal tobacco smoking (Little et al., 2004) and passive smoking (Sabbagh et al., 2015) in NSCPO etiology"

Martinelli, M., Palmieri, A., Carinci, F., & Luca Scapoli. (2020). Non-syndromic Cleft Palate: An Overview on Human Genetic and Environmental Risk Factors. Frontiers in Cell and Developmental Biology, 8. https://doi.org/10.3389/fcell.2020.592271

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