Cleft Lip and Palate in Children
Introduction to Cleft Lip and Palate
Cleft lip and palate are common congenital anomalies affecting the orofacial region. They occur due to incomplete fusion of the facial processes during embryonic development. These conditions can occur separately or in combination, affecting approximately 1 in 700 live births worldwide, with variations based on ethnicity and geographic location.
Key points:
- Cleft lip: A physical split or separation of the two sides of the upper lip
- Cleft palate: An opening in the roof of the mouth
- Can occur unilaterally or bilaterally
- May be associated with other syndromes or occur in isolation
- Multifactorial etiology involving genetic and environmental factors
Embryology of Cleft Lip and Palate
Understanding the embryological development of the face and palate is crucial for comprehending the etiology of cleft lip and palate.
Face Development:
- Occurs between 4th and 8th weeks of gestation
- Involves five facial processes: frontonasal prominence, paired maxillary processes, and paired mandibular processes
- Fusion of medial nasal processes with maxillary processes forms the upper lip and primary palate
Palate Development:
- Occurs between 6th and 12th weeks of gestation
- Primary palate: Anterior to the incisive foramen
- Secondary palate: Posterior to the incisive foramen
- Palatal shelves elevate and fuse in the midline, separating oral and nasal cavities
Disruption in any of these processes can lead to cleft lip and/or palate formation.
Classification of Cleft Lip and Palate
Several classification systems exist for cleft lip and palate. The most commonly used is the LAHSHAL system:
- L: Lip
- A: Alveolus
- H: Hard palate
- S: Soft palate
Each side is represented, with uppercase letters indicating complete clefts and lowercase letters indicating incomplete clefts.
Types of Clefts:
- Cleft Lip:
- Unilateral: Complete or incomplete
- Bilateral: Complete or incomplete
- Cleft Palate:
- Complete: Involves both hard and soft palate
- Incomplete: Involves only part of the palate
- Submucous cleft: Defect in muscular layer with intact mucosa
- Combined Cleft Lip and Palate:
- Unilateral
- Bilateral
Clinical Presentation of Cleft Lip and Palate
The clinical presentation varies depending on the type and extent of the cleft:
Cleft Lip:
- Visible separation of the upper lip
- May extend into the nose (complete cleft)
- Asymmetry of the nose and face
- Feeding difficulties
Cleft Palate:
- Opening in the roof of the mouth
- Feeding difficulties (poor suction, nasal regurgitation)
- Speech problems (hypernasality, articulation errors)
- Recurrent ear infections
Combined Cleft Lip and Palate:
- Features of both cleft lip and palate
- More severe functional and aesthetic concerns
Associated features may include dental anomalies, hearing impairment, and in some cases, other congenital anomalies as part of syndromes.
Diagnosis of Cleft Lip and Palate
Diagnosis of cleft lip and palate can be made prenatally or postnatally:
Prenatal Diagnosis:
- Ultrasound: Can detect cleft lip as early as 13 weeks gestation
- 3D ultrasound: Provides better visualization of facial structures
- MRI: Used in select cases for better soft tissue definition
Postnatal Diagnosis:
- Physical examination: Visual inspection and palpation
- Nasendoscopy: For submucous cleft palate
- Radiographic studies: 3D CT or MRI for complex cases
Associated Investigations:
- Genetic testing: For syndromic cases
- Hearing assessment
- Dental evaluation
Early diagnosis is crucial for timely intervention and management planning.
Management of Cleft Lip and Palate
Management of cleft lip and palate requires a multidisciplinary approach involving various specialists:
Surgical Management:
- Cleft Lip Repair:
- Typically performed at 3-6 months of age
- Techniques: Millard rotation advancement, Tennison-Randall
- Cleft Palate Repair:
- Usually performed at 9-18 months of age
- Techniques: Von Langenbeck, Furlow double-opposing Z-plasty
- Alveolar Bone Grafting:
- Performed between 7-9 years of age
- Uses autologous bone graft (often from iliac crest)
- Secondary Procedures:
- Rhinoplasty, lip revision, pharyngoplasty as needed
Non-surgical Management:
- Feeding support: Special bottles, positioning techniques
- Speech therapy: Ongoing assessment and intervention
- Orthodontic treatment: Pre-surgical orthopedics, dental alignment
- Hearing management: Regular assessments, grommet insertion if needed
- Psychological support: For patients and families
Long-term follow-up is essential to monitor growth, development, and address any emerging issues.
Complications of Cleft Lip and Palate
Various complications can arise in patients with cleft lip and palate:
Early Complications:
- Feeding difficulties
- Failure to thrive
- Recurrent upper respiratory tract infections
- Otitis media with effusion
Late Complications:
- Speech disorders:
- Velopharyngeal insufficiency
- Articulation errors
- Dental abnormalities:
- Malocclusion
- Supernumerary or missing teeth
- Hearing impairment
- Nasal deformities
- Psychological and social issues
Surgical Complications:
- Wound dehiscence
- Fistula formation
- Hypertrophic scarring
- Midface growth restriction
Regular follow-up and timely intervention can help minimize these complications.
Prognosis of Cleft Lip and Palate
The prognosis for children with cleft lip and palate has significantly improved with advancements in surgical techniques and multidisciplinary care:
Factors Affecting Prognosis:
- Type and severity of the cleft
- Timing and quality of surgical interventions
- Presence of associated anomalies or syndromes
- Access to comprehensive care
- Family support and compliance with treatment
Outcomes:
- Aesthetic: Most patients achieve good facial appearance with minimal scarring
- Functional:
- Speech: Majority achieve normal or near-normal speech with therapy
- Hearing: Can be preserved with proper management of ear infections
- Dental: Orthodontic treatment can achieve good occlusion
- Psychosocial: Many patients lead normal lives with appropriate support
Long-term follow-up into adulthood is recommended to address any residual issues and monitor overall well-being.
Cleft Lip and Palate in Children
- Q1: What is a cleft lip?
A: A congenital split or separation in the upper lip. - Q2: What is a cleft palate?
A: A congenital opening or split in the roof of the mouth. - Q3: What is the most common type of orofacial cleft?
A: Cleft lip with or without cleft palate. - Q4: At what stage of fetal development do cleft lip and palate typically occur?
A: During the first trimester, typically between the 6th and 9th weeks of pregnancy. - Q5: What is the approximate incidence of cleft lip and palate worldwide?
A: Approximately 1 in 700 live births - Q6: What are the two main types of cleft lip?
A: Unilateral cleft lip and bilateral cleft lip. - Q7: What is a submucous cleft palate?
A: A type of cleft palate where the defect is covered by the oral mucosa, making it less visible. - Q8: What is the recommended age for surgical repair of a cleft lip?
A: Typically around 3-6 months of age. - Q9: When is cleft palate repair usually performed?
A: Usually between 9-18 months of age. - Q10: What are the main feeding challenges for infants with cleft lip and palate?
A: Difficulty creating suction, nasal regurgitation, and prolonged feeding times. - Q11: What specialized feeding device is often used for infants with cleft palate?
A: Special squeezable bottles or cups with soft, cross-cut nipples. - Q12: What is the role of a speech-language pathologist in cleft care?
A: To assess and treat speech and language disorders associated with cleft lip and palate. - Q13: What is velopharyngeal insufficiency?
A: Incomplete closure between the soft palate and pharynx, leading to hypernasality in speech. - Q14: What dental issues are common in children with cleft lip and palate?
A: Missing, malformed, or supernumerary teeth, and malocclusion. - Q15: What is an alveolar bone graft?
A: A surgical procedure to fill the cleft in the gum ridge with bone, typically performed around age 7-9. - Q16: What is the purpose of presurgical infant orthopedics (e.g., nasoalveolar molding)?
A: To align the segments of the cleft lip and palate before surgery, potentially improving surgical outcomes. - Q17: What genetic syndrome is most commonly associated with cleft lip and palate?
A: Van der Woude syndrome. - Q18: What environmental factors may increase the risk of cleft lip and palate?
A: Maternal smoking, alcohol consumption, certain medications, and nutritional deficiencies during pregnancy. - Q19: What is a pharyngeal flap surgery?
A: A procedure to correct velopharyngeal insufficiency by creating a flap of tissue at the back of the throat. - Q20: How can cleft lip and palate affect a child's hearing?
A: It can lead to frequent ear infections and potential hearing loss due to dysfunction of the Eustachian tubes. - Q21: What is the role of orthodontics in cleft lip and palate treatment?
A: To align teeth, correct bite issues, and prepare the mouth for alveolar bone grafting and further surgeries. - Q22: What is a fistula in the context of cleft palate repair?
A: An abnormal opening between the mouth and nose that may occur after cleft palate repair. - Q23: What is the recommended composition of a cleft lip and palate care team?
A: Multidisciplinary team including plastic surgeon, orthodontist, speech therapist, ENT specialist, pediatrician, and psychologist. - Q24: What is Pierre Robin sequence?
A: A condition characterized by a small lower jaw, posterior displacement of the tongue, and cleft palate. - Q25: How can cleft lip and palate affect a child's social and emotional development?
A: It may lead to self-esteem issues, social anxiety, and challenges in peer relationships due to differences in appearance and speech. - Q26: What is the purpose of speech bulbs or speech prostheses?
A: To help close the velopharyngeal port during speech in cases where surgical correction is not possible or complete. - Q27: What is the role of genetic counseling for families affected by cleft lip and palate?
A: To provide information about recurrence risks, associated syndromes, and potential preventive measures for future pregnancies. - Q28: What is a Simonart's band in cleft lip?
A: A strip of tissue that bridges the cleft in incomplete cleft lip. - Q29: How can 3D ultrasound be useful in diagnosing cleft lip and palate?
A: It can provide detailed images of the fetal face, allowing for early detection and better parental preparation. - Q30: What long-term follow-up is typically required for children with repaired cleft lip and palate?
A: Regular evaluations of speech, hearing, dental development, and overall growth until adulthood, with potential additional surgeries as needed.
