Overview
Definition:
Cleft lip and palate are congenital conditions characterized by incomplete fusion of facial structures during embryonic development
A cleft lip involves a fissure in the upper lip, which can be unilateral or bilateral, and may extend into the nostril
A cleft palate is an opening in the roof of the mouth, affecting the hard and/or soft palate
These conditions often occur together as orofacial clefts.
Epidemiology:
Orofacial clefts are among the most common congenital anomalies worldwide, with an estimated incidence of 1 in 700 to 1 in 1000 live births
Incidence varies significantly by ethnicity, geographic region, and socioeconomic factors
In India, the prevalence is estimated to be around 0.8 to 1.5 per 1000 live births
Males are more frequently affected by cleft lip, while females have a higher incidence of isolated cleft palate.
Clinical Significance:
Cleft lip and palate pose significant challenges impacting feeding, speech, hearing, dental development, and psychosocial well-being
Early and appropriate management is crucial for optimal physical and social outcomes, requiring a multidisciplinary approach involving pediatricians, surgeons, orthodontists, speech therapists, and audiologists
Understanding feeding strategies and surgical timing is fundamental for pediatric residents preparing for board examinations.
Feeding Strategies
Nutritional Challenges:
Infants with clefts often struggle with effective sucking and swallowing due to inadequate intraoral seal and lack of palatal integrity, leading to poor weight gain, dehydration, and aspiration risk
Problems include inefficient milk transfer, nasal regurgitation, and increased air swallowing.
Breastfeeding Techniques:
While challenging, breastfeeding may be possible with modifications
Specialized techniques include proper positioning, using a lactation consultant, and manual expression of milk
A finger or nipple can help occlude the cleft temporarily.
Bottle Feeding Devices:
Specialized feeding bottles with cross-cut nipples or nipples designed for clefts (e.g., pigeon bottles, Mead-Johnson bottles) are often necessary
These allow for a controlled flow of milk and require less forceful sucking
Squeeze bottles can also be employed to assist milk delivery.
Nasogastric And Orogastric Feeding:
In cases of severe feeding difficulties or failure to thrive, nasogastric (NG) or orogastric (OG) tube feeding may be required temporarily to ensure adequate caloric intake and hydration, especially in the neonatal period before surgical intervention.
Surgical Timing And Principles
Cleft Lip Repair:
Primary cleft lip repair (cheiloplasty) is typically performed between 3 to 6 months of age, often when the infant weighs at least 10 pounds (4.5 kg), has a hemoglobin of 10 g/dL, and is generally healthy
The goal is to achieve a functional and aesthetically pleasing lip, restoring muscle continuity and symmetry.
Cleft Palate Repair:
Primary cleft palate repair (palatoplasty) is generally performed between 9 to 18 months of age
This timing is crucial to allow for adequate speech development, as early repair may interfere with maxillary growth, while delayed repair can negatively impact speech
The aims are to achieve competent velopharyngeal function for speech, prevent oronasal fistulae, and create a patent airway.
Multidisciplinary Team Approach:
Management of cleft lip and palate requires a coordinated effort from a multidisciplinary team
This includes plastic surgeons, pediatric surgeons, otolaryngologists, orthodontists, speech-language pathologists, audiologists, geneticists, and psychologists
The team guides feeding strategies, surgical planning, and long-term rehabilitation.
Goals Of Surgery:
The overarching goals of surgical intervention are to restore normal facial form and function, enabling proper feeding, speech, hearing, and psychosocial development
This involves achieving aesthetic symmetry, competent velopharyngeal closure, and adequate dental arch form for future prosthodontic and orthodontic interventions.
Associated Concerns And Management
Hearing Impairment:
Children with cleft palate have an increased risk of otitis media with effusion and hearing loss due to dysfunction of the Eustachian tube
Regular audiological assessment and consideration for tympanostomy tube insertion are essential.
Speech And Language Development:
Velopharyngeal insufficiency (VPI) is common after cleft palate repair, leading to hypernasal speech
Speech therapy is vital, and in persistent cases, secondary surgical procedures like pharyngeal flap or sphincter pharyngoplasty may be necessary.
Dental And Orthodontic Issues:
Clefts often result in malocclusion, missing or malformed teeth, and alveolar ridge defects
Early orthodontic assessment and management, including nasoalveolar molding (NAM) and alveolar bone grafting (ABG), are critical for optimizing dental arch form and facilitating eruption of teeth.
Nasal Deformity:
The nasal asymmetry is a significant component of cleft lip and palate
Secondary rhinoplasty is often performed in late adolescence to refine nasal aesthetics and function, often in conjunction with or after other reconstructive surgeries.
Key Points
Exam Focus:
Recall the typical age ranges for primary lip repair (3-6 months) and palate repair (9-18 months)
Understand the importance of achieving adequate weight and hemoglobin before lip repair
Recognize the link between cleft palate and Eustachian tube dysfunction, leading to otitis media and hearing loss.
Clinical Pearls:
Emphasize patient and family education regarding feeding techniques
Consider specialized feeding equipment early
A multidisciplinary team approach is paramount for comprehensive management
Early identification of speech and hearing issues allows for timely intervention.
Common Mistakes:
Delaying palate repair beyond 18 months can impact speech development
Inadequate nutritional support leading to failure to thrive can postpone necessary surgeries
Neglecting regular audiological and speech assessments can lead to long-term functional deficits
Rushing surgical interventions without adequate preparation or patient stability.