Residency · Residency · Plastic Surgery

Cleft Palate: Evaluation and Primary Repair

Introduction

Cleft palate occurs in approximately 1 in 2000 live births as an isolated anomaly. Isolated cleft palate (CP) is embryologically and genetically distinct from cleft lip with/without palate (CL/P). Female predominance in isolated CP (2:1 female:male). Isolated CP has a higher rate of associated anomalies and syndromes (~50%) compared to CL/P (~30%).

The primary goal of palatal repair is to create a functional velopharyngeal mechanism for normal speech while minimizing impact on maxillary growth.

Anatomy of the Palate

Hard Palate

Anterior 2/3 of the palate. Bony components: premaxilla (primary palate), palatine processes of maxilla, horizontal plates of palatine bones. Mucoperiosteum is tightly adherent to bone. Greater palatine artery: primary blood supply, emerges from greater palatine foramen, runs anteriorly in a submucosal groove. Incisive foramen: landmark dividing primary from secondary palate.

Soft Palate (Velum)

Posterior 1/3; no bony scaffold. Five paired muscles: Levator veli palatini: primary muscle of velopharyngeal closure; elevates and retracts the velum. Tensor veli palatini: tenses the palate and opens the eustachian tube; wraps around the hamulus.

Musculus uvulae: adds bulk to the nasal surface of the velum, aids velopharyngeal seal. Palatoglossus: forms the anterior tonsillar pillar; depresses palate/elevates tongue. Palatopharyngeus: forms the posterior tonsillar pillar; depresses palate/constricts pharynx. In the cleft: levator muscles insert abnormally along the posterior edge of the hard palate rather than forming a continuous sling across the midline (the levator sling).

Velopharyngeal Mechanism

During speech and swallowing, the soft palate elevates to contact the posterior pharyngeal wall. Velopharyngeal closure patterns: coronal (most common), sagittal, circular, circular with Passavant ridge. Adequate closure prevents nasal air escape and hypernasality.

Classification

Complete cleft palate: involves both primary and secondary palate (lip through soft palate). Incomplete cleft palate: involves secondary palate only (posterior to incisive foramen). Submucous cleft palate: triad of bifid uvula, zona pellucida (midline translucency), notched posterior hard palate; intact mucosal surface with underlying muscle diastasis. Occult submucous cleft: muscle diastasis without classic surface findings; diagnosed on nasendoscopy or MRI.

Veau classification: I (soft palate only), II (hard and soft palate), III (unilateral complete), IV (bilateral complete).

Associated Syndromes and Conditions

Pierre Robin sequence: micrognathia → glossoptosis → airway obstruction → U-shaped cleft palate; associated with Stickler syndrome in 25-35%. Stickler syndrome: connective tissue disorder (COL2A1, COL11A1), cleft palate, myopia, retinal detachment, hearing loss, joint hypermobility. Velocardiofacial (22q11.2 deletion) syndrome: cleft palate/VPI, conotruncal cardiac defects, T-cell deficiency, hypocalcemia, learning disabilities. Van der Woude syndrome: autosomal dominant, IRF6 mutation, cleft lip/palate with lower lip pits.

Treacher Collins syndrome: mandibulofacial dysostosis with cleft palate in 30%. Apert syndrome: FGFR2 mutation, craniosynostosis, midface hypoplasia, cleft palate common.

<image>Anatomical illustration of the soft palate musculature in normal versus cleft palate anatomy, shown in inferior (oral) view. The left panel shows normal anatomy with the levator veli palatini muscles forming a continuous muscular sling across the midline, the tensor veli palatini hooking around the hamulus, the musculus uvulae in the midline, and the palatoglossus and palatopharyngeus forming the tonsillar pillars. The right panel shows the cleft palate with the levator muscles inserting abnormally along the posterior edge of the bony hard palate on each side rather than meeting in the midline, creating a V-shaped deficiency. Arrows indicate the direction of surgical dissection needed to detach and reorient the levator muscles into the correct transverse sling position (intravelar veloplasty). All muscles are labeled and color-coded.</image>

Preoperative Management

Feeding

Most cleft palate infants cannot generate adequate negative pressure for breastfeeding. Specialized bottles: Haberman feeder, Dr. Brown's Specialty Feeding System, pigeon nipple. Upright feeding position to reduce nasal regurgitation. Obturator plate may assist feeding (rarely necessary with proper bottle technique). Adequate weight gain must be documented before surgery.

Hearing and ENT

>90% of cleft palate children develop middle ear effusions due to eustachian tube dysfunction (tensor veli palatini malfunction). Audiologic evaluation mandatory. Myringotomy with tube placement at the time of palate repair (standard practice).

Airway Assessment (Pierre Robin)

Prone positioning, side-lying. Tongue-lip adhesion if positioning fails. Mandibular distraction osteogenesis for severe obstruction. Tracheostomy as last resort. Delayed palatoplasty if airway concerns persist.

Timing of Repair

Standard: 9-12 months of age (before speech development). Earlier repair (6-9 months) advocated by some for speech benefits. Two-stage repair: soft palate at 6 months, hard palate at 12-18 months (Schweckendiek protocol; largely abandoned due to persistent VPI and fistula risk, though some centers report reduced growth impairment). Delayed repair (>18 months) associated with worse speech outcomes. Balance: early repair optimizes speech; later repair may minimize maxillary growth disturbance.

Surgical Techniques

Von Langenbeck Bipedicled Flap

Oldest technique (1861). Bilateral bipedicled mucoperiosteal flaps based on greater palatine arteries. Lateral relaxing incisions along alveolar ridge. Flaps mobilized medially and sutured in the midline.

Advantages: technically straightforward, reliable. Disadvantages: does not lengthen the palate; higher VPI rate, tension on closure. Rarely used as primary technique today; useful for narrow clefts.

Bardach Two-Flap Palatoplasty

Most widely used technique in North America. Two large mucoperiosteal flaps raised from the hard palate, based on the greater palatine arteries. Greater palatine neurovascular bundle preserved; may be fractured from the foramen for additional mobilization. Flaps transposed medially; nasal layer closed separately.

Intravelar veloplasty performed for muscle repositioning. Advantages: wide exposure, versatile for all cleft widths. Disadvantages: extensive periosteal elevation may impact maxillary growth (bare bone areas).

Furlow Double-Opposing Z-Plasty

Described by Leonard Furlow (1986). Concept: two opposing Z-plasties — one on the oral layer, one on the nasal layer — transposed in opposite directions. Accomplishes two goals simultaneously: 1. Lengthens the palate (Z-plasty geometric lengthening).

1. Reconstructs the levator sling (muscle repositioned into a transverse orientation within the Z-plasty flaps). Technique: Oral Z-plasty: posteriorly based myomucosal flap on the left containing levator muscle; anteriorly based mucosal flap on the right. Nasal Z-plasty: mirror image — posteriorly based myomucosal flap on the right; anteriorly based mucosal flap on the left.

Interdigitation creates muscular overlap in the midline. Advantages: palatal lengthening, levator sling reconstruction, lower VPI rate reported, no lateral relaxing incisions. Disadvantages: technically demanding, limited use for wide clefts or hard palate clefts, risk of dehiscence in wide clefts. Best suited for: isolated soft palate clefts, narrow incomplete clefts, submucous clefts with VPI.

Intravelar Veloplasty (IVV)

Critical adjunctive step in any palatoplasty. Technique: levator veli palatini muscles dissected free from their abnormal insertions on the posterior hard palate. Muscles retrodisplaced into a transverse orientation and sutured to each other across the midline. Reconstructs the functional levator sling.

Should be performed with every palatoplasty regardless of primary technique. Originally described by Kriens; considered essential for optimal speech outcomes.

<image>Surgical illustration of the Furlow double-opposing Z-plasty palatoplasty, shown in three panels. Panel A: inferior (oral) view of the cleft soft palate with the Z-plasty design marked — the oral surface Z-plasty with a posteriorly based left myomucosal flap (containing levator muscle, shown in red) and an anteriorly based right mucosal-only flap. Panel B: the nasal surface Z-plasty (mirror image) with a posteriorly based right myomucosal flap and an anteriorly based left mucosal-only flap. Panel C: the completed repair after transposition of the Z-plasty flaps, showing the levator muscles now overlapping in the midline in a transverse sling configuration, with the resulting palatal lengthening demonstrated by comparing pre- and post-repair palate length. Labels identify the levator muscle within each flap, the direction of flap transposition, and the reconstructed levator sling.</image>

Key Surgical Principles

Nasal layer closure: critical first step; use vomer flaps (unilateral or bilateral) for hard palate nasal layer; turn mucoperiosteal flaps for soft palate nasal layer. Muscle dissection and repair: intravelar veloplasty is essential regardless of technique. Oral layer closure: tension-free approximation of mucoperiosteal flaps. Greater palatine artery preservation: protect during flap elevation; can be delivered from its foramen by fracturing the bony canal.

Raw areas: exposed bone on the hard palate from flap elevation will granulate and re-epithelialize; concern for growth restriction. Hamulus fracture: some surgeons fracture the pterygoid hamulus to release the tensor veli palatini and reduce lateral tension (controversial — may affect eustachian tube function).

Complications

Fistula

Most common complication: 5-15% reported (variable by technique and cleft width). Most common location: junction of hard and soft palate. Risk factors: wide clefts, tension on closure, surgeon experience, infection. Small asymptomatic fistulae may be observed. Symptomatic fistulae (nasal air escape, fluid regurgitation) require surgical repair with local flaps or tongue flap.

Velopharyngeal Insufficiency (VPI)

Occurs in 10-30% of patients after primary palatoplasty. Manifests as hypernasality, nasal air emission, compensatory articulation errors. Evaluation: perceptual speech assessment, nasometry, videofluoroscopy, nasopharyngoscopy. Treatment options: speech therapy, secondary surgery (pharyngeal flap, sphincter pharyngoplasty, Furlow revision).

Other Complications

Bleeding (greater palatine artery injury). Airway obstruction (edema, particularly in Pierre Robin patients). Wound infection/dehiscence. Maxillary growth restriction (long-term).

Clinical Pearls

Intravelar veloplasty is the single most important step in palatoplasty; failure to reconstruct the levator sling is the primary cause of VPI after cleft palate repair. The Furlow Z-plasty is ideal for soft palate clefts and VPI secondary to short palate, as it both lengthens the palate and reconstructs the levator sling in one operation. Wide complete clefts are best managed with the Bardach two-flap technique plus intravelar veloplasty; the Furlow technique alone is often insufficient for wide clefts.

Myringotomy tubes should be placed at the time of palatoplasty; chronic effusions are nearly universal and untreated conductive hearing loss impacts speech development. Timing matters: repair between 9-12 months optimizes the balance between speech development and maxillary growth; delayed repair beyond 18 months significantly worsens speech outcomes. Always counsel families that 10-30% of patients will require secondary speech surgery despite optimal primary repair.

References

• Furlow LT Jr. Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg. 1986;78(6):724-738.
• Bardach J, Salyer KE. Surgical Techniques in Cleft Lip and Palate. 2nd ed. Mosby; 1991.
• Sommerlad BC. A technique for cleft palate repair. Plast Reconstr Surg. 2003;112(6):1542-1548.
• Kirschner RE, Wang P, Gaughan JP, et al. Cleft-palate repair by modified Furlow double-opposing Z-plasty. Plast Reconstr Surg. 1999;104(7):2103-2114.
• Smith DM, Vecchione L, Jiang S, et al. The Pittsburgh Fistula Classification System: a standardized scheme for the description of palatal fistulas. Cleft Palate Craniofac J. 2007;44(6):590-594.
• Losee JE, Kirschner RE. Comprehensive Cleft Care. 2nd ed. CRC Press; 2015.

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