# Secondary Cleft Deformities and Velopharyngeal Insufficiency

## Introduction
Despite optimal primary repair, most cleft patients require secondary procedures during growth and development. Secondary deformities include residual nasal asymmetry, lip revision, palatal fistula, alveolar cleft, velopharyngeal insufficiency (VPI), and maxillary hypoplasia. Multidisciplinary team management spans from infancy through skeletal maturity. Coordinated timing of interventions is critical to avoid disrupting facial growth while optimizing function.

## Alveolar Bone Grafting

### Rationale
Alveolar cleft leaves a bony gap in the dental arch. Bone grafting provides: continuity of the dental arch, bone for eruption of the canine tooth, support for alar base, closure of oronasal fistula.

### Timing
**Secondary alveolar bone grafting (SABG)**: 8-11 years of age. Timed to mixed dentition phase, before canine eruption (when 1/2 to 2/3 of the canine root is formed). Too early: potential growth restriction of the maxilla. Too late: canine erupts ectopically, may lose the tooth.

### Technique
Cancellous bone graft from the iliac crest (anterior approach, gold standard donor site). Alternative donors: tibial plateau, calvarial bone, mandibular symphysis (for smaller defects). Surgical steps: 1. Raise mucoperiosteal flaps to expose the cleft.

2. Close the nasal floor/layer. 3. Pack cancellous bone chips into the alveolar defect. 4. Close the oral mucoperiosteum with tension-free layered closure. BMP-2 (rhBMP-2) as an alternative to autogenous bone: promising results but off-label, cost concerns, inconsistent evidence in children.

### Outcomes
Success rate >90% with iliac crest graft. Canine eruption through grafted bone in 75-90% of patients. Complications: graft resorption, donor site morbidity (pain, contour deformity, lateral femoral cutaneous nerve injury), infection, fistula recurrence.

## Secondary Lip Revision

### Indications
Vermilion notching (whistle deformity). Scar hypertrophy or widening. Lip length discrepancy (short lip on cleft side). Muscle deficiency (visible depression or bulge asymmetry on animation). Cupid's bow asymmetry. Tight upper lip with insufficient vermilion show.

### Timing
Minor revisions: preschool age (4-5 years) to minimize social impact. Major revisions: may wait until skeletal maturity. Intermediate revisions as needed for functional concerns.

### Techniques
Scar revision: excision with or without Z-plasty/W-plasty. Vermilion augmentation: cross-lip mucosal flap (Abbe flap), V-Y advancement, fat grafting. Orbicularis oris muscle re-repair. Full-thickness lip revision with re-execution of primary repair principles.

Abbe flap: cross-lip arterial pedicle flap from the lower lip to reconstruct central upper lip deficiency, particularly useful in bilateral cleft lip revision.

## Secondary Cleft Rhinoplasty

### Timing
Intermediate rhinoplasty: 4-6 years if severe deformity impacting social development. Definitive rhinoplasty: after skeletal maturity (age 16-18). Septoplasty can be performed earlier if significant nasal obstruction exists.

### Key Deformities Addressed
Deviated nasal septum (caudal and cartilaginous). Lower lateral cartilage asymmetry (flattened, displaced on cleft side). Short columella (especially bilateral cleft). Wide alar base on cleft side. Deficient alar rim/nostril asymmetry. Dorsal deviation and irregularity.

### Techniques
Open rhinoplasty approach (preferred for cleft rhinoplasty). Septoplasty with cartilage harvest for grafting. Lower lateral cartilage repositioning, suture techniques, and cartilage grafts. Columellar strut, tip grafts, alar batten grafts.

Alar base repositioning (cinch suture, wedge excision). Dorsal onlay grafting (septal, auricular, or rib cartilage). Rib cartilage grafting for severe deformities with cartilage deficiency. Bilateral cleft: columella lengthening, forked flap revision, V-Y columellar advancement.

<image>Illustration showing the staged management of cleft-associated deformities on a timeline from birth to adulthood. The timeline shows: primary lip repair at 3-6 months, primary palatoplasty at 9-12 months, speech assessment at 3-4 years, secondary alveolar bone grafting at 8-11 years with an inset showing cancellous iliac crest bone packed into the alveolar cleft, orthodontic treatment spanning ages 7-18, definitive rhinoplasty at age 16-18 with an inset showing open rhinoplasty with cartilage graft placement for lower lateral cartilage repositioning, and orthognathic surgery (Le Fort I) at age 17-19 if needed. Each intervention is labeled with its purpose and key surgical details.</image>

## Velopharyngeal Insufficiency (VPI)

### Definition
Inability to achieve adequate closure between the soft palate and posterior/lateral pharyngeal walls during speech and swallowing. Results in hypernasality, nasal air emission, nasal regurgitation, and compensatory articulation patterns. Occurs in 10-30% of cleft palate patients after primary repair.

### Evaluation
**Perceptual speech assessment**: gold standard for identifying VPI; performed by experienced speech-language pathologist. Hypernasality rating. Nasal air emission (audible vs. inaudible). Compensatory articulation errors (glottal stops, pharyngeal fricatives).

**Nasometry**: objective measurement of nasalance (ratio of nasal to total acoustic energy). Normal nasalance: <32% on oral passages. Elevated nasalance indicates VPI. **Videofluoroscopy (multiview)**: lateral and frontal views show velar elevation, pharyngeal wall movement, and closure pattern.

**Nasopharyngoscopy (flexible fiberoptic)**: direct visualization of velopharyngeal port during speech. Determines closure pattern: coronal, sagittal, circular, circular with Passavant ridge. Guides surgical planning (closure pattern determines optimal procedure). **MRI**: dynamic assessment of velopharyngeal motion; radiation-free alternative.

### Conservative Management
Speech therapy alone is effective only for compensatory articulation errors, NOT for structural VPI. Speech therapy should accompany but not replace surgical correction of VPI. Prosthetic speech appliance (palatal lift or speech bulb obturator): temporizing measure or for patients who are not surgical candidates.

### Surgical Management

| Procedure | Indication (Closure Pattern) | Mechanism | Success Rate | Key Complication |
|-----------|------------------------------|-----------|-------------|-----------------|
| Pharyngeal Flap | Coronal/circular with good lateral wall motion | Central partition; speech through lateral ports | 85-95% | OSA (10-20%) |
| Sphincter Pharyngoplasty | Sagittal with poor lateral wall motion | Dynamic sphincter from bilateral palatopharyngeus flaps | 80-90% | OSA, pharyngeal stenosis |
| Furlow Z-Plasty (revision) | Short palate with adequate lateral wall motion | Palatal lengthening + levator sling reconstruction | 70-85% | Dehiscence |
| Fat Injection | Small central gap, marginal VPI | Bulk augmentation of posterior pharyngeal wall | Variable | Requires repeat injections |

#### Pharyngeal Flap
**Indication**: coronal or circular closure pattern with good lateral wall motion. **Technique**: superiorly based myomucosal flap from the posterior pharyngeal wall inset into the soft palate. Creates a central partition; speech occurs through lateral ports. Flap width tailored to velopharyngeal gap size.

**Advantages**: reliable reduction of hypernasality, high success rate (85-95%). **Complications**: obstructive sleep apnea (10-20%), hyponasality, snoring, flap dehiscence. **Contraindication**: 22q11.2 deletion with medially displaced internal carotid arteries (risk of life-threatening hemorrhage).

#### Sphincter Pharyngoplasty
**Indication**: sagittal closure pattern with poor lateral wall motion. **Technique**: bilateral superiorly based palatopharyngeus myomucosal flaps (from posterior tonsillar pillars) inset into the posterior pharyngeal wall, overlapping to create a dynamic sphincter. Reduces the velopharyngeal port size centrally; remaining port allows nasal breathing. **Advantages**: dynamic mechanism, may reduce OSA risk compared to wide pharyngeal flaps. **Complications**: OSA, hyponasality, flap dehiscence, pharyngeal stenosis (rare).

#### Furlow Z-Plasty (Revision or Primary VPI)
**Indication**: short palate with adequate lateral wall motion; submucous cleft with VPI. Lengthens the palate and reconstructs the levator sling. Particularly effective for submucous cleft palate with VPI. Can be performed as revision after failed initial Furlow or after other palatoplasty techniques.

#### Fat Injection / Autologous Fat Grafting
Injection into posterior pharyngeal wall to reduce gap. Minimally invasive option for small residual gaps. Variable results; may require repeated injections. Best for small central gaps in marginal VPI.

## Maxillary Hypoplasia and Orthognathic Surgery

### Pathophysiology
Midface growth restriction occurs in 25-50% of cleft patients. Multifactorial: intrinsic growth deficiency, surgical scarring from palatoplasty, lack of functional muscular forces. Results in Class III malocclusion, concave facial profile, negative overjet.

### Evaluation
Cephalometric analysis: SNA angle, ANB relationship. Clinical: midface retrusion, negative overjet, Class III dental relationship, short upper lip. Dental models and virtual surgical planning.

### Treatment
**Le Fort I osteotomy**: maxillary advancement (most common). Performed after skeletal maturity (age 17-19 in males, 16-17 in females). Requires pre-surgical orthodontics (12-18 months). Advancement of 4-10+ mm typical in cleft patients.

Rigid fixation with plates and screws. Relapse rate higher in cleft patients than non-cleft (consider overcorrection). **Distraction osteogenesis**: for large advancements (>10 mm), reduces relapse. Internal or external distraction devices.

Le Fort I osteotomy followed by gradual distraction (1 mm/day). Consolidation phase (6-8 weeks). **Combined Le Fort I + mandibular surgery**: if mandibular asymmetry or excess also present.

## Clinical Pearls
Alveolar bone grafting should be timed to the erupting canine; obtain a panoramic radiograph to assess canine root development and plan surgery when 1/2 to 2/3 of the root is formed. Closure pattern on nasopharyngoscopy dictates the choice between pharyngeal flap (coronal pattern) and sphincter pharyngoplasty (sagittal pattern); performing the wrong procedure for the closure pattern leads to suboptimal outcomes. Always screen 22q11.2 deletion patients for medially displaced internal carotid arteries before any pharyngoplasty; this is potentially life-threatening. Secondary rhinoplasty in cleft patients is one of the most challenging rhinoplasty operations; the open approach with rib cartilage grafting is often necessary due to septal cartilage deficiency. Le Fort I advancement in cleft patients has a higher relapse rate than in non-cleft patients; plan for 15-20% overcorrection or consider distraction osteogenesis for large movements.

## References
- Bergland O, Semb G, Abyholm FE. Elimination of the residual alveolar cleft by secondary bone grafting and subsequent orthodontic treatment. Cleft Palate J. 1986;23(3):175-205.
- Losee JE, Smith DM. Cleft palate repair. In: Comprehensive Cleft Care. 2nd ed. CRC Press; 2015.
- Mehendale FV, Birch MJ, Birkett L, et al. Surgical management of velopharyngeal insufficiency in velocardiofacial syndrome. Cleft Palate Craniofac J. 2004;41(2):124-135.
- Ysunza A, Pamplona MC, Mendoza M, et al. Surgical treatment of submucous cleft palate: a comparative trial of two modalities for the treatment of velopharyngeal insufficiency. Plast Reconstr Surg. 2001;107(1):9-14.
- Posnick JC. Orthognathic Surgery: Principles, Planning and Practice. Elsevier; 2014.
- Tse R. Unilateral cleft lip: principles and practice of surgical management. Semin Plast Surg. 2012;26(4):145-155.