Secondary cleft deformities and surgery are crucial aspects of long-term care for individuals born with cleft lip and/or palate. These deformities, which can affect the lip, nose, palate, and associated structures, arise for various reasons, including the initial severity of the cleft, primary surgical techniques, individual growth patterns, and scarring. Understanding the multifactorial causes and available treatment options is essential for achieving the best possible functional and aesthetic outcomes, significantly improving a patient's quality of life.

Understanding Secondary Cleft Deformities: Causes and Impact

Secondary deformities after primary cleft repair are common, often presenting a complex challenge for surgeons and patients. Unlike the initial, more uniform cleft, secondary issues are highly varied in their appearance and underlying causes. This section summarizes why these problems develop and their long-term implications.

Why Secondary Deformities Occur

The development of secondary cleft deformities is multifactorial. Key contributing factors include the type and severity of the original cleft, the techniques used in the primary surgical repair, and the surgeon's expertise. Technical errors or poor technique selection during the initial repair can directly lead to secondary issues.

Additionally, factors like individual scarring responses, genetic predisposition, and ethnic heritage can profoundly impact aesthetic outcomes. Scar contraction can distort the delicate anatomical landmarks of the lip and nose, while excessive scar tissue can create unsightly irregularities in contour and color.

The "Fourth Dimension": Growth and Scarring

Growth is often referred to as the "fourth dimension" in cleft lip and palate surgery. The human face undergoes rapid growth phases from birth to age 5 and again during puberty, with final osseocartilaginous facial growth concluding with puberty's cessation. Since primary cleft lip surgery typically occurs within the first year of life, these subsequent periods of dramatic growth, combined with scar maturation, profoundly affect the final result.

Early repair is critical for functional development and psychosocial benefits, even though delaying definitive repair might minimize bony growth disturbance. Unrestrained growth of cleft margins can actually lead to more severe secondary deformities if not addressed early. Scar maturation itself can lead to color and contour abnormalities, distortion of adjacent mobile landmarks, and shortening of lip dimensions.

Early Interventions: Minimizing Secondary Cleft Deformity Sequelae

While some secondary changes are inevitable, proactive steps can significantly reduce their severity. Early intervention focuses on scar management and timely assessment for further surgical needs.

Scar Management and Sun Protection

Parents play a vital role in minimizing scar severity. Advising massage with lotions like vitamin E, cocoa butter, zinc, or Mederma, along with strict sun protection for at least a year, helps prevent persistent hyperpigmentation. These interventions not only improve scars but also psychologically involve the family in the child's care. Silicone sheeting and hypoallergenic taping may also be beneficial, though maintaining them on a child's upper lip can be challenging.

If there's evidence of hypertrophic or keloid scarring, or if the patient is high-risk, intralesional steroid injections may be initiated. These acute interventions are crucial for optimal conservative care.

When to Consider Surgical Revision for Scars

If a scar remains problematic after optimal conservative care and a minimum of 12-18 months has been allowed for scar maturation, surgical revision may be considered. Several surgical options exist, tailored to the scar's characteristics. Dermabrasion is an option for elevated scars. For hypertrophic and significantly widened scars, excision in a diamond or ellipse shape facilitates a straight-line closure. If a scar is depressed or overlies a philtral column, skin-only excision with a vest-over-pants closure or other bulking techniques can be helpful.

Evaluating Secondary Cleft Deformities: A Systematic Approach

A thorough evaluation is essential for planning secondary cleft surgery. This involves assessing functional impairments, aesthetic concerns, and the patient's maturity.

Patient Assessment and Timing of Surgery

Secondary deformity management is an integral part of treating cleft lip and/or palate. Patients often require at least one nose and lip revision by adolescence, with many needing multiple interventions. Proper timing of intervention addresses functional impairments and aesthetic deformities that could impact psychosocial development.

Around age 6, the risk of peer teasing increases substantially. Therefore, all patients should be evaluated for secondary surgery at age 4 or 5, before kindergarten. Decisions on timing balance problem severity, function, appearance, and the patient's emotional and physical maturity. As children become adolescents, their opinions on appearance should play a significant role in surgical decision-making.

Categorizing Lip and Nasal Deformities

When evaluating secondary deformities, a systematic framework is helpful. Deformities are typically categorized into five broad areas: scarring, lip, vermillion, muscle, and buccal sulcus. Scarring is assessed for immaturity (redness, firmness), contour changes, and distortion of local structures. Lip and nasal deformities require detailed examination.

Specific Deformities to Look For

Lip deformities include abnormal dimensions or landmark distortions of the philtral columns, cupid's bow, and lateral lip segments. Examples are a short lip (cleft-side philtral column vertically shorter), a long lip (cleft-side philtral column vertically longer), tight upper lip (decreased width or anteroposterior projection disparity), wide upper lip (increased horizontal width), short lateral lip, philtral column distortion, and cupid's bow distortion.

Vermillion deformities involve thin or thick lip segments, mismatches between wet and dry vermillion, notching or border malalignment (white roll and vermillion), and the whistle deformity (median tubercle paucity leading to non-apposition of lips at rest).

Muscle deformities may stem from inadequate muscle reapproximation or dehiscence during primary repair. Orbicularis oris dehiscence appears as bulging with animation or a short, widened lip scar. Incomplete dissection of aberrant muscular attachments can lead to subtle deformities like tethering of the nasal ala. The importance of creating a nasal floor and nostril sill during primary repair is also emphasized for lasting results.

Buccal sulcus deformities occur when the lip is tethered to the maxilla due to scar contracture or tissue paucity, affecting lip freedom and movement.

Surgical Solutions for Lip Deformities After Cleft Repair

Correcting secondary lip deformities requires a nuanced approach, often involving complex revisions tailored to the specific issue.

Addressing a Short Lip Deformity

A vertically short lip can result from scar contraction, a primary deficiency of prolabial soft tissues, or inadequate primary rotation and advancement. Mild shortening due to scar contraction may improve over time with scar management. If the deformity persists beyond one year or scar maturation, surgical intervention is considered.

Minor Deformities (< 2–3 mm): May be treated with a diamond-shaped scar excision or a unilimb Z-plasty to lengthen the philtrum.
Greater Shortening (> 2–3 mm): Standard Z-plasty can lengthen the philtral column, but it adds more scars. For significant shortening or if Z-plasty scars are undesirable, the entire repair may need to be taken down and repeated.

When a short philtral column results from under-rotation of the flap (often due to minimizing the backcut), it typically causes vermillion notching and a vertically oriented, non-continuous white roll. A slightly larger backcut/scar (even onto the columella) may be needed to achieve necessary rotation.

Correcting a Long Lip

This deformity is rare but can occur from over-rotation of the medial lip segment. Simply excising lip tissue beneath the alar base to "hitch up" the elongated side is often ineffective due to orbicularis action and gravity. It is generally preferable to take down the entire repair and excise tissue in all dimensions for correction.

Managing a Tight or Wide Lip

Horizontally Tight Lip: Options include fat injections to the upper lip and reduction of the lower lip via a wedge excision to reduce bulk disparity. More significant deformities are best treated with the Abbé flap, a two-stage procedure transferring full-thickness lower lip elements to the upper lip. This is particularly useful for decreased upper lip projection, excessive scarring of the central aesthetic unit, or a significantly narrowed/shortened central unit. It provides a pseudodimple for the philtrum and continuity of surface landmarks, but has donor site morbidity and patient discomfort.
Horizontally Wide Lip: Often seen in bilateral clefts where the prolabial philtral segment was designed too wide, or due to persistent tension from a protruding maxilla or orbicularis muscle function. The solution is excision of excess philtral tissue, with meticulous approximation of the orbicularis oris and philtral landmarks. The philtrum should be made slightly smaller than desired in anticipation of stretching.

Philtral Column Distortion and Short Lateral Lip

A decrease in the horizontal width of the lateral upper lip segment is common after achieving an optimal cupid's bow. While traditionally thought to stretch, this is often not the case; intact central landmarks and good lip contour are more important than lateral lip segment symmetry. Philtral column distortion can be due to excessive scarring, short length, or lack of prominence. Methods include fat grafts (free or dermal) or a "vest-over-pants" closure which buries underlying dermis to add bulk.

Remodeling the Vermillion

Vermillion deformities address the aesthetic and functional aspects of the lip's red portion.

Thin Lip: Goals include increasing vermillion show, improving anteroposterior projection, and replacing contour landmarks (e.g., the break point above the white roll, prominent median tubercle). Fat grafting (free fat injections or dermal fat graft) is an excellent option for patients with adequate vermillion but a paucity of volume. Fat grafting can also improve skin quality in scarred areas. For severe deficiency, especially in bilateral clefts, an Abbé flap is often the best solution.
Thick Lip: Can result from inadequate rotation advancement, excess tissue, or insufficient gingivobuccal sulcus depth. If due to relative excess adjacent to a paucity, the lateral lip element should be redistributed. If it exists alone, direct excision via an inner lip incision can be performed. Recreation and deepening of the gingivobuccal sulcus can correct a thick lip appearance caused by inadequate sulcus creation.
Vermillion Mismatch: Abnormal exposure of wet vermillion is aesthetically displeasing and prone to dryness. If the mismatch involves the median tubercle, excision with medialization of adjacent undermined vermillion flaps can close the defect, realigning the wet-dry border. Prevention during primary repair (marking the wet-dry junction, designing a dry vermillion triangle, half Z-plasty) is ideal.
Vermillion Notching/Border Malalignment: Similar to short-lip management, this involves scar management, diamond excisions, Z-plasties, and local tissue rearrangement. For significant notching, repeating the entire repair may be necessary.
Whistle Deformity: Caused by scar contracture across the vermillion, failure to fill the central tubercle, or orbicularis muscle diastasis. Treatment depends on the underlying cause, using local advancement flaps, fat grafting, autologous grafts, or the Abbé flap. Incisions crossing the wet-dry vermillion border should be Z-plasties, W-plasties, or lazy-S to prevent future contracture.

Correcting Orbicularis Muscle Deformities

Continuity of the orbicularis muscle is vital for lip appearance and function. In cleft lip, the muscle is often discontinuous and aberrantly inserted. Failure to adequately reconstruct the orbicularis during primary repair leads to continued attachments and worsening nasal deformities. In secondary operations, this generally involves reopening the repair, dissecting the orbicularis, and suture reapproximation. Complete and wide take-down of orbicularis and nasalis musculature from abnormal insertion points is crucial for optimal results.

Managing Buccal Sulcus Deformities After Cleft Repair

Deformities of the superior gingivobuccal sulcus can involve excess or deficit of tissue.

Excess Tissue: Simple excision and re-tacking the mucosa to the nasal spine or periosteum near the columellar-lip junction.
Deficiencies: Most common after bilateral cleft lip repair, reflecting underlying anatomy. Techniques include local flaps, mucosal grafts, and split-thickness or full-thickness skin grafts. A common method uses an inferiorly based mucosal upper lip flap, dissecting the prolabium off the premaxilla, and rotating the flap into the columellar-lip junction region. Buccal mucosa is the first choice for graft material, often used with a dental amalgam stent to maximize graft take.

Palatal Fistulas: Diagnosis and Treatment Options

Palatal fistulas are significant complications following cleft palate repair, ranging from asymptomatic to symptomatic.

Evaluating and Timing Palatal Fistula Repair

Palatal fistulas present with varied clinical symptoms, including nasal air emission, hypernasal speech, and nasal regurgitation of fluid and food. Symptomatic fistulas are bothersome, posing hygiene problems and infection risks. Interestingly, fistula size doesn't always correlate with clinical severity.

The Pittsburgh Classification Scheme categorizes fistulas by anatomic location and designates whether they are symptomatic. Evaluation involves questioning patients/families about nasal regurgitation, malodorous nasal smell, recurrent sinus infections, and hypernasal speech. A complete exam of the hard and soft palates using a tongue blade and light source is performed. Symptomatic fistulas typically require surgical repair; asymptomatic ones can be addressed if other procedures are planned.

Surgical Techniques for Palatal Fistula Closure

Fistula closure requires a two-layered repair (nasal and oral layers). These layers are usually formed using local random-pattern flaps from adjacent tissue, which can be challenging due to scarred and tenuous surrounding tissues.

Nasal Layer Closure: Can be accomplished with turnover flaps from fistula lining mucosa, mucoperiosteal vomer flaps, or mucosa from the posterior pharyngeal wall (for posterior fistulas – also helpful for velopharyngeal insufficiency).
Oral Layer Closure: Local palatal mucoperiosteum is used, with flaps designed much larger than the defect due to inelastic scarred mucosa. Minimal tension on flaps is critical; osteotomizing the greater palatine foramen or islandization of hemi-palatal flaps can release tension. Overlapping nasal and oral layer suture lines should be avoided.
Biomaterials: Acellular dermis (e.g., Alloderm) can augment repair by sandwiching a thin sheet between recreated nasal and oral mucosa layers, reducing recurrence rates.
Regional Axial Pattern Flaps: For situations where tension-free repair isn't possible with palatal tissue. Intraoral options include buccal mucosa flaps (e.g., facial artery myomucosal (FAMM) flap) or tongue flaps. The FAMM flap can be designed long and folded for a two-layered structure. Tongue flaps provide a robust vascular plexus but lead to tethering, poor match in texture/color, and potential articulation changes. The temporoparietal flap is a more remote option for large defects in adults.
Microvascular Tissue Transfer: For large, recalcitrant fistulas, especially anteriorly located, the radial forearm flap is preferred due to its long pedicle, pliability, and ability to form both nasal and oral layers. Other options include foot or arm fasciocutaneous flaps. Prosthetic obturation is also a consideration.
Alveolar Bone Graft Failure: If a persistent nasoalveolar fistula occurs, bone grafting can be reattempted, or dento-osseous transport distraction osteogenesis can be performed.

Secondary Cleft Nose Deformities and Rhinoplasty

Secondary cleft nose deformities are a common consequence of cleft lip. Addressing these requires a comprehensive understanding of nasal anatomy and advanced surgical techniques.

Cleft Nose Anatomy and Evaluation

The nose is a three-layered structure (cutaneous, osseocartilaginous, mucoperichondrium). The osseocartilaginous layer (upper, middle, lower vaults) provides structural features. The tripod theory helps understand nasal tip dynamics. Cleft nose anatomy involves abnormalities of the osseocartilaginous skeleton, including a webbed alar rim, flaring alae, wide nostril, short columella, and obtuse angles, contributing to nasal airflow obstruction.

Evaluation involves a thorough history (especially regarding nasal airway obstruction) and physical exam from frontal, profile, worm's eye/basilar, and internal views. Underlying skeletal/midface hypoplasia must be assessed. The examination systematically covers skin thickness, nasal bones, middle vault symmetry, ULC collapse, nasal tip morphology, columella height/width, alar height/width/position, nasal sill, pyriform rim, radix depth, dorsal hump, nasolabial angle, alar rim retraction, and internal nasal structures for deviation or hypertrophy.

Timing of Nasal Surgery

Decision-making for secondary cleft nose surgery depends on deformity severity, patient/family wishes, and optimal timing. Revisionary procedures before preschool (age 4 or 5) focus on reshaping and repositioning the cleft-sided ala. Modifications to facial/nasal bones and septum are typically delayed until nasal growth concludes (age 11-12 in females, 13-14 in males) to prevent disruption of growth centers. If orthognathic surgery is needed for midface hypoplasia, it should precede definitive rhinoplasty. Patients must also be emotionally mature for decision-making and postoperative care.

Surgical Techniques: Access, Sutures, and Grafts

Correcting secondary cleft nose deformities aims to achieve nasal tip projection, bony dorsum projection, correct alar base position, contour to the alar rim/lateral crus, an acute alar facial relationship, an adequate nasolabial angle, and nasal airway patency.

Access: Both closed (incisions within the nostril) and open (columellar incision) approaches are used. The closed approach is minimally invasive, while the open approach provides superior visualization for difficult cases, especially tip work, though it involves external scarring and prolonged swelling.
Suture Techniques: Sutures manipulate the cartilaginous and soft-tissue framework. Examples include: cephalic trim (removes ULC-LLC attachments), transdomal sutures (narrows nasal tip), interdomal sutures (provides symmetry and reduces interdomal width), lateral crural mattress sutures (modifies lateral crus curvature and nostril flare), and columellar septal sutures (adjusts tip height). The Stal–Feldman alar facial rotation excision reshapes the nostril, eliminates excess ala, creates a full nostril sill, and narrows the alar base, combining excision with cinching sutures.
Supporting Grafts: When suture modification is inadequate, cartilage grafts provide additional support.
Columellar Struts: Placed between medial crura to support nasal tip projection, columellar length, and nasolabial angle.
Spreader Grafts: Placed between dorsal septum and ULC to restore internal nasal valve, straighten deviated septum, improve dorsal aesthetic lines, and reconstruct open-roof deformities.
Septal Extension/Extended Spreader Grafts: Control nasal tip projection, support, shape, rotation, and create a supratip break.
Tip Grafts/Shield Grafts: Camouflage tip irregularities and add projection. Shield grafts, though popular, can lead to long-term inadequacy.
Alar Batten Grafts: Provide extra-anatomic support along the alar rim to open the external nasal valve and prevent alar notching. Lateral crural strut grafts are placed underneath the LLC for structural support.
Dorsal Onlay Grafts: Manipulate nasal dorsum appearance using cartilage (diced or intact) or allogenous materials (e.g., Alloderm for minor contours, costal cartilage for significant augmentation). Diced onlay grafts are safe and predictable for moderate deformities.
Graft Materials: Autologous materials (septal, auricular, rib cartilage) are preferred for biocompatibility and low risk of infection, despite donor site morbidity and resorption. Bony osteotomies address widened nasal bones and abnormal dorsal lines.

When "Enough is Enough": Balancing Intervention and Well-being

Patients with cleft lip and palate can present with a formidable array of secondary deformities. When deciding on surgical intervention, it's crucial to assess the impact on the patient and the likelihood of substantial improvement. At some point, further surgery may not significantly enhance appearance or function.

While secondary deformities can have a negative psychosocial impact, patients often exhibit resilience and positive coping strategies. Surgery for secondary deformities should be reserved for problems that bother the patient and are genuinely likely to improve with intervention. The goal is to avoid unnecessary procedures, recognizing that "the enemy of good is better," especially when "better" is uncertain.

Secondary Cleft Deformities and Surgery FAQ

What causes secondary cleft deformities after initial repair?

Secondary cleft deformities are caused by a combination of factors including the initial severity of the cleft, the primary surgical techniques used, individual genetic predisposition to scarring, and the natural growth and maturation of facial structures over time. Technical errors during the first surgery can also contribute.

How important is scar management for secondary cleft deformities?

Scar management is extremely important! Early and consistent interventions like scar massage with lotion (vitamin E, cocoa butter) and rigorous sun protection for at least a year can significantly minimize the severity of color and contour abnormalities. This not only improves aesthetic outcomes but also involves the family in the child's care, offering psychological benefits.

When is the best time for secondary cleft lip or nose surgery?

For secondary cleft lip and nose deformities, evaluation typically occurs at age 4 or 5, before kindergarten, to address aesthetic and functional concerns that could impact psychosocial development. Definitive rhinoplasty and major bone modifications for nasal deformities are usually delayed until nasal growth is complete, around 11-12 for females and 13-14 for males, and after any necessary orthognathic surgery.

What are some common lip deformities that require secondary surgery?

Common lip deformities requiring secondary surgery include a vertically short or long lip, a horizontally tight or wide upper lip, distortion of the philtral column or cupid's bow, thin or thick vermillion, vermillion mismatches, and the "whistle deformity" where the median tubercle is lacking, preventing full lip closure.

What surgical techniques are used to reshape the nose in secondary cleft rhinoplasty?

Secondary cleft rhinoplasty utilizes various techniques, including suture manipulation (e.g., transdomal, interdomal sutures to reshape cartilage), and supportive cartilage grafts (e.g., columellar struts, spreader grafts, alar batten grafts) to improve tip projection, alar base position, and overall nasal symmetry. Access can be through closed or open approaches, depending on the complexity.

Secondary Cleft Deformities and Surgery