Bilateral cleft lip and nose repair is a complex surgical procedure that has seen significant advancements over the past half-century. Once considered twice as difficult to repair as a unilateral cleft, with often less satisfactory results, modern techniques aim for outcomes comparable to, or even surpassing, those of unilateral repairs. This comprehensive guide will break down the essential aspects, historical context, and current approaches to bilateral cleft lip and nose repair, providing students with a clear understanding of this vital field.

Understanding Bilateral Cleft Lip and Nose Repair

A bilateral cleft lip and nose repair involves correcting deformities on both sides of the lip and nose. The primary goal is to restore normal appearance and function for children born with this condition. Two major advancements have revolutionized management: the need for preoperative manipulation of the protuberant premaxilla and the acceptance of principles for synchronous correction of the nasal deformity alongside labial repair.

Core Principles Guiding Bilateral Cleft Repair

Modern surgical approaches are built on established principles that ensure the best possible outcomes, even as specific techniques evolve. These include:

Maintain Nasolabial Symmetry: Even minor differences between the two sides of the lip and nose can become more noticeable with growth.
Secure Muscular Continuity: Creating a complete oral muscle ring improves labial function, eliminates lateral bulges, and prevents later distortions of the philtrum and interalar widening.
Design Proper Philtral Size and Shape: The philtrum rapidly elongates and widens, particularly at the columellar-labial junction, requiring careful planning for growth.
Construct Median Tubercle from Lateral Labial Elements: The prolabium lacks a white roll and its vermilion doesn't grow to full height, so the median tubercle is best formed using lateral vermilion-mucosal elements.
Position and Sculpt Lower Lateral Cartilages: Correcting the slumped or splayed lower lateral cartilages and removing excess soft tissue in the nasal tip and columella is crucial for normal nasal projection and columellar length and width.

These principles have fundamentally changed the surgical strategy, particularly recognizing that the "absent columella" is often an illusion, and its components are simply malpositioned within the nasal tip.

Historical Perspective: Evolution of Techniques

The journey to modern bilateral cleft repair is marked by various attempts to address the challenging deformities. Understanding this history highlights the breakthroughs that led to current best practices.

The Challenge of the Premaxilla

The protruding premaxilla has historically been a major obstacle. Early aggressive surgeons sometimes resected this bone, often with poor results. Gustav Simon introduced paring the prolabium edges and creating bilateral labial adhesions, a method sometimes linked to the "Simonart's bands" eponym. Premaxillary "set-back" by vomerine resection was practiced into the 20th century. Later, clumsy headcaps and compressive devices emerged, foreshadowing today's passive and active dentofacial orthopedics.

Early Labial Closure Techniques

Initial techniques adapted unilateral repair methods, often recommending staged bilateral repair (one side, then the other), which predictably resulted in asymmetry. Surgeons believed the prolabium lacked growth potential, leading to techniques using rectangular or triangular lateral labial flaps inserted beneath it, often creating unnaturally long lips or peculiar geometric scars. It was later recognized that the philtrum has a remarkable capacity for vertical growth once attached to lateral labial elements.

A long-standing debate existed on forming the median tubercle. Preserving prolabial vermilion often led to a "whistling lip deformity" with a flat upper lip and deficient tubercle. This was often worsened by the "cleft lip lower lip deformity" as the child struggled for bilabial closure over a protrusive premaxilla.

The Overlooked Orbicularis Oris Muscle

For many years, little attention was given to closing the orbicularis oris muscle. Some surgeons feared it would inhibit premaxillary growth. However, without muscular continuity, patients would develop bulges when puckering and be unable to whistle. Primary muscular closure is now considered essential to minimize distortions like lateral drift of alar bases and philtral widening.

Neglect of the Nasal Deformity

Historically, surgeons focused solely on labial closure, deferring nasal correction due to fears of compromising blood supply or interfering with growth, or simply being intimidated by the "short columella." This often worsened the "bilateral cleft lip nasal deformity," characterized by a broad nasal tip, dislocated lower lateral cartilages, flared alae nasi, and increased alar elevation when smiling.

Advances in Nasal Correction

Over time, the understanding of the nasal deformity evolved, leading to a shift from secondary to primary nasal correction.

Limitations of Secondary Nasal Correction

Traditional teaching held that the columella was deficient. Numerous secondary procedures were devised to lengthen the "short columella." The Cronin method (1958) used bipedicled strap flaps from nostril sills, yielding modest and often temporary length. Millard's forked flap procedure (also 1958) was popular but caused its own tertiary distortions, including scars across the columellar-labial junction, a circumferential philtral scar, and an unusually appearing nose (rectangular columella, sharp angle, elongated nostrils, downward drift of base). These issues led surgeons to question whether labial skin truly belonged in the columella and whether the columella was actually deficient.

The Rise of Primary Nasal Repair

Dissections revealed that the columella only appeared short due to malpositioned lower lateral cartilages; the tissue was present but splayed. Broadbent and Woolf described primary medial advancement of alar domes. McComb pioneered primary columellar elongation, initially with a two-stage method involving an external incision ("flying bird") to appose and suspend splayed genua, and later abandoning forked flaps and vertical incisions. He demonstrated that normal nasal tip projection and columellar length could be achieved by apposition of the genua, elevation and fixation of lower lateral cartilages, and sculpting soft tissues.

Other surgeons, like Trott and Mohan, developed single-stage nasolabial repairs using open rhinoplasty. Cutting and associates used an open-tip approach following presurgical stretching. The key insight was that the columella's components are "unzipped" within the broad nasal tip, and correct anatomical positioning and redraping of existing tissues are the solution, not recruiting more tissue.

The Three- and Fourth-Dimensional Challenge

Surgeons must consider not just the immediate three-dimensional repair but also how features will change in the "fourth dimension"—over time with normal growth and abnormal alterations.

Growth Patterns: Farkas and colleagues documented normal nasolabial growth. Fast-growing features (nasal height/width, labial landmarks) reach over 75% of adult size by age 5. Slow-growing features (tip protrusion, columellar length) only reach two-thirds. This explains why fast-growing features can become overly long or wide (e.g., interalar distance, philtral length/width), while slow-growing features remain abnormally short after conventional repair.
Surgical Implications: Features programmed for rapid growth should be crafted on a smaller scale, while slow-growing features, like the median tubercle, should be fashioned as full as possible, anticipating insufficient growth.

Anatomical Forms and Presentation

Bilateral cleft lip presents in three main anatomical forms, each with varying degrees of severity and associated palatal clefting:

Bilateral Symmetrical Complete (50%): Complete clefts on both sides, almost always associated with a bilateral complete cleft of the secondary palate.
Bilateral Symmetrical Incomplete (25%): Lesser degrees of clefting, often with minor or absent notching of the alveolar ridge and an intact secondary palate.
Bilateral Asymmetrical (Complete/Incomplete) (25%): One side is complete, the other incomplete. The palate can also vary. Terminology for the lesser side needs refinement.

Lesser-Forms of Incomplete Cleft Lip

"Incomplete" cleft lip means there's cutaneous continuity between the medial and lateral processes. Yuzuriha and Mulliken further classified these lesser-forms:

Minor-Form: Cleft extends 3–5 mm above the normal Cupid's bow peak (50% or less of normal cutaneous labial height). Features include deficient vermilion, a cutaneous groove, muscular depression, hypoplastic median tubercle, and minor nasal deformity.
Microform: Notched vermilion-cutaneous junction with Cupid's bow peak elevated less than 3 mm. Similar features to minor-form but less obvious, with slight sill depression, slumped alar genu, and minor alar base displacement.
Mini-Microform: Disruption of the white roll without Cupid's bow peak elevation, usually with a notch of the free mucosal margin. Variable muscular depression and nasal deformity.

This detailed subcategorization helps guide operative strategy and predict likely revisions.

Preoperative Dentofacial Orthopedics

Aligning the three maxillary elements (two lateral segments and the premaxilla) is critical for successful synchronous bilateral nasolabial repair. This sets the skeletal stage for proper philtral flap design, anatomical nasal cartilage positioning, and alveolar cleft closure, which stabilizes the maxillary arch and reduces oronasal fistulas. Premaxillary retropositioning also minimizes distortions during childhood growth.

Two main strategies exist:

Passive Molding Plate (e.g., Nasoalveolar Molding - NAM): This plate maintains transverse width, with external forces (taping, elastic bands) retracting the premaxilla. It involves differential pressure on segments and later adds nasal molding prongs. NAM is labor-intensive and slow, and while effective for sagittal plane correction and rotation, it has little effect on vertical position. Potential complications include mucosal inflammation, blistering, feeding difficulties, and airway obstruction risk.
Active Dentofacial Orthopedic Device (e.g., Latham appliance): This device is surgically pinned to the maxillary shelves. Elastic chains connected to a transvomer wire, adjusted daily by parents, expand the anterior palatal segments and retrocline the premaxilla. It's effective for sagittal correction and rotation but has little vertical effect. Critics argue it can cause midfacial retrusion, though its impact beyond expected growth inhibition is debated.

Operative Techniques: A Detailed Overview

The day of repair is paramount. The surgeon must work slowly and deliberately. The sequence described below often shifts between lip and nose. Video resources are available for visual learners (not included in this text).

Markings for Bilateral Complete Cleft Lip and Palate

Using brilliant green dye and a sharpened toothpick, anatomical points are marked. The philtral flap length (sn-ls) is typically 6–7 mm (for 5–6 months old), and its width is 2 mm at the columellar-labial junction (cphs-cphs) and 3.5–4 mm between Cupid's bow peaks (cphi-cphi). Philtral sides are drawn slightly concave. De-epithelialized flanking flaps are designed to lie beneath lateral labial flaps, simulating columns and adding vascularity. Cupid's bow peaks are marked on lateral labial elements, allowing for medial extension of the white roll and sufficient vermilion height for the median tubercle. Curvilinear lines are drawn at alar base junctions. After anthropometric measurements and a lidocaine/epinephrine wait, critical points are tattooed.

Labial Dissection

Lightly score all labial lines.
De-epithelialize philtral flanking flaps, discard extra prolabial skin, and elevate the philtral flap (with subcutaneous tissue) to the anterior nasal spine.
Incise lateral white-roll-vermilion-mucosal flaps, stopping 2–3 mm short of tattooed Cupid's bow peak-points.
Disjoin lateral labial elements from alar bases and free basilar flaps from piriform attachments by incision along the lower vestibulocutaneomucosal junction.
Extend mucosal incisions distally along the gingivolabial sulcus to the premolar region.
Widely dissect lateral labial elements off the maxillae in the supraperiosteal plane, extending over the malar eminences, to minimize tension at closure.
Dissect orbicularis oris bundles in the subdermal and submucosal plane for 1 cm or more.

Alveolar and Muscular Closure

Release lateral nasal mucosal flaps, elevate medial nasal mucosal flaps, and close the nasal floors.
Continue premaxillary mucosal incisions and make vertical incisions in facing gingiva of lesser segments.
Apply digital pressure to the premaxilla to allow alveolar gingivoperiosteal closure.
Advance alar base flaps medially and suture inner edge to the anterior nasal floor.
Trim the thin strip of vermilion from the premaxillary mucosa and secure the remaining mucosal flange high to the premaxillary periosteum to form the posterior wall of the central gingivolabial sulcus.
Advance lateral labial elements during sulcal closure, using a back-cut at the sulcal incision's distal end. This forms the anterior wall of the central gingivolabial sulcus.
Appose orbicularis bundles (end-to-end, inferior-to-superior) with polydioxanone sutures.
Place a polydioxanone suture through the maxillary periosteum on each side (near depressor alae nasi origin) and leave untied.
Suspend uppermost muscular elements to the anterior nasal spine periosteum with a polypropylene suture.

Constructing the Median Tubercle

Place a fine chromic suture about 3 mm medial to the tattooed lateral Cupid's bow peak-point, joining the white-roll-mucosal flaps in the midline.
Trim excess vermilion-mucosa from each flap and align them to form the median raphe. Avoid saving too much, which can cause a furrowed raphe.

Nasal Dissection and Cartilage Positioning

Visualize slumped/splayed lower lateral cartilages through bilateral rim incisions ("semi-open" approach).
Dissect fibrofatty tissue off the anterior surface and between cartilages, extending across the dorsal septum to expose upper lateral cartilages.
Place a horizontal mattress suture (5–0 polydioxanone) between the genua and leave untied.
Insert mattress sutures through each upper lateral cartilage and then through the ipsilateral lateral crus, often placing a second suture to suspend it.
Trim the C-flap on each side of the columellar base to 3–5 mm.
Advance alar bases medially, rotate endonasally, and suture side-to-end to the C-flaps. Trim alar base flap tips to close sills.
Place a polypropylene "cinch suture" through the dermis of each alar base, passing under the philtral flap, and tie it to narrow the inter-alar dimension to less than 25 mm.
Bring maxillary periosteal sutures (placed earlier) above the muscular layer, insert through alar bases (superficial to cinch suture), and tie them to form cymal sills and prevent alar elevation.

Final Touches and Postoperative Care

Attempt to fashion a philtral dimple by suturing dermis in the lower one-third of the philtral flap to the orbicularis layer.
Inset the philtral flap tip into the handle of Cupid's bow. Leave lateral labial flaps untrimmed initially to simulate columns.
Ensure no tension at philtral closure, using fine, interrupted sutures.
Trim the cephalic margin of labial flaps to match the sills' configuration.
Excise redundant domal skin in soft triangles and upper columella in a crescentic fashion from rim incisions, extending along the columella sides. This narrows the nasal tip and defines the mid-columella.
Perform lenticular excision on the cutaneous side of the intercartilaginous junction to flatten the lateral vestibular ridge.
Document immediate postoperative nasolabial anthropometry.
Insert vented "stents" (Xeroform gauze on silicone tubing) into nostrils for 48 hours.
Postoperatively, apply a Logan bow to protect the repair and hold iced-saline sponges. Parents are instructed on suture-line care and nasal cleaning. Percutaneous sutures are removed 5–6 days later under anesthesia. Steri-Strips are applied for 6 weeks, followed by digital massage and sun-block ointment.

Technical Modifications for Bilateral Variants

Specific anatomical variations require adapted techniques.

Late Presentation of Bilateral Complete Cleft Lip/Palate

If the premaxilla is rigid and dentofacial orthopedics are not feasible (e.g., in late infancy or developing countries), consider ostectomy and set-back. This can be combined with nasolabial repair or palatoplasty, with careful attention to mucosal blood supply. The latter is safer if the child is nearing age 1 or older, prioritizing speech.

Binderoid Bilateral Complete Cleft Lip/Palate

This rare variant features orbital hypotelorism, hypoplastic bony/cartilaginous elements (short septum, absent anterior nasal spine), conical columella, hypoplastic prolabium/premaxilla (single incisor), and thin lateral labial vermilion. The floppy premaxilla often precludes dentofacial orthopedics. Synchronous nasolabial repair proceeds similarly, but the philtral flap needs less allowance for expansion, and interalar narrowing should be aggressive. Secondary procedures are common for augmentation.

Bilateral Complete Cleft Lip and Intact Secondary Palate

This is another very rare form with a solid premaxilla. If not severely protrusive, synchronous nasolabial repair and closure of the posterior premaxillary cleft can be done. Alternatively, premaxillary set-back and gingivoperiosteoplasties, with delayed palato-premaxillary cleft closure, can be considered.

Bilateral Incomplete Cleft Lip

One-quarter of bilateral clefts are incomplete, most symmetrical. This is the easiest variant to repair. Design and execution are similar to the complete form, with adjustments for growth. If a prominent central white roll exists in lesser-form cases, the prolabial vermilion-mucosa can form the central segment. Interalar narrowing is always needed. If columellar length is normal and cartilages are well-positioned, extensive tip sculpting may be unnecessary, but bilateral rim incisions are used to over-correct if needed.

Asymmetrical Bilateral (Complete/Incomplete) Cleft Lip

Symmetry is paramount here. The operative strategy depends on the severity of the lesser side:

Contralateral Incomplete (including minor-form): Synchronous bilateral repair is indicated. Exaggerate maneuvers on the complete side due to greater distortions. Use bilateral rim incisions and over-correct cartilage on the complete side, even if the incomplete side seems normal.
Contralateral Microform or Mini-Microform: If the greater side is complete, manage it with unilateral dentofacial orthopedics, followed by nasolabial adhesion and alveolar gingivoperiosteoplasty, converting it to an incomplete cleft. Then, if the contralateral (lesser side) is a minor-form or severe incomplete, perform simultaneous bilateral nasolabial repair. For a microform, repair the complete side first, observing the microform when designing incisions. Later, correct the microform using double unilimb Z-plasty, dermal graft, and nasal correction. Mini-microforms may be corrected by vertical lenticular excision during greater side repair or deferred until older. Mirror-image symmetry is the goal.

Outcomes and Assessment

The surgeon's responsibility extends to long-term assessment of outcomes, learning from observations, and refining techniques.

Photography as Documentation

Preoperative, intraoperative (after markings and immediately post-repair), and periodic postoperative photographs are essential. Standardized frontal, submental, and lateral views help assess nasal configuration and symmetry.

Revision Rates and Common Issues

Documenting revisions helps surgeons refine primary procedures. For bilateral deformities, the cutaneous lip should ideally not need reopening, though nasal cartilages, mucosal free margin, or nasal width may require adjustment. Asymmetries become more obvious before school age and can be magnified during adolescence.

Labial Revisions: Most common were resuspension of prolapsed anterior gingivolabial mucosa (now minimized by trimming and securing mucosa) and augmentation of a weak median tubercle with a dermal graft (often during alveolar bone grafting).
Nasal Revisions: Disproportionate widening of the inter-alar dimension was frequent but rarely required correction in childhood. Secondary "columellar lengthening" or revisions for wide/long philtrum were less common with modern techniques.
Asymmetry: Revision rates correlate with preoperative asymmetry. Lower rates are seen in contralateral minor-form subgroups, higher in contralateral mini-microform, where primary symmetry is harder to achieve.
Maxillary Advancement: High rates of Le Fort I osteotomy and maxillary advancement are observed, reflecting a preference for operative correction of midfacial retrusion.

Quantitative Assessment: Anthropometry

Objective, quantitative methods are crucial for assessing nasolabial appearance.

Direct Anthropometry: Farkas's work established normative values for nasal and labial measurements in various age groups and ethnicities. This method uses calipers and requires training. Intraoperative anthropometry assesses deformity severity and records baseline dimensions, confirming the strategy of crafting fast-growing features smaller and slow-growing features larger than normal, anticipating growth.
Indirect Anthropometry (Photogrammetry): This eliminates measurement inaccuracies from a fidgety child. It uses two-dimensional photographs to measure linear, angular, and proportional features. While useful, it can introduce errors due to magnification or inconsistent lighting/angles. Studies show primary nasal correction yields near-normal columellar length but may still have wider nasal dimensions.
Stereophotogrammetry (3D): This advanced method uses synchronized high-resolution cameras and software to create a three-dimensional image. It allows for accurate and reliable measurement of nasolabial dimensions, soft tissue projection, and mirror-image symmetry. Systems like 3dMDface™ and Vectra® are used for intra- and inter-institutional comparative studies.

Conclusion

Bilateral cleft lip and nose repair demands a surgeon's patience, precision, and passion. The primary procedures are the major determinants of a child's appearance and ability to communicate. Continued assessment into adulthood is a critical obligation, ensuring that surgical techniques constantly evolve to provide the best possible outcomes for every child.

FAQ: Bilateral Cleft Lip and Nose Repair

What are the main principles of modern bilateral cleft lip and nose repair?

Modern repair focuses on maintaining nasolabial symmetry, securing primary muscular continuity, designing the philtral flap with proper size and shape, constructing the median tubercle from lateral labial elements, and anatomically positioning/sculpting the lower lateral cartilages and nasal tip. These principles aim to restore both appearance and function.

Why is preoperative manipulation of the premaxilla important for bilateral cleft lip?

Preoperative dentofacial orthopedic manipulation, using devices like the Latham appliance or nasoalveolar molding (NAM), helps to retract and centralize the protuberant premaxilla. This alignment of the maxillary elements creates a better foundation for surgical repair, allowing for proper philtral flap design, anatomical nasal cartilage positioning, and closure of alveolar clefts.

How has the approach to nasal correction in bilateral cleft lip changed over time?

Historically, nasal correction was often deferred until later, leading to secondary procedures like the forked flap to lengthen a perceived "short columella." However, it's now understood that the columella isn't absent but rather its cartilaginous components are splayed and malpositioned. Modern approaches focus on primary nasal correction during the initial surgery, anatomically positioning the lower lateral cartilages and sculpting existing soft tissues to achieve normal nasal projection and columellar length.

What are the main anatomical forms of bilateral cleft lip?

Bilateral cleft lip typically presents in three major anatomical forms: bilateral symmetrical complete (affecting both sides fully), bilateral symmetrical incomplete (less severe clefts on both sides), and bilateral asymmetrical (where one side is complete and the other is incomplete). The extent of palatal involvement often corresponds to the severity of the labial clefts.

How are outcomes of bilateral cleft lip and nose repair assessed?

Outcomes are assessed through various methods, including serial photography for visual comparison and documentation of revision rates. Direct anthropometry, using precise measurements of nasolabial features, is considered the gold standard. Advanced techniques like two-dimensional photogrammetry and three-dimensional stereophotogrammetry provide accurate and reliable quantitative data, allowing for detailed analysis of changes over time and comparisons between patients.

Bilateral Cleft Lip and Nose Repair