You are in: eMedicine Specialties > Plastic Surgery > CRANIOFACIAL Craniofacial, Bilateral Cleft Lip RepairArticle Last Updated: Jun 16, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Pravin K Patel, MD, Associate Professor of Surgery, Division of Plastic Surgery, Northwestern University School of Medicine, Chief of Plastic & Craniofacial Surgery, Shriners Hospitals for Children, Head of Craniofacial Surgery, Children's Memorial Hospital Coauthor(s): Kara K Criswell, MD, Staff Physician, Department of Surgery, Division of Plastic, Reconstructive and Cosmetic Surgery, University of Illinois at Chicago; Bryan K Criswell, MD, Staff Physician, Division of Plastic, Reconstructive and Cosmetic Surgery, University of Illinois at Chicago; Mimis Cohen, MD, FACS, FAAP, Professor and Chief, Division of Plastic, Reconstructive and Cosmetic Surgery, University of Illinois at Chicago; Consulting Staff, Division of Plastic Surgery, John H Stroger Jr Hospital of Cook County Editors: Larry Hollier, Jr, MD, Assistant Professor, Department of Plastic Surgery, Baylor University College of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; R Edward Newsome, MD, Associate Professor, Program Director and Chief, Department of Surgery, Section of Plastic Surgery, Tulane University Health Sciences Center; Nicolas (Nick) G Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center at Swedish Medical Center; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics Author and Editor Disclosure Synonyms and related keywords: bilateral cleft lip repair, bilateral cleft lip, cleft lip and palate, cleft lip, cleft palate, facial cleft, congenital deformities, hare lip INTRODUCTIONSection 2 of 12
  No other congenital deformity has the potential to alter the facial form as significantly as does a bilateral cleft lip and palate. The central third of the face is distorted significantly by the "double" cleft, and restoring the normal facial form is a formidable challenge for the reconstructive surgeon. History of the ProcedureThe history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilizing the underlying musculature. Accompanying the evolution of surgical repair is the increasingly important role of orthodontic support with early presurgical alveolar and nasal molding. Repositioning the maxillary and alveolar segments into a more anatomic position allows the surgeon to repair the lip and associated nasal deformity under more optimal conditions. The reader is referred to the references cited for a more detailed historical review of individual repairs and supportive management. ProblemThe cleft affects the obvious facial form as an anatomic deformity and has functional consequences, affecting the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and be staged appropriately with the child's development. The need for intervention must be balanced against its subsequent effect on normal growth. In the child born with a bilateral cleft, the surgeon initially is faced with a protrusive premaxilla and the difficulty of achieving adequate columellar length and vertical height to the lip during reconstruction. FrequencyThe overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births, making it the second most common congenital deformity (after club foot). Incidence varies by race, with clefts occurring more commonly in Asians (1 in 500 births), less frequently in Caucasians (1 in 750 births), and even more uncommonly in African Americans (1 in 2000 births). These racial variations in incidence are not observed with the isolated cleft palate. In terms of gender, the incidence of cleft lip/palate is more common in males, while the incidence of cleft palate alone is more common in females. The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip:right cleft lip:bilateral cleft lip - 6:3:1). In a large series reported in the literature, bilateral clefts are observed in approximately 15% of patients with clefts. As a guideline, bilateral clefts occur in approximately 1 in 5000-6500 births based on the overall occurrence. EtiologyThe etiology of clefts is multifactorial, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate). Environmental causes such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants) during the first trimester have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology. PathophysiologyWhile the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows. Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, two lateral maxillary, mandibular processes) at 4-7 weeks gestation is necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen). The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidence. ClinicalAs with the unilateral cleft, a wide range of clinical presentations of the bilateral cleft lip and palate is possible, from the simple microform cleft to the complete cleft bilaterally involving the lip, alveolus, palate, and nose. In addition, the cleft may present with varying degrees of asymmetric involvement of the lip and palate. Any degree of combination of involvement of the lip, primary palate, and secondary palate can exist. Recognizing these potentially asymmetric variations is essential in planning operative treatment and evaluating postoperative results. In the complete form of clefts, the premaxilla is unrestrained by the lateral maxillary segments and the vomer grows anterior to the lateral segments. This distorts the alar cartilages away from the nasal tip and stretches the alae across a widening cleft. The columella is significantly deficient, can be virtually nonexistent, and without a distinct demarcation from the prolabium, which also is vertically deficient. No prolabial muscle, philtral columns, dimples, or Cupid's bow is present. No nasal floor is present as the cleft continues through the palatal shelf along either side of the vomer through the soft palate. INDICATIONSSection 3 of 12
The indication for reconstruction is the need to restore the normal morphologic facial form and function, as they are intimately related to proper development of dentition, mastication, hearing, speech, and breathing. RELEVANT ANATOMYSection 4 of 12
Understanding normal lip and nasal anatomy is essential to recognize the distortion caused by a facial cleft. The elements of the normal lip are the central philtrum, which is demarcated laterally by the philtral columns and inferiorly by the cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge referred to as the white roll. Within the red vermilion of the lip are two distinct areas: the dry vermilion, the more keratinized portion of the lip that is exposed to air, and the wet vermilion, which is exposed to the moist environment of the labial mucosa. The primary muscle of the lip is the orbicularis oris. It has two well-defined components: the deep (internal) layer and the superficial (external) layer. The deep fibers run circumferentially from commissure (modiolus) to commissure (modiolus) and function as the primary sphincter for oral feeding. The superficial fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis, and functions to provide subtle shades of expression and precise movements of the lip for speech. The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove, forming the philtral columns. The resulting philtral dimple is centrally depressed as there are no muscle fibers that directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle. In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers arise from the medial aspect of the infraorbital rim and sweep down to insert near the vermilion cutaneous junction. The most medial fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the cupid's bow. The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura. A bilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate laterally and cephalad. CONTRAINDICATIONSSection 5 of 12
Malnutrition, anemia, or other conditions that may contribute to the patient's inability to tolerate general anesthesia are contraindications to this procedure until corrected. The true risks of cleft lip repair are primarily the risks associated with anesthesia. Frequently, children with associated cardiac and other possibly life-threatening anomalies require correction of those issues prior to cleft lip repair. WORKUPSection 6 of 12
Lab Studies
TREATMENTSection 7 of 12
Surgical therapyChildren born with a facial cleft benefit from a multidisciplinary approach in a team-based setting so that all aspects of their care can be coordinated efficiently. Beyond lip repair are hearing, speech, dental, and psychosocial integration issues. These should be addressed throughout the child's growth and development from birth through adolescence. These issues are as important as the anatomic reconstruction. Ultimately, the functional outcome of the reconstruction depends on cooperation between the surgeon, orthodontist, and speech pathologist. Each specialty involved must evaluate the child individually, formulate a treatment plan, and integrate it with those of the other specialties based on the child's needs. The Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association should be followed. However, rather than strictly adhering to one protocol, physicians should assess each child individually and formulate the treatment plan based on the team's experience, its overall philosophy of treatment, and available resources. Preoperative detailsWhile the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs. Parents who suddenly are faced with caring for a child with a facial cleft are overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to stress the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized. No agreement is found in the literature regarding the ideal timing of lip repair. Some have advocated surgery in the early neonatal period, with a theoretical benefit in scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin of 10 g, weight of 10 lb, and is aged 10 weeks. In general, most centers prefer to perform the lip reconstruction when the patient is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction. A presurgical, fabricated, passive, intraoral orthodontic palatal appliance can be used to maintain the arch width to prevent the nearly inevitable collapse that occurs with lip repair. Rarely is there an indication for active expansion of the maxillary segment. This passive plating and gentle traction is an integral component of surgical cleft lip repair. The repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Recently, the palatal appliance has been modified to include a nasal extension to help improve the nasal tip form. The orthodontist take impressions and the custom appliance is fitted as soon as possible after birth and well before the lip repair. The appliance also aids in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction. Some centers have chosen either no presurgical orthopedic intervention or an active pin-based appliance (eg, Latham) to align the maxillary arch segments. For bilateral clefts, external pressure is routinely used to help maintain the premaxillary component within the arch alignment. Soft elastic tape (eg, 3M Microfoam tape) across the premaxilla, a head cap with elastic traction, or lip adhesion can be used prior to a definitive lip repair once the arch segments have approximated. Intraoperative detailsThe ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; adequate columellar length; a well-defined philtral dimple and columns; a natural-appearing cupid's bow with a pout to the vermilion tubercle; and an adequate labial sulcus. In addition, lip scars are used to approximate the natural landmarks. The ideal repair results in functional muscle repair that with animation that mimics a normal lip. A number of surgical procedures with many variations for the repair of bilateral cleft lip are well described. Among these are the repairs of Veau, Tennison, Manchester, Millard, and others. The Veau III operation is a straight-line closure without elevation of the prolabial skin and correspondingly without any attempt at restoring the continuity of the orbicularis oris. The central cupid's bow and tubercle is constructed from the vermilion of the lateral lip elements. In contrast, Millard's repair involved complete elevation of the prolabium and reconstitution of the orbicularis across the premaxilla. In addition, Millard banked lateral segments of the prolabium as "forked flaps" that were meant to add columellar height at a later stage. As with Veau, the central vermilion is recreated from the lateral lip elements. Unlike Veau and Millard, Manchester preferred to maintain the prolabial vermilion to create the cupid's bow and tubercle, but similarly to Veau, Manchester's repair did not involve repairing the orbicularis as he felt this would create an overly tight lip. In recent years, significant contributions by McComb, Mulliken, Nakajima, and Cutting have integrated the correction of the associated nasal deformity with simultaneous lip repair that appears to achieve adequate primary columellar lengthening and nasal tip projection. More recently, McComb's experience led him to stage the repair. The initial stage involves approximating and repositioning the splayed alar cartilages through a V-Y nasal tip "gull-wing" incision that allows redraping the overlying skin with a simultaneous bilateral lip adhesion. A definitive lip repair follows at a second stage. Mulliken's extensive experience has evolved from a median nasal tip incision for exposure to bilateral rim incisions that allow adequate access to correct the nasal cartilage deformity. Nakajima and Cutting have introduced presurgical molding of the nasal tip and columellar with acrylic outriggers attached to a palatal appliance. For the specific details of each of these repairs and supportive treatment, the reader is referred to more detailed references of the individual surgeons or more comprehensive texts cited in the Bibliography. A cursory description follows of an operative technique the authors have used:
Postoperative details
Follow-upFollowing cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and proper dental care need to be emphasized. Psychosocial evaluation and treatment should be made available. Follow-up appointments with speech pathologists should be continued until normal or near normal speech is achieved. Close cooperation among the members of the cleft team is necessary for optimal outcomes. COMPLICATIONSSection 8 of 12
Aside from an unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair and hypertrophic scar formation and/or contracture of the lip scars. If dehiscence occurs, re-operation is postponed until the induration has subsided completely. In the intervening period, control of the premaxilla with orthodontic devices to prevent rotation caused by asymmetric forces may be needed. With lip scars that remain red, thick, and contracted, the authors use an occlusive tape dressing and, occasionally, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of normal healing and improves with time. Wait to perform intervention until the lip scar matures (generally 1 y), and the intervention should be guided by the severity of the residual deformity with the goal of minimizing the number of revisions. A number of secondary deformities of a less than ideal outcome are well characterized. These primarily include the whistle deformity of the lip, a vertically deficient upper lip, a constricted lip and muscular diastasis. Correction of these residual deformities must be specifically tailored and range from minor revisions to fully re-creating the defect and reconstructing each of the elements of the lip to discarding the prolabial element and replacing it with a lower lip Abbe reconstruction. OUTCOME AND PROGNOSISSection 9 of 12
While maintaining symmetry typically is not the main issue in reconstructing the bilateral cleft lip, the outcome can be less satisfying than with unilateral clefts. Careful preoperative assessment of the cleft lip deformity and attention to appropriate presurgical management and detail in the reconstruction typically results in an acceptable repair that achieves some of the characteristics of the natural lip and nose. Many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip. While a poor initial result is unlikely to improve with time, it cannot be assumed that an excellent initial result will not require some revisional procedure because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures to achieve nasal symmetry are commonly required, despite the initial primary nasal surgery incorporated as an integral part of lip repair. Realistically, one must realize that despite physicians' best attempts, the stigmata of a bilateral cleft deformity remains in many children. FUTURE AND CONTROVERSIESSection 10 of 12
Bilateral cleft lip surgery has evolved from discarding the premaxillary and prolabial elements in a simple approximation of the cleft margins to, currently, a definitive single-stage lip and primary cleft nasal repair that incorporates the underlying musculature. Accompanying the evolution of the surgical repair is the increasingly important involvement of early presurgical alveolar and nasal molding in possibly improving surgical outcome. Only close long-term follow-up care and an honest assessment of the results can establish these improvements in outcome. Advances in the treatment of children with bilateral clefts will come only from a team-based approach, where a close cooperation of multiple disciplines can address all the child's needs. Bilateral clefts are an uncommon entity. Children with this deformity deserve to be cared for at major centers where an interdisciplinary approach is possible and substantial experience is available. MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Craniofacial, Bilateral Cleft Lip Repair excerpt Article Last Updated: Jun 16, 2006 | |||||||||||||||||||||||||||||||||
