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Secondary Cleft Lip Reconstruction
3.3 Secondary Cleft Lip Reconstruction Jonathan T. Xu, June K. Wu
SYNOPSIS Primary cleft lips are congenital and result from a failure of the medial nasal prominence of the frontonasal process to join with the maxillary process during embryogenesis. Variability in primary cleft lip defects consists of a spectrum based on their severity, such as complete/ incomplete, the width of the cleft, and the alignment of the medial and lateral segments. In primary cleft lips, multiple surgical techniques and methods exist specifically to address the deformity and achieve the goal of symmetrical labial and nasal anatomy and are generally performed before 6 months of age. However, unlike primary cleft deformities, secondary cleft deformities have varied etiologies: they can result from persistent residual deformities, develop over time during facial growth, or form as a sequelae of primary repair techniques. Secondary cleft deformities include (but are not limited to) a too-short or too-long white lip, misalignment of vermilio-cutaneous junctions, or abnormal contour of the free mucosal border. Because secondary deformities are more variable than the primary cleft deformities, both in etiology and presentation, secondary corrective procedures need to be individualized to address the specific deformity for each patient. As such, the goal of secondary cleft lip procedures is to redress specific cleft stigmata that are either residual from the initial surgery or have arisen over time. Secondary deformities can be corrected through application of general plastic surgery principles and techniques. Pre-operative examination of the issue and attentiveness to the timing of the procedure are crucial to a successful secondary repair, though prevention of secondary deformities arising from primary repair should remain paramount. Lastly, it is important to note that because many of these secondary deformities can appear concomitantly in a patient, one must prioritize treatment that will provide the best result to the patient, and utilizing the fewest number of operations.
CLINICAL PROBLEM AND PRINCIPLES OF TREATMENT Clinical problems of secondary cleft lip reconstruction are rooted in stigmata that arise after primary cleft lip surgery. Recognizing the various deformities allows one to be better informed in enacting pre-operative care and planning the course of action for subsequent procedures. This section attempts to cover the most commonly recognizable deformities, which can be sorted into five basic categories: general scarring, white lip, vermilion and mucosa, muscle, and buccal sulcus. The principal stigmata in unilateral clefts are usually related to asymmetry, whereas the stigmata in bilateral clefts are related to disproportion of repaired elements to the face. Both unilateral and bilateral clefts can have
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distortions based on deeper soft tissue elements such as muscle and mucosa. The following section will be organized by discussing commonly seen stigmata as well as common procedures to rectify them. From an operative perspective, one major difference between primary and secondary cleft lip repair is that whereas the former case employs tailored cleft lip repairs with the aim of properly orienting lip and nasal elements, the latter case uses general plastic surgery principles and techniques to achieve its goal. To reiterate, secondary cleft lip procedures aim to fix stigmata that have either arisen from the initial procedure or have arisen with time. Thus for an in-depth discussion of cleft lip repair techniques, please refer to the chapters on primary cleft lip repair (Chapters 3.1 and 3.2).
General Scarring Scarring from the previous surgical intervention(s) should be examined for signs of immaturity, contour changes, and distortion of local structures because worsening scarring may require acute intervention, whereas improving scars should be carefully monitored until they mature.1 Scarring along the philtral ridge on the cleft side may be thick and prominent. Scars are still considered immature within 1 year of the primary repair, and during this time, scar massage is recommended. Occasionally, for a very firm and raised hypertrophic scar, injection with triamcinolone is acceptable and can usually be done during cleft palate repair. However, due to the child’s age, the injection may not be given without general anesthesia. Persistent incisional hypertrophic scars nonresponsive to massage or triamcinolone injections should be excised and reclosed.
Short and Long Lip (Unilateral Cleft) Short and long lip are usually asymmetries seen compared with the normal half of the lip and are stigmata of unilateral clefts, occurring when the repair is not matched to the normal side. A short lip is recognized by having a philtral column on the cleft side that is at least 3 mm shorter than the contralateral non-cleft side, whereas a philtral column that is longer vertically on the cleft side compared with the non-cleft side is characteristic of a long lip. Active underlying scar contracture (that improves over time) may be responsible for producing a minor short lip, whereas more severe cases are usually the result of an improperly designed rotation advancement lip repair. A long lip stigma resulting from a rotation advancement repair is much rarer and is more frequently associated with the Tennison repair.1,2 Short lip deformities are usually associated with the rotationadvancement repair of Millard and similar techniques. A very minimally shortened white lip on the cleft side can be attributable to superficial scar contracture during wound healing, worst at 6 to 12 weeks after
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
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FIG. 3.3.1 (A) A hyperpigmented scar just cephalad to Cupid’s peak on the cleft side gives the appearance of an under-rotated repair. (B) This scar can be excised with a diamond shape and closed primarily.
primary repair, and will eventually resolve and can be improved with active massaging.2 In the case of a minor short lip deformity (<3 mm), a diamond-shaped scar excision is sufficient in augmenting the lip 2 to 3 mm (Fig. 3.3.1). For short lip deformities that are greater than 3 mm, Z-plasties along the philtral column of the cleft side can lengthen the white lip length. However, oblique and horizontal scars out of line with the philtral column may result. Severe mismatch may be a result of technical error from the primary repair (Fig. 3.3.2), and will require complete takedown and re-rotation and advancement of the cleft lip.1,2 Long lip stigmata have become increasingly rare due to a reduction in the usage of the Tennison repair in favor of the Millard rotationadvancement technique in primary cleft lip repairs.3 Nonetheless, in the case of a long lip deformity, multiple studies have shown that superficially excising tissue from the alar base in an effort to elevate the cleft side is insufficient in redressing the issue. The use of permanent suspension sutures to the periosteum to aid in long lip repair also has mixed results. Thus it is recommended to take down the entire repair and to excise tissue from all dimensions to fully fix the stigma. In bilateral clefts, an overly long and overly wide philtrum will appear disproportionate to the face and is not primarily from asymmetry from left to right side and will be addressed in a later section.
Tight and Wide Lip (Bilateral Clefts) An overly long and overly wide philtrum may result because those nasolabial elements are fast-growing, potentially causing the stigmata to worsen as the child grows (Fig. 3.3.3A). The best way to prevent these stigmata is to perform a primary repair that intentionally minimizes the philtral flap dimensions.4 This correction can be achieved by marking a shorter and narrower philtral flap within the philtrum, then re-raising the smaller philtral flap while discarding the rest of the oversized philtral skin (Fig. 3.3.3B). The medial tubercle is then divided, and the lateral lip elements are re-advanced and raised to meet the new philtral flap. The underlying muscles and mucosa may need to be re-approximated as well. A tight lip may occur in Binder syndrome, where the prolabial elements were abnormally small and underdeveloped, or in overly wide clefts due to inadequate advancement of the lateral lip elements (Fig. 3.3.3C). Another stigma presents as a whistling deformity in the median tubercle; unlike the whistling deformity of unilateral clefts, this defect typically occurs in the midline and tends to be symmetrical. Mild defects can be improved by addressing the mucosa alone (see mucosal section). For moderately tight lips, the bilateral repair can be taken down and the lateral lip elements re-advanced. In cases of severely tight lips, an Abbé flap can be used by borrowing vermilio-mucosal tissues from the lower lip.
FIG. 3.3.2 This shows an under-rotated repair with shortened vertical height of the white lip on the cleft side, as well as a whistling deformity.
Vermilio-Cutaneous Mismatch (Unilateral Clefts) Distortions of the Cupid’s bow can result from vermilio-cutaneous misalignment from the primary repair. Imperfect opposition of the white roll from the non-cleft side to the cleft side can also be visually obvious. These vermilio-cutaneous mismatches can be corrected by a simple Z-plasty or diamond notch excision (Fig. 3.3.4). In more severe cases, it may be necessary to take down the previous lip repair and to repeat the rotation advancement. In addition, as described previously, an Abbé flap can be used to transfer healthy, unscarred tissue from the lower to the upper lip.1,2 Additionally, it is important to note that only vermilion tissue should be used to reconstruct vermilion tissue.
Vermilio-Mucosal Mismatch and Deficiencies (Unilateral and Bilateral Clefts) Usually, the vermilion of the lip on the non-cleft side is thinner than the vermilion on the cleft side; therefore it is very important to align the vermilio-mucosal junctions during the primary repair, including the use of triangular vermilion flaps from the cleft side. However, if there is mismatch present, it may result in a whistling deformity (see the following section); in that case, there is a possibility that no more vermilion is available for correcting the mismatch. For that reason, it is important to ensure proper vermilio-mucosal alignment at the time of primary repair.
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FIG. 3.3.3 (A) A wide philtrum after bilateral cleft lip repair. This results from the fast growth of the philtral elements. (B) After revision. (C) A tight lip with loss of median tubercle after bilateral cleft lip repair. This can result from a wide cleft or from lack of premaxillary tissue, such as in Binder syndrome.
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FIG. 3.3.4 (A) A mismatch, or step-off, in the vermilio-cutaneous junction can be corrected with a simple Z-plasty. The medially based vermilion flap (green-blue) is transposed inferiorly, while the laterally based cutaneous flap (green-black) is transposed superiorly. Care should be taken to align the white roll (blue black lines, B).
Mucosal and Buccal Sulcus Deformity A whistling deformity can result from tissue deficiencies of either the vermilion and/or the mucosa. In cases where the deformity is due to a maldistribution of vermilion, as in the case of a central deficiency with lateral excess, a V-Y advancement can be performed to correct this defect. This can be repaired with a V-Y advancement flap from the posteriorly based buccal mucosa (Fig. 3.3.5). Additional volume can be achieved with dermal fat-grafts taken from the groin as a means of lip augmentation, as demonstrated by Coleman et al., and has been shown with mixed success.2,5 For cases of severe notching deficiency of
the medial tubercle of a unilateral bilateral lip, the Abbé flap is the best choice of treatment due to the need for fresh tissue. Deformities involving the gingivobuccal sulcus can be due to either a surfeit or paucity of mucosal tissue and are commonly seen in bilateral deformities. Examining the buccal sulcus will also reveal how free the lip is from the maxilla; a tethered lip could be the result of scar contracture or a lack of tissue related to the initial deformity.1,2 In the case of surfeit of tissues, one can simply excise excess tissue in the area and re-suspend the mucosa to the maxillary periosteum. When there is insufficient mucosal tissue or abnormal tethering of the lip to the maxilla with loss of the buccal sulcus, the lateral lip can be opened
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
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FIG. 3.3.5 (A) For correction of mucosal deficiency resulting in a mild whistling deformity, a V-incision is planned on the mucosa. (B) A vertical limb of a “Y” is made when the inferior portion is sutured to each other (blue arrowheads).
and re-advanced medially to supply mucosal tissue. The buccal sulcus can also be opened, and mucosal grafts harvested and grafted to cover the defect. Though skin grafts (split-thickness and full-thickness) can be used, buccal mucosa remains the first choice for graft material.1
Muscle Deformities Deformities in muscle—namely the orbicularis oris—can arise from either inadequate muscle re-approximation at the time of the initial surgery or from subsequent tissue dehiscence. For example, dehiscence of the orbicularis oris can present as a bulge on either side of the philtral incision, resulting in a short and widened lip scar. Misalignment of the pars marginalis portion of the orbicularis oris may efface the prominence of the white roll.6 Other muscles in the oronasal region, such as the alar portion of the nasalis, can also be deformed, resulting in abnormal elevation of the alar base upon smiling. Nevertheless, because muscle reconstruction has become a standard part of primary repair, this chapter will not focus on specific surgical techniques that address muscle deformities.
Nasal Repair Even with primary nasal repair, nasal revision will often be necessary. In unilateral cleft lip and nasal deformities, the underlying cleft nasal deformity includes a deviated septum to the non-cleft side, and inferior and lateral displacement of the cleft alar base (Fig. 3.3.6). The goal of primary repair and subsequent revision is to centralize the caudal septum, symmetrize the lower lateral cartilage, and elevate and narrow the alar base to match the non-cleft side. In bilateral cleft lip and nose deformities, there is loss of projection of the nasal tip and an overly wide alar base. In that case, the goal of repair is to lengthen the columella and narrow the alar base. While some have advocated the use of forked flaps, Mulliken has suggested, along with others, that the “columella is in the nose.”7 The nasal projection can be corrected by repositioning the medial crus of the lower lateral cartilage. In both unilateral and bilateral deformities, the alar base should be anchored to the underlying orbicularis oris and maxillary periosteum to keep the alar base from elevating upon the action of smiling. Even with primary nasal repair, nasal deformities can persist, necessitating secondary revisions. The goals of secondary revision are usually to symmetrize the nasal tip and improve nasal projection, and these procedures can be performed with closed or open rhinoplasty techniques. Patients may also need additional support to the lower lateral cartilages.8 The definitive rhinoplasty should be performed after facial growth is complete or near complete, in one’s teenage years. Assessment of the nasal appearance, symmetry, and function (breathing, airflow) should be the same as that for any patient seeking a rhinoplasty; the primary cleft nasal deformity as well as previous nasal surgeries should be taken into account.
PRE-OPERATIVE MANAGEMENT The best time to address secondary deformities is at the time of the primary operation—to take the prophylactic approach and prevent (or at least reduce) these deformities from occurring! Current standard practices have moved away from staged repair and have advocated the use of pre-surgical orthopedics and the completion of cleft nasal repair at the time of primary lip repair.9 Attention should be paid to overcorrecting slow-growing nasolabial features and to undercorrecting fastgrowing ones.4 The goals of these principles serve to minimize the need for secondary revisions. That being said, most cleft lip patients will require secondary procedures at some point in their life.
Timing As is the case with primary cleft lip repair, a surgeon must be able to conceptualize his or her alterations in the context of the fourth dimension; failure to fully take into account nasolabial and facial growth over time can lead to secondary repairs of stigmata that could have been potentially avoided. Regarding the stigmata of repaired unilateral or bilateral cleft lips, the general rule is that fast-growing features become too long or wide, whereas slow-growing features remain short; hence, fast-growing features should be crafted smaller than normal, whereas slow-growing features should be constructed on a slightly larger scale. The only exception to these aforementioned guidelines is the formation of the fast-growing median tubercle, which actually lags behind in a repaired bilateral cleft lip and therefore should be made as full as possible in the initial procedure.7,10 Another consideration regarding the timing of secondary procedures takes into account the severity of an individual’s deformity in the context of his or her psychosocial development. Thus proper secondary procedural intervention will need to address both functional impairments (speech, breathing, or eating) and aesthetic deformities. As peer-to-peer interactions begin to develop in early childhood (beginning from ages 4–5), the risk of teasing by one’s peers can significantly impact a child’s psychosocial development.2 For that reason, it is recommended that all patients should be evaluated for secondary surgery around ages 4 to 5.1 Another common period for secondary surgical correction is during adolescence, due to the changing nature of peer interactions and increased concern regarding one’s own appearance. As adolescents become more capable of vocalizing their own opinions, their wishes should also play a significant role in the decision-making behind secondary corrective procedures.1,2 As is the case with primary repair, marking the lip properly is critical to secondary procedures. One can apply methylene blue ink with either a 25-gauge needle or Beaver blade to prevent markings from being distorted or washed off during surgical preparation.2
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FIG. 3.3.6 (A and B) A stigma of an inferior cleft nasal deformity and lateral displacement of the alar base on the cleft side 6 years after original repair. (C) The lower lateral cartilage on the cleft side is splayed (black arrowhead). (D) Suspension and repositioning of the lower lateral cartilage with interdomal suture will improve nasal symmetry.
POST-OPERATIVE CARE AND MANAGEMENT OF COMPLICATIONS Post-operative care management for secondary cleft lip repairs is akin to that for primary cleft lip repairs, centering around wound care and infection prevention. It is customary to use saline solution to clean the wound and antibiotic ointment to cover the suture.9 Post-operative care should also take into account scar maturation and should attempt to minimize its severity. Appropriate treatment of surgical scars from secondary procedures thus depends on the surgical operation as well as on characteristics of the scar itself. Steroid injections can also be done if a patient seems to be at risk for hypertrophic or keloid scarring, although a minimum of 12 to 18 months should be allowed for scar maturation before turning to surgical revision to treat aberrant scarring. Hypertrophic scars that have significantly widened can be treated with a diamond-shaped excision or ellipse that facilitates closure in a straight line, whereas a depressed scar can be treated with a bulking technique.1
CASE ILLUSTRATION An 8-year-old girl was born with left unilateral complete cleft lip/palate. After the primary repair, there were still stigmata of a unilateral cleft lip (Fig. 3.3.7). On examination, the scar along the left philtral ridge was widened. This led to a separation at the peak of Cupid’s bow on the cleft side and a discontinuous white roll. In addition, there was under-rotation of the major segment as well as a whistling deformity.
These residual deformities thus required a complete revision. The Cupid’s peak on both cleft and non-cleft sides was marked, and the widened scar in between these two points was excised. The medial segment was re-rotated inferiorly to restore white-lip height. A dermal fat-graft was harvested from the patient’s buttock, deepithelialized, and placed in the free mucosal border to restore lip thickness and correct the whistling deformity. Alternatively, the patient’s whistling deformity could also have been corrected with a V-Y advancement flap.
KEY PRINCIPLES • Secondary cleft lip procedures seek to address deformities that either were not corrected from the initial procedure or have arisen over time due to facial growth. • Secondary cleft deformities may include (but are not limited to) a too-short or too-long white lip, misalignment of vermilio-cutaneous junctions, or abnormal contour of the free mucosal border. • Secondary reconstruction utilizes standard plastic surgery techniques to rectify various stigmata. • The decision to go through with secondary surgery should seek to balance rectification of a patient’s deformity with the psychosocial benefits and consequences of a procedure.
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
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FIG. 3.3.7 Pre-operative (A) appearance. Note widened scar (B), outlined and discontinuation of the white roll (black arrowheads) and whistling deformity (blue arrowhead). Post-operative appearance after complete revision and re-rotation advancement revision (C). Note narrowed scar (D), re-alignment of the white roll (black arrowheads), and correction of whistling deformity (blue arrowhead).
KEY REFERENCES 1. Feldman EM, Koshy JC, Hollier Jr. LH, Stal S. Secondary deformities of the cleft lip, nose, and palate. In: Rodriguez ED, Losee JE, eds. Plastic Surgery. Elsevier; 2013:631–654. 2. Stal S, Hollier L. Correction of secondary cleft lip deformities. Plast Reconstr Surg. 2002;109(5):1672–1681, quiz 1682. 3. Hoffman S, Wesser DR, Calostypis F, Simon BE. The rotation-advancement technique (Millard) as a secondary procedure in cleft lip deformities. Cleft Palate J. 1968;5:37–43. 4. Mulliken JB, LaBrie RA. Fourth-dimensional changes in nasolabial dimensions following rotation-advancement repair of unilateral cleft lip. Plast Reconstr Surg. 2012;129(2):491–498. 5. Coleman SR Facial recontouring with lipostructure. Clin Plast Surg. 1997;24(2):347–367.
6. Hwang K, Kim DJ, Hwang SH. Musculature of the pars marginalis of the upper orbicularis oris muscle. J Craniofac Surg. 2007;18(1):151–154. 7. Mulliken JB. Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg. 2001;108(1):181–194, examination, 195–196. 8. Gosain AK, Fathi AH. Assessment of secondary cleft rhinoplasty using resorbable plates at the age of primary school. J Craniofac Surg. 2009;20(suppl 2):1801–1805. 9. Millard DR, Latham R, Huifen X, Spiro S, Morovic C. Cleft lip and palate treated by presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion (POPLA) compared with previous lip adhesion method: a preliminary study of serial dental casts. Plast Reconstr Surg. 1999;103(6):1630–1644. 10. Mulliken JB, Kim DC. Repair of bilateral incomplete cleft lip: techniques and outcomes. Plast Reconstr Surg. 2013;132(4):923–932.
SYNOPSIS Primary cleft lips are congenital and result from a failure of the medial nasal prominence of the frontonasal process to join with the maxillary process during embryogenesis. Variability in primary cleft lip defects consists of a spectrum based on their severity, such as complete/ incomplete, the width of the cleft, and the alignment of the medial and lateral segments. In primary cleft lips, multiple surgical techniques and methods exist specifically to address the deformity and achieve the goal of symmetrical labial and nasal anatomy and are generally performed before 6 months of age. However, unlike primary cleft deformities, secondary cleft deformities have varied etiologies: they can result from persistent residual deformities, develop over time during facial growth, or form as a sequelae of primary repair techniques. Secondary cleft deformities include (but are not limited to) a too-short or too-long white lip, misalignment of vermilio-cutaneous junctions, or abnormal contour of the free mucosal border. Because secondary deformities are more variable than the primary cleft deformities, both in etiology and presentation, secondary corrective procedures need to be individualized to address the specific deformity for each patient. As such, the goal of secondary cleft lip procedures is to redress specific cleft stigmata that are either residual from the initial surgery or have arisen over time. Secondary deformities can be corrected through application of general plastic surgery principles and techniques. Pre-operative examination of the issue and attentiveness to the timing of the procedure are crucial to a successful secondary repair, though prevention of secondary deformities arising from primary repair should remain paramount. Lastly, it is important to note that because many of these secondary deformities can appear concomitantly in a patient, one must prioritize treatment that will provide the best result to the patient, and utilizing the fewest number of operations.
CLINICAL PROBLEM AND PRINCIPLES OF TREATMENT Clinical problems of secondary cleft lip reconstruction are rooted in stigmata that arise after primary cleft lip surgery. Recognizing the various deformities allows one to be better informed in enacting pre-operative care and planning the course of action for subsequent procedures. This section attempts to cover the most commonly recognizable deformities, which can be sorted into five basic categories: general scarring, white lip, vermilion and mucosa, muscle, and buccal sulcus. The principal stigmata in unilateral clefts are usually related to asymmetry, whereas the stigmata in bilateral clefts are related to disproportion of repaired elements to the face. Both unilateral and bilateral clefts can have
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distortions based on deeper soft tissue elements such as muscle and mucosa. The following section will be organized by discussing commonly seen stigmata as well as common procedures to rectify them. From an operative perspective, one major difference between primary and secondary cleft lip repair is that whereas the former case employs tailored cleft lip repairs with the aim of properly orienting lip and nasal elements, the latter case uses general plastic surgery principles and techniques to achieve its goal. To reiterate, secondary cleft lip procedures aim to fix stigmata that have either arisen from the initial procedure or have arisen with time. Thus for an in-depth discussion of cleft lip repair techniques, please refer to the chapters on primary cleft lip repair (Chapters 3.1 and 3.2).
General Scarring Scarring from the previous surgical intervention(s) should be examined for signs of immaturity, contour changes, and distortion of local structures because worsening scarring may require acute intervention, whereas improving scars should be carefully monitored until they mature.1 Scarring along the philtral ridge on the cleft side may be thick and prominent. Scars are still considered immature within 1 year of the primary repair, and during this time, scar massage is recommended. Occasionally, for a very firm and raised hypertrophic scar, injection with triamcinolone is acceptable and can usually be done during cleft palate repair. However, due to the child’s age, the injection may not be given without general anesthesia. Persistent incisional hypertrophic scars nonresponsive to massage or triamcinolone injections should be excised and reclosed.
Short and Long Lip (Unilateral Cleft) Short and long lip are usually asymmetries seen compared with the normal half of the lip and are stigmata of unilateral clefts, occurring when the repair is not matched to the normal side. A short lip is recognized by having a philtral column on the cleft side that is at least 3 mm shorter than the contralateral non-cleft side, whereas a philtral column that is longer vertically on the cleft side compared with the non-cleft side is characteristic of a long lip. Active underlying scar contracture (that improves over time) may be responsible for producing a minor short lip, whereas more severe cases are usually the result of an improperly designed rotation advancement lip repair. A long lip stigma resulting from a rotation advancement repair is much rarer and is more frequently associated with the Tennison repair.1,2 Short lip deformities are usually associated with the rotationadvancement repair of Millard and similar techniques. A very minimally shortened white lip on the cleft side can be attributable to superficial scar contracture during wound healing, worst at 6 to 12 weeks after
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
A
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B
FIG. 3.3.1 (A) A hyperpigmented scar just cephalad to Cupid’s peak on the cleft side gives the appearance of an under-rotated repair. (B) This scar can be excised with a diamond shape and closed primarily.
primary repair, and will eventually resolve and can be improved with active massaging.2 In the case of a minor short lip deformity (<3 mm), a diamond-shaped scar excision is sufficient in augmenting the lip 2 to 3 mm (Fig. 3.3.1). For short lip deformities that are greater than 3 mm, Z-plasties along the philtral column of the cleft side can lengthen the white lip length. However, oblique and horizontal scars out of line with the philtral column may result. Severe mismatch may be a result of technical error from the primary repair (Fig. 3.3.2), and will require complete takedown and re-rotation and advancement of the cleft lip.1,2 Long lip stigmata have become increasingly rare due to a reduction in the usage of the Tennison repair in favor of the Millard rotationadvancement technique in primary cleft lip repairs.3 Nonetheless, in the case of a long lip deformity, multiple studies have shown that superficially excising tissue from the alar base in an effort to elevate the cleft side is insufficient in redressing the issue. The use of permanent suspension sutures to the periosteum to aid in long lip repair also has mixed results. Thus it is recommended to take down the entire repair and to excise tissue from all dimensions to fully fix the stigma. In bilateral clefts, an overly long and overly wide philtrum will appear disproportionate to the face and is not primarily from asymmetry from left to right side and will be addressed in a later section.
Tight and Wide Lip (Bilateral Clefts) An overly long and overly wide philtrum may result because those nasolabial elements are fast-growing, potentially causing the stigmata to worsen as the child grows (Fig. 3.3.3A). The best way to prevent these stigmata is to perform a primary repair that intentionally minimizes the philtral flap dimensions.4 This correction can be achieved by marking a shorter and narrower philtral flap within the philtrum, then re-raising the smaller philtral flap while discarding the rest of the oversized philtral skin (Fig. 3.3.3B). The medial tubercle is then divided, and the lateral lip elements are re-advanced and raised to meet the new philtral flap. The underlying muscles and mucosa may need to be re-approximated as well. A tight lip may occur in Binder syndrome, where the prolabial elements were abnormally small and underdeveloped, or in overly wide clefts due to inadequate advancement of the lateral lip elements (Fig. 3.3.3C). Another stigma presents as a whistling deformity in the median tubercle; unlike the whistling deformity of unilateral clefts, this defect typically occurs in the midline and tends to be symmetrical. Mild defects can be improved by addressing the mucosa alone (see mucosal section). For moderately tight lips, the bilateral repair can be taken down and the lateral lip elements re-advanced. In cases of severely tight lips, an Abbé flap can be used by borrowing vermilio-mucosal tissues from the lower lip.
FIG. 3.3.2 This shows an under-rotated repair with shortened vertical height of the white lip on the cleft side, as well as a whistling deformity.
Vermilio-Cutaneous Mismatch (Unilateral Clefts) Distortions of the Cupid’s bow can result from vermilio-cutaneous misalignment from the primary repair. Imperfect opposition of the white roll from the non-cleft side to the cleft side can also be visually obvious. These vermilio-cutaneous mismatches can be corrected by a simple Z-plasty or diamond notch excision (Fig. 3.3.4). In more severe cases, it may be necessary to take down the previous lip repair and to repeat the rotation advancement. In addition, as described previously, an Abbé flap can be used to transfer healthy, unscarred tissue from the lower to the upper lip.1,2 Additionally, it is important to note that only vermilion tissue should be used to reconstruct vermilion tissue.
Vermilio-Mucosal Mismatch and Deficiencies (Unilateral and Bilateral Clefts) Usually, the vermilion of the lip on the non-cleft side is thinner than the vermilion on the cleft side; therefore it is very important to align the vermilio-mucosal junctions during the primary repair, including the use of triangular vermilion flaps from the cleft side. However, if there is mismatch present, it may result in a whistling deformity (see the following section); in that case, there is a possibility that no more vermilion is available for correcting the mismatch. For that reason, it is important to ensure proper vermilio-mucosal alignment at the time of primary repair.
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FIG. 3.3.3 (A) A wide philtrum after bilateral cleft lip repair. This results from the fast growth of the philtral elements. (B) After revision. (C) A tight lip with loss of median tubercle after bilateral cleft lip repair. This can result from a wide cleft or from lack of premaxillary tissue, such as in Binder syndrome.
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FIG. 3.3.4 (A) A mismatch, or step-off, in the vermilio-cutaneous junction can be corrected with a simple Z-plasty. The medially based vermilion flap (green-blue) is transposed inferiorly, while the laterally based cutaneous flap (green-black) is transposed superiorly. Care should be taken to align the white roll (blue black lines, B).
Mucosal and Buccal Sulcus Deformity A whistling deformity can result from tissue deficiencies of either the vermilion and/or the mucosa. In cases where the deformity is due to a maldistribution of vermilion, as in the case of a central deficiency with lateral excess, a V-Y advancement can be performed to correct this defect. This can be repaired with a V-Y advancement flap from the posteriorly based buccal mucosa (Fig. 3.3.5). Additional volume can be achieved with dermal fat-grafts taken from the groin as a means of lip augmentation, as demonstrated by Coleman et al., and has been shown with mixed success.2,5 For cases of severe notching deficiency of
the medial tubercle of a unilateral bilateral lip, the Abbé flap is the best choice of treatment due to the need for fresh tissue. Deformities involving the gingivobuccal sulcus can be due to either a surfeit or paucity of mucosal tissue and are commonly seen in bilateral deformities. Examining the buccal sulcus will also reveal how free the lip is from the maxilla; a tethered lip could be the result of scar contracture or a lack of tissue related to the initial deformity.1,2 In the case of surfeit of tissues, one can simply excise excess tissue in the area and re-suspend the mucosa to the maxillary periosteum. When there is insufficient mucosal tissue or abnormal tethering of the lip to the maxilla with loss of the buccal sulcus, the lateral lip can be opened
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
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FIG. 3.3.5 (A) For correction of mucosal deficiency resulting in a mild whistling deformity, a V-incision is planned on the mucosa. (B) A vertical limb of a “Y” is made when the inferior portion is sutured to each other (blue arrowheads).
and re-advanced medially to supply mucosal tissue. The buccal sulcus can also be opened, and mucosal grafts harvested and grafted to cover the defect. Though skin grafts (split-thickness and full-thickness) can be used, buccal mucosa remains the first choice for graft material.1
Muscle Deformities Deformities in muscle—namely the orbicularis oris—can arise from either inadequate muscle re-approximation at the time of the initial surgery or from subsequent tissue dehiscence. For example, dehiscence of the orbicularis oris can present as a bulge on either side of the philtral incision, resulting in a short and widened lip scar. Misalignment of the pars marginalis portion of the orbicularis oris may efface the prominence of the white roll.6 Other muscles in the oronasal region, such as the alar portion of the nasalis, can also be deformed, resulting in abnormal elevation of the alar base upon smiling. Nevertheless, because muscle reconstruction has become a standard part of primary repair, this chapter will not focus on specific surgical techniques that address muscle deformities.
Nasal Repair Even with primary nasal repair, nasal revision will often be necessary. In unilateral cleft lip and nasal deformities, the underlying cleft nasal deformity includes a deviated septum to the non-cleft side, and inferior and lateral displacement of the cleft alar base (Fig. 3.3.6). The goal of primary repair and subsequent revision is to centralize the caudal septum, symmetrize the lower lateral cartilage, and elevate and narrow the alar base to match the non-cleft side. In bilateral cleft lip and nose deformities, there is loss of projection of the nasal tip and an overly wide alar base. In that case, the goal of repair is to lengthen the columella and narrow the alar base. While some have advocated the use of forked flaps, Mulliken has suggested, along with others, that the “columella is in the nose.”7 The nasal projection can be corrected by repositioning the medial crus of the lower lateral cartilage. In both unilateral and bilateral deformities, the alar base should be anchored to the underlying orbicularis oris and maxillary periosteum to keep the alar base from elevating upon the action of smiling. Even with primary nasal repair, nasal deformities can persist, necessitating secondary revisions. The goals of secondary revision are usually to symmetrize the nasal tip and improve nasal projection, and these procedures can be performed with closed or open rhinoplasty techniques. Patients may also need additional support to the lower lateral cartilages.8 The definitive rhinoplasty should be performed after facial growth is complete or near complete, in one’s teenage years. Assessment of the nasal appearance, symmetry, and function (breathing, airflow) should be the same as that for any patient seeking a rhinoplasty; the primary cleft nasal deformity as well as previous nasal surgeries should be taken into account.
PRE-OPERATIVE MANAGEMENT The best time to address secondary deformities is at the time of the primary operation—to take the prophylactic approach and prevent (or at least reduce) these deformities from occurring! Current standard practices have moved away from staged repair and have advocated the use of pre-surgical orthopedics and the completion of cleft nasal repair at the time of primary lip repair.9 Attention should be paid to overcorrecting slow-growing nasolabial features and to undercorrecting fastgrowing ones.4 The goals of these principles serve to minimize the need for secondary revisions. That being said, most cleft lip patients will require secondary procedures at some point in their life.
Timing As is the case with primary cleft lip repair, a surgeon must be able to conceptualize his or her alterations in the context of the fourth dimension; failure to fully take into account nasolabial and facial growth over time can lead to secondary repairs of stigmata that could have been potentially avoided. Regarding the stigmata of repaired unilateral or bilateral cleft lips, the general rule is that fast-growing features become too long or wide, whereas slow-growing features remain short; hence, fast-growing features should be crafted smaller than normal, whereas slow-growing features should be constructed on a slightly larger scale. The only exception to these aforementioned guidelines is the formation of the fast-growing median tubercle, which actually lags behind in a repaired bilateral cleft lip and therefore should be made as full as possible in the initial procedure.7,10 Another consideration regarding the timing of secondary procedures takes into account the severity of an individual’s deformity in the context of his or her psychosocial development. Thus proper secondary procedural intervention will need to address both functional impairments (speech, breathing, or eating) and aesthetic deformities. As peer-to-peer interactions begin to develop in early childhood (beginning from ages 4–5), the risk of teasing by one’s peers can significantly impact a child’s psychosocial development.2 For that reason, it is recommended that all patients should be evaluated for secondary surgery around ages 4 to 5.1 Another common period for secondary surgical correction is during adolescence, due to the changing nature of peer interactions and increased concern regarding one’s own appearance. As adolescents become more capable of vocalizing their own opinions, their wishes should also play a significant role in the decision-making behind secondary corrective procedures.1,2 As is the case with primary repair, marking the lip properly is critical to secondary procedures. One can apply methylene blue ink with either a 25-gauge needle or Beaver blade to prevent markings from being distorted or washed off during surgical preparation.2
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SECTION 3 Cleft and Craniofacial Surgery
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FIG. 3.3.6 (A and B) A stigma of an inferior cleft nasal deformity and lateral displacement of the alar base on the cleft side 6 years after original repair. (C) The lower lateral cartilage on the cleft side is splayed (black arrowhead). (D) Suspension and repositioning of the lower lateral cartilage with interdomal suture will improve nasal symmetry.
POST-OPERATIVE CARE AND MANAGEMENT OF COMPLICATIONS Post-operative care management for secondary cleft lip repairs is akin to that for primary cleft lip repairs, centering around wound care and infection prevention. It is customary to use saline solution to clean the wound and antibiotic ointment to cover the suture.9 Post-operative care should also take into account scar maturation and should attempt to minimize its severity. Appropriate treatment of surgical scars from secondary procedures thus depends on the surgical operation as well as on characteristics of the scar itself. Steroid injections can also be done if a patient seems to be at risk for hypertrophic or keloid scarring, although a minimum of 12 to 18 months should be allowed for scar maturation before turning to surgical revision to treat aberrant scarring. Hypertrophic scars that have significantly widened can be treated with a diamond-shaped excision or ellipse that facilitates closure in a straight line, whereas a depressed scar can be treated with a bulking technique.1
CASE ILLUSTRATION An 8-year-old girl was born with left unilateral complete cleft lip/palate. After the primary repair, there were still stigmata of a unilateral cleft lip (Fig. 3.3.7). On examination, the scar along the left philtral ridge was widened. This led to a separation at the peak of Cupid’s bow on the cleft side and a discontinuous white roll. In addition, there was under-rotation of the major segment as well as a whistling deformity.
These residual deformities thus required a complete revision. The Cupid’s peak on both cleft and non-cleft sides was marked, and the widened scar in between these two points was excised. The medial segment was re-rotated inferiorly to restore white-lip height. A dermal fat-graft was harvested from the patient’s buttock, deepithelialized, and placed in the free mucosal border to restore lip thickness and correct the whistling deformity. Alternatively, the patient’s whistling deformity could also have been corrected with a V-Y advancement flap.
KEY PRINCIPLES • Secondary cleft lip procedures seek to address deformities that either were not corrected from the initial procedure or have arisen over time due to facial growth. • Secondary cleft deformities may include (but are not limited to) a too-short or too-long white lip, misalignment of vermilio-cutaneous junctions, or abnormal contour of the free mucosal border. • Secondary reconstruction utilizes standard plastic surgery techniques to rectify various stigmata. • The decision to go through with secondary surgery should seek to balance rectification of a patient’s deformity with the psychosocial benefits and consequences of a procedure.
CHAPTER 3.3 Secondary Cleft Lip Reconstruction
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FIG. 3.3.7 Pre-operative (A) appearance. Note widened scar (B), outlined and discontinuation of the white roll (black arrowheads) and whistling deformity (blue arrowhead). Post-operative appearance after complete revision and re-rotation advancement revision (C). Note narrowed scar (D), re-alignment of the white roll (black arrowheads), and correction of whistling deformity (blue arrowhead).
KEY REFERENCES 1. Feldman EM, Koshy JC, Hollier Jr. LH, Stal S. Secondary deformities of the cleft lip, nose, and palate. In: Rodriguez ED, Losee JE, eds. Plastic Surgery. Elsevier; 2013:631–654. 2. Stal S, Hollier L. Correction of secondary cleft lip deformities. Plast Reconstr Surg. 2002;109(5):1672–1681, quiz 1682. 3. Hoffman S, Wesser DR, Calostypis F, Simon BE. The rotation-advancement technique (Millard) as a secondary procedure in cleft lip deformities. Cleft Palate J. 1968;5:37–43. 4. Mulliken JB, LaBrie RA. Fourth-dimensional changes in nasolabial dimensions following rotation-advancement repair of unilateral cleft lip. Plast Reconstr Surg. 2012;129(2):491–498. 5. Coleman SR Facial recontouring with lipostructure. Clin Plast Surg. 1997;24(2):347–367.
6. Hwang K, Kim DJ, Hwang SH. Musculature of the pars marginalis of the upper orbicularis oris muscle. J Craniofac Surg. 2007;18(1):151–154. 7. Mulliken JB. Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg. 2001;108(1):181–194, examination, 195–196. 8. Gosain AK, Fathi AH. Assessment of secondary cleft rhinoplasty using resorbable plates at the age of primary school. J Craniofac Surg. 2009;20(suppl 2):1801–1805. 9. Millard DR, Latham R, Huifen X, Spiro S, Morovic C. Cleft lip and palate treated by presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion (POPLA) compared with previous lip adhesion method: a preliminary study of serial dental casts. Plast Reconstr Surg. 1999;103(6):1630–1644. 10. Mulliken JB, Kim DC. Repair of bilateral incomplete cleft lip: techniques and outcomes. Plast Reconstr Surg. 2013;132(4):923–932.