- Email: [email protected]
Nuance in bilateral cleft lip repair
JID: YOTOT
ARTICLE IN PRESS
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology xxx (xxxx) xxx
Nuance in bilateral cleft lip repair Tsung-Yen Hsieh, MD, Mary Roz Timbang, MD, Travis T Tollefson, MD MPH, FACS From the Facial Plastic & Reconstructive Surgery, Department of Otolaryngology-Head and Neck Surgery, UC Davis Medical Center, Sacramento, California Available online xxx
KEYWORDS Cleft lip; Cleft lip repair; Cleft lip-palate; Nasoalveolar molding; Bilateral cleft; Rhinoplasty
The management of patients with bilateral cleft lip and palate requires an understanding of anatomy and a focus on function. Comprehensive care in a collaborative multidisciplinary setting is crucial for the future development of these children. The operative technique begins with meticulous measurement and creation of symmetry to set the foundation for successful repair. In severely wide or asymmetric cases, preoperative orthopedics can be used to narrow the cleft prior to surgery. Consistency of design, anticipation of future growth and critical evaluation of results are essential for a cleft surgeon to obtain the best result. © 2020 Elsevier Inc. All rights reserved.
Introduction Overview of management The management of bilateral cleft lip and associated issues with dentition, speech, swallowing, hearing, and psychosocial factors are best managed by a collaborative approach by an interdisciplinary cleft team.1 Prior to repair, an infant should demonstrate achievement of expected growth milestones as poor weight gain may be associated with systemic abnormalities that require additional evaluation by a pediatrician before surgery. Presurgical planning can be used to optimize surgical outcomes and include lip taping, presurgical infant orthopedics (PSIO), and/or naAddress reprint requests and correspondence: Travis T. Tollefson MD, MPH, FACS, Department of Otolaryngology-Head and Neck Surgery, University of California Davis, 2521 Stockton Blvd., Suite 7200, Sacramento, CA 95817. E-mail address: [email protected]
soalveolar molding (NAM). The treatment of bilateral cleft lip is a multistage process which is more complex with wider deformities. The precision of the initial repair may predict the ultimate outcome as even millimeters of error during the initial surgery could result in permanent stigmata.2 Sequential treatment of lip and columellar length, nasal cartilage position, and dental and skeletal deformities are performed from infancy to adulthood. A surgeon’s sincere commitment to excellence is required in the repair of a patient’s bilateral cleft deformity. The outcome of the surgery will impact this infant for a lifetime. The major obstacles to attaining consistent excellence in outcomes in bilateral cleft lip are a diminutive or short columella, a protruding premaxilla, and the typical flattened and amorphous nasal deformities. This manuscript will outline the author’s current management framework for a child born with a bilateral cleft, including the presurgical management, staging and timing of repair, nuances of repair differences, and postoperative care.
http://doi.org/10.1016/j.otot.2019.12.012 1043-1810/© 2020 Elsevier Inc. All rights reserved.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
2
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020 Table 1
Veau classification of clefts4
I. Clefts of the soft palate II. Clefts of the soft and hard palate, up to the incisive foramen III. Clefts of the soft and hard palate extending unilaterally through alveolus IV. Clefts of the soft and hard palate extending bilaterally through alveolus
Figure 1 (A) bilateral complete cleft lip and palate with (B) prolabium (blue) and lateral lip segment (green). (Color version of figure is available online.)
Definition/anatomy In a bilateral cleft lip (with or without cleft palate), the deformity involves both sides to a variable degree. In more severe presentations, the nose, alar sill, maxillary alveolus, dental structures, premaxilla, and prolabium are affected. The premaxilla is detached from the lateral maxillary segments and is often projected anteriorly. The columella can be extremely short and there may an absent or small anterior nasal spine. The lateral piriform apertures are posteriorly displaced, which contributes to the maxillary deficiency and typical underprojected bilateral cleft lip nasal deformity (Figure 1A). A bilateral cleft lip can present as complete, incomplete, microform or asymmetric. The central lip segments include the prolabium sitting atop the premaxillary segment (Figure 1B). The prolabium is derived embryologically from the paired median nasal processes, which do not contribute to the formation of the orbicularis oris. In a bilateral complete cleft lip, there is no muscle under the prolabial skin, whereas in an bilateral incomplete cleft lip
muscle can migrate into the prolabium. A complete cleft lip extends through the lip and nasal sill. An incomplete cleft lip has a gap in the orbicularis oris and skin but is intact for approximately three-fourth of the lip height. The microform cleft, also known as a form fruste, has a philtral skin groove, minor nasal alar hooding and alar base asymmetry, furrowing of the orbicularis oris muscle, and a notch at the vermilion-cutaneous junction. It does not extend more than one-fourth of the labial height as measured from the normal peak of Cupid’s bow to the nasal sill. The surgeon’s design of the philtral subunit is a key choice in the repair. The size and shape should balance being small enough to grow into a relatively normal size, while having adequate vascularity. The prolabium can vary greatly in size and shape in a bilateral cleft, and a small prolabium can be defined as being less than 6 mm of vertical height. The surgical plan in a case with a small prolabium must address the tendency for a short lip height, when compared to age-appropriate comparative norms.2 , 3 The initial assessment of an infant with bilateral cleft lip should include examination of the lip, premaxilla, prolabium, maxillary alveoli, columella, nasal tip and ala, and palate for symmetry and defect severity. To improve outcomes analysis and communication with others, a system of classification can be chosen. The Veau system defines type I (soft palate cleft), type II (soft and hard cleft palate), type III (unilateral complete cleft lip and palate), and type IV (bilateral complete cleft lip and palate; Table 1).4 The authors favor classifying the cleft severity (wide/typical/narrow), providing a description (complete/incomplete/microform), and stating the structures involved (nasal floor, lip, alveolar cleft, primary, and secondary palate). A wider cleft lip is more difficult to repair and can result in higher wound tension at closure. The severity of a cleft deformity can be defined by the distance between the premaxilla and the lateral maxillary segments. The amount of displacement affects the degree of tension on potential lip repair. Milder cases maintain contact between the premaxilla and the maxillary arches. A displacement greater than 1 cm is considered “wide” or “severe” and usually requires more extensive presurgical planning. A pinch test can help in determining the need for presurgical management, which is performed by pinching bilateral lateral segments of the lip together toward the prolabium in order to
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
3
determine if the closure can be reached without tension. If closure cannot be accomplished without tension, then presurgical orthopedics should be considered.
Presurgical planning Presurgical preparation of a wide bilateral cleft lip can include (1) lip taping, (2) PSIO, which can include NAM, (3) a staged repair or lip adhesion procedure, or (4) in only extreme settings and cases, a premaxillary vomerine osteotomy. Lip taping The goal of lip taping is to narrow the gaps between lip segments by applying pressure to the protruding premaxilla.5 Tape is placed across cleft lip segments with enough tension to encourage tissue expansion. A skin protectant such as Duoderm sheets (ConvaTec, Princeton, NJ) are applied to the cheeks before tape is applied. Lip adhesion Another method used to stage wide bilateral cleft lip repairs is to use a lip adhesion. This surgically brings together the prolabial and lateral lip segments, mucosa, and lip skin without dissection of the orbicularis oris muscle. A second, definitive stage of lip repair is then completed months later. Lip adhesion was originally described by Johanson and Ohlsson in 1960. It can be used to narrow the alveolar cleft, reduce cleft severity for second stage repair, and increase the thickness of the orbicularis oris.2 , 6 Bilateral cleft lip adhesion can thus increase prolabium size and facilitate definitive surgery.7 This procedure is typically performed between 1 and 3 months of age. After 3-4 months, the lip adhesion scar matures, and definitive surgery is then carried out. For infants with significant bilateral cleft lip asymmetry, lip adhesion and NAM are especially useful. Some have also advocated for the use of lip adhesion for wide bilateral cleft lip along with the application of orthopedic pressure to the maxillary and premaxillary arches prior to definitive repair to diminish wound tension.8 Nasoalveolar molding: a presurgical infant orthopedics An infant with a cleft lip-palate can be prepared with an adjustable oral appliance prior to surgery. This process, called NAM, was introduced by Grayson and colleagues, and utilizes a custom device to incorporate nasal molding with the traditional PSIO device. The device is adjusted by the specialized orthodontist to move the premaxilla and lateral alveolar segments.9 This appliance is secured with taping after fitting of the device to the maxilla with molding (Figure 2). The senior author is in favor of NAM in wide bilateral cleft lip patients whose family support structure is adequate for the repeated visits and additional care required for the NAM appliance.
Figure 2 Table 2
Presurgical treatment with NAM appliance.
Aims of the bilateral cleft lip repair
• Reconstruction of a concentric orbicularis oris • Formation of a midline lip tubercle (prominent central red lip fullness with variable presentation) • Creation of age-appropriate alar base width to account for differential widening with growth) • Construction of a philtral column that widens with growth • Establishment of symmetry with consideration of future growth
Premaxillary setback A surgeon must use all other options before considering a premaxillary vomerine osteotomy due to the potential adverse effects. In rare cases, closure of a severe bilateral cleft lip and palate can be enhanced by a premaxillary vomerine osteotomy and setback. This is only used as a technique of last resort and is usually done in older patients with unrepaired, severely wide bilateral cleft lip and palate.8 , 10 Repositioning is accomplished by osteotomy along the vomer after mucoperichondrial flaps are elevated off the septum. The premaxilla is then gently set back and fixated using sutures, wires, orthodontic brackets, or titanium plates and screws. One of the major complications of the procedure is devascularization and ischemia to the premaxillary segment.2 Thus, careful consideration of the risks and benefits of the procedure must be evaluated and discussed prior to proceeding.
Bilateral cleft lip repair technique The authors’ preferred approach to bilateral cleft lip repair is based on a set of aims (Table 2) and uses a 6-step process that involves design and execution, incisions and flap elevation, and closure (Table 3). The priorities of these steps will be outlined in a more detailed fashion.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
4
Table 3
1) 2) 3) 4) 5) 6)
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020 Steps of the bilateral cleft lip repair (TTT)
Markings and design Lateral lip dissection Medial/prolabium dissection Rhinoplasty and nasal septal dissection Closure of muscle layer Closure of mucosa and skin
Markings and design The design of the cleft lip repair is marked with methylene blue tattoos after vasoconstriction with a mixture of 1% lidocaine with 1/100,000 epinephrine. These injections help to minimize bleeding by vasoconstriction, but should be delivered in small amounts and slowly to avoid distorting the lip contours. Injections are performed in a supraperiosteal plane sublabially, into the lip commissures, nasal base, alar rims, and columella. The infraorbital nerve can be blocked with bupivacaine 0.25% with 1:100,000 epinephrine injections or delayed to the end of the procedure for postoperative anesthesia. The philtrum is designed from the prolabial skin of adequate size and vascularity. The designed philtrum will widen during wound healing and facial growth and this should be taken into consideration during the design process. A narrow 4-5 mm philtrum segment lends to a more natural appearance after facial growth. There is usually insufficient vermilion in the prolabium and a white roll that is indistinct or absent. In order to reconstruct the midline lip tubercle, vermilion flaps are derived from the lateral elements.2 , 11 , 12 The prolabium markings begin where the corresponding bilateral columella base points meet the lip-columellar junction. The midline in this junction is marked (subnasale). The lateral-most nasal ala points in the alar-cheek groove are marked (alare). Next, the central vermilioncutaneous junction in the midline of the philtrum is marked. Calipers are used to measure and record the bilateral philtral lip heights (approximately 8-10 mm) and alare-subnasale widths. The philtral column is designed with a slight central taper of around 4-5 mm wide. The design may resemble the shape of a standard necktie with the triangle pointing inferiorly (Figure 3). The lateral prolabial flaps are usually de-epithelized to leave bulk under the philtral column or discarded (Figure 4).
Figure 3 (A) Prolabium lip markings (B) Dots connected for prolabium planned incisions.
Figure 4 Blue shaded area to be de-epithelialized for recreation of philtral columns. (Color version of figure is available online.)
Lateral lip dissection The design of the lateral lip segments is based on the balance of 2 aspects: (1) inclusion of the cutaneous (aka white) roll prior to it tapering off as it approaches the nasal base, and (2) designing equal lip heights for both lateral lip segments and the philtral column height.
Transferring the lip height from the philtrum height to lateral lip height Using a caliper, the lateral lip segment height is measured to be equal to the philtral column height (usually 8-10 mm). The caliper can be started at the point at which the white roll fades from the commissure to the alar base, the
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
5
the alar crease. The skin and hair in this area can help identify this junction. The nasal vibrissae are above this line while the fine velum hairs of the cutaneous lip are inferior to this junction. The point is tattooed at the superior-most lateral lip segment on each side (Figure 5). Planning nasal floor closure The lateral lip incision design is extended into the nose along the piriform aperture to the midpoint of the insertion of the inferior turbinate. Release of the alar base soft tissues and abnormal insertion sites of the cleft musculature from the periosteum of the maxilla is imperative. The tension of the alar base and nasal floor is checked with a double prong retractor placed under the nasal alar base and by rotating/pulling medially. Additional release may be needed if tension is encountered. The planned closure of the nasal floor flaps is marked laterally and medially on the inferior-most septal mucosa. Designing the flaps to close the alveolar clefts and anterior palate are optional.
Incisions and flap elevation
Figure 5 (A) Complete lip markings (B) Dots connected for planned incision design.
Noordhoff point.13 The high point of the lateral lip height should be at the junction of the nasal alar-lip crease. This crease can be seen by pushing the lateral upper lip upward and the natural fold in the skin marked with a tattooing of
Figure 6
After sterilely prepping and draping, a small mouth pack is placed, and the design points are connected with a fine tipped pen (Figure 6). The lateral lip segment incisions are created first to avoid bleeding from the prolabium blurring the designs. The first incision is made perpendicular to the lip in the dry mucosa approximately 2-3 mm proximal to the distal point of the advancement flap incision. This has been designed for the cut-edge of the dry lip vermillion to unfold to fit into the triangular base of the necktie shaped philtral column. This dry vermillion flap is then back elevated in order to create a robust flap to form the central lip tubercle. Second, lateral lip height incision is made accurately and carried up to the inferior turbinate internally and down to the buccal sulcus sublabially. This creates an L-flap which when elevated reveals the orbicularis oris edges.
Illustration with labels and incision design color coded for skin edges to be reapproximated.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
6
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020
Figure 7 Prolabial flap retracted superiorly, demonstrating creation of a central sulcus over the premaxilla by suturing the prolabium to the periosteum of the premaxilla.
The lateral soft tissue segments are separated from the maxilla in a supraperiosteal plane using blunt dissection and scissors. A wider cleft lip requires a more extensive release to minimize wound tension. The double prong retractor is held by the nondominant hand and then placed into the incision. The ring finger on this hand palpates on the infraorbital foramen and orbital rim to protect during dissection. All muscular and soft tissue attachments should be released completely from the alar base to the piriform aperture to allow repositioning and a tension-free closure. This may require the incision to be extended to the midpoint of the insertion of the inferior turbinate with a cautery. These actions are repeated on the opposite side. Perialar incisions (alotomies) are usually unnecessary and pose scarring risks.
Medial prolabium dissection To create the philtrum, the philtral column is incised through the dermis at the planned incision sites while placing tension inferiorly with a small double prong hook on the prolabial mucosa and superiorly with a wide double prong under the nostrils. Next, the prolabial flap is bluntly dissected. The inferior prolabial mucosa is preserved as an inferiorly based flap to create a central labial sulcus (Figure 7). The orbicularis oris muscle is then dissected from the overlying skin of the lateral lip segments for 3-4 mm. Wider clefts will require more undermining. Muscle is minimally elevated from the underlying mucosa just deep to the minor salivary glands. The tension is tested by pushing the lip edges together.
Rhinoplasty and nasal septal dissection The extent and execution of primary rhinoplasty in the setting of bilateral lip repair is at the discretion of the
surgeon. The senior author prefers to perform primary rhinoplasty using a minimal access approach primary rhinoplasty with goals of addressing the (1) poor tip projection, (2) malpositioned lower lateral cartilages, and (3) alar hooding. The dysmorphic lower lateral cartilages are released from the overlying skin-soft tissue envelope with careful dissection through the prolabial dissection. This exposure can be augmented with minimal marginal or rim incisions to access the nasal cartilages. Repositioning of the lower lateral cartilages, if desired, can be achieved using a smaller absorbable suture (e.g. 5-0 Polydiaxone). Similarly, if needed, projection can be increased with use of intradomal suturing to extend columella length via a lateral crural steal effect. This was historically accomplished with the use of forked flaps or V-Y columellar advancement secondary surgery, which are now avoided. To reduce alar hooding, triangular fixation sutures can be placed through and through the alar crease skin. These suspend the lower lateral cartilages into a more anatomic position while retracting the nasal tip cartilages upwards with a Ragnal retractor in each nostril. Residual alar hooding of the soft tissue triangle can be addressed with a Tajima “reverse U” approach.14 Closure of the marginal incisions are done using 5-0 or 6-0 chromic or monocryl sutures. If these incisions are made, nasal conformers should be considered to prevent synechia. Circumferential intranasal incisions should be avoided due to the risk of nasal stenosis. How much septal mucosal dissection is safe? Preservation of the blood supply of the premaxillary segment and lip-columella soft tissue are a priority. On the other hand, guarded dissection of the anterior inferior septum can be done. The submucoperichondrial (septoplasty) plane can be entered and a cotton tipped applicator used to gently release septal flaps to be used in the nasal floor closure. Closure of the anterior alveolar cleft is not often possible.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Figure 8
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
7
Orbicularis oris muscle reconstruction with prolabium retracted superiorly.
An alar cinching suture using a 3-0 or 4-0 resorbable suture is then placed to narrow the alar base to approximately 22-25 mm in a 3-5-month old.2 , 3 Nasal conformers are placed and sewn into the membranous septum using a 3-0 prolene suture for 1 week, at which point they are removed, cleaned, and replaced with a looped tape onto the nasal tip. Parents are taught the nasal stent/ conformer placement/ taping and advised to continue for 6 weeks to assist in molding of the nasal cartilages during the period when the cartilages are still malleable from maternal hormones.15
Closure of orbicularis oris layer To begin closure, the prolabial mucosa is sutured to the exposed premaxilla to recreate the central gingivobuccal sulcus using 5-0 chromic absorbable sutures. Closure of the nasal floor is achieved using a 4-0 chromic, PDS, or vicryl suture to cinch the alar base width with an “alar-cinching suture.” The needle of this suture is placed from deep to superficial just under the lateral alar base soft tissues. Bilateral alar bases are captured with the suture and the knot tied over the nasal spine region of the premaxilla. A caliper is used to set the alare-alare width to approximately 25 mm, based on Farkas anthropometric normative data for age 5-6-month-old infant.2 , 3 This may seem too narrow, but will widen with time (Figure 8).
Closure of mucosa and skin The lip mucosa is closed with 5-0 chromic sutures in the sulcus. Using 3-0 or 4-0 vicryl or polydiaxone sutures, the muscle is closed starting from under the vermilion cutaneous junction moving superiorly. The sutures should be placed orthogonal to the muscle fibers in order to symmetrically orient the lateral muscle segments. Vertical mattress sutures are then placed to evert the orbicularis oris under the tubercle. To accentuate the nasolabial angle, a permanent suture is placed between the superior-most aspect of the orbicularis oris and secured to the periosteum of the nasal spine. The lateral prolabial aspect is de-epithelialized to allow the fullness of the lateral philtrum to add fullness to the philtral columns. The 2 triangular dry vermilion flaps are trimmed to create a tubercle with symmetry of the dry vermilion and closed with 7-0 vicryl on a spatula needle. The wet vermilion is then trimmed to fit into the triangular flaps and 5-0 chromic sutures are used for closure. Finally, the skin edges are closed with deep dermal sutures with 6-0 Monocryl sutures and cyanoacrylate surgical glue (Figure 9).
Controversies There remain areas of controversy in bilateral cleft lip repair. One concern is inhibition of maxillary growth with periosteal elevation which is only based on anecdotal evi-
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
8
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020
Figure 9
Postoperative photograph (A) frontal (B) lateral views.
dence. There is also debate over the repair of the orbicularis oris muscle. Some surgeons advocate for the Millard, Mulliken, or Cutting techniques to create a concentric muscular sphincter while others advocate for the Manchester repair, in which the muscle edges are only joined to the soft tissue of the prolabium in an effort to avoid wound tension and dissection.2 , 8 , 12 , 16-18 The senior author typically avoids the Manchester technique17 in most cases.
Postoperative management The main objectives in the postoperative period include pain control, early feeding, prevention of infection, and avoidance of trauma. Our postoperative pain management protocol starts in the operating room with intraoperative use of bupivacaine infraorbital nerve blocks. Once the patient is out of the operating room, scheduled oral acetaminophen and ibuprofen is provided for pain management. Our practice incorporates use of catheter-tipped syringe feeding for the first 3-7 days to minimize tension on the repair. In addition, a small layer of bacitracin ointment is applied to the mucosal lip closure, but not over the surgical glue. Saline drops are applied to the silicone nasal conformers while they are in place to reduce crusting. These are removed and replaced at 1 week for a total duration of 6 weeks if the patient and parents can tolerate it. To avoid manipulation and trauma to the repair, soft arm restraints are utilized for up to 2 weeks.
over staging or lip adhesions except in severely wide, recalcitrant cases. The technique described is most influenced by a Mulliken-type repair with primary rhinoplasty, while secondary columellar advancement procedures are avoided (columellar forked-flaps and V-Y lip advancement techniques). Consistency of design and critical appraisal of results are essential for a cleft surgeon to give a child the best possible result.
Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-forprofit sectors.
Disclosures Senior Author (TT) has royalties for Thieme Publishing, Inc for textbook, Complete Cleft Care.
Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
References Conclusion The repair of the bilateral cleft lip and palate is a labor of love, requiring meticulous attention to measurements and creation of symmetry, while accounting for differential growth of the nose, lip, and maxilla. Beginner cleft surgeons will benefit from simulation and design practice as well as careful observation of the techniques described. The senior author prefers preparation with NAM
1. American Cleft Palate-Craniofacial Association. Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies, 2009. Revised edition https://acpa-cpf. org/ wp-content/ uploads/ 2017/ 06/ Parameters_Rev_2009_9_.pdf Accessed May 10, 2019. 2. Mulliken JB, Wu JK, Padwa BL: Repair of bilateral cleft lip: Review, revisions, and reflections. J Craniofac Surg 14:609–620, 2003. 3. Farkas LG, Posnick JC, Hreczko TM, et al: Growth patterns of the nasolabial region: A morphometric study. Cleft Palate Craniofac J 29:318–324, 1992.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson 4. Veau V, Récamier J. Bec-de-Lièvre: Formes Cliniques. Chirurgie. Paris: Masson, 1938. 5. Pool R, Farnworth TK: Preoperative lip taping in the cleft lip. Ann Plast Surg 32:243–249, 1994. 6. Johanson B, Ohlsson A: Osteoplasty in the late treatment of harelip and cleft palate. Langenbecks Arch Klin Chir Ver Dtsch Z Chir 295:876–880, 1960. 7. Hamilton R, Graham WP III, Randall P: The role of the lip adhesion procedure in cleft lip repair. Cleft Palate J 8:1–9, 1971. 8. Perlyn CA, Brownstein JN, Huebener DV, et al: Occlusal relationship in patients with bilateral cleft lip and palate during the mixed dentition stage: Does neonatal maxillary arch configuration predetermine outcome? Cleft Palate Craniofac J 39:317–321, 2002. 9. Grayson BH, Cutting CB: Presurgical nasoalveolar orthopedic molding in primary correction of the nose, lip, and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J 38:193–198, 2001. 10. Aburezq H, Daskalogiannakis J, Forrest C: Management of the prominent premaxilla in bilateral cleft lip and palate. Cleft Palate Craniofac J 43:92–95, 2006.
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
9
11. Chen PKT, Noordhoff MS, Liou EJW: Treatment of complete bilateral cleft lip-nasal deformity. Semin Plast Surg 19:329–342, 2005. 12. Mulliken JB: Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg 108:181–194, 2001 195–196. 13. Noordhoff MS: Reconstruction of vermilion in unilateral and bilateral cleft lips. Plast Reconstr Surg 73:52–61, 1984. 14. Tajima S, Maruyama M: Reverse-U incision for secondary repair of cleft lip nose. Plast Reconstr Surg 60:256–261, 1977. 15. Matsuo K, Hirose T: Preoperative non-surgical over-correction of cleft lip nasal deformity. Br J Plast Surg 44:5–11, 1991. 16. Cutting C, Grayson B, Brecht L, et al: Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg 101:630–639, 1998. 17. Manchester WM: The repair of double cleft lip as part of an integrated program. Plast Reconstr Surg 45:207–216, 1970. 18. Hamamoto J: Bilateral cleft lip repairs: The Manchester method and presurgical orthodontic treatment. Cong Anom 24:421–428, 1984.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology xxx (xxxx) xxx
Nuance in bilateral cleft lip repair Tsung-Yen Hsieh, MD, Mary Roz Timbang, MD, Travis T Tollefson, MD MPH, FACS From the Facial Plastic & Reconstructive Surgery, Department of Otolaryngology-Head and Neck Surgery, UC Davis Medical Center, Sacramento, California Available online xxx
KEYWORDS Cleft lip; Cleft lip repair; Cleft lip-palate; Nasoalveolar molding; Bilateral cleft; Rhinoplasty
The management of patients with bilateral cleft lip and palate requires an understanding of anatomy and a focus on function. Comprehensive care in a collaborative multidisciplinary setting is crucial for the future development of these children. The operative technique begins with meticulous measurement and creation of symmetry to set the foundation for successful repair. In severely wide or asymmetric cases, preoperative orthopedics can be used to narrow the cleft prior to surgery. Consistency of design, anticipation of future growth and critical evaluation of results are essential for a cleft surgeon to obtain the best result. © 2020 Elsevier Inc. All rights reserved.
Introduction Overview of management The management of bilateral cleft lip and associated issues with dentition, speech, swallowing, hearing, and psychosocial factors are best managed by a collaborative approach by an interdisciplinary cleft team.1 Prior to repair, an infant should demonstrate achievement of expected growth milestones as poor weight gain may be associated with systemic abnormalities that require additional evaluation by a pediatrician before surgery. Presurgical planning can be used to optimize surgical outcomes and include lip taping, presurgical infant orthopedics (PSIO), and/or naAddress reprint requests and correspondence: Travis T. Tollefson MD, MPH, FACS, Department of Otolaryngology-Head and Neck Surgery, University of California Davis, 2521 Stockton Blvd., Suite 7200, Sacramento, CA 95817. E-mail address: [email protected]
soalveolar molding (NAM). The treatment of bilateral cleft lip is a multistage process which is more complex with wider deformities. The precision of the initial repair may predict the ultimate outcome as even millimeters of error during the initial surgery could result in permanent stigmata.2 Sequential treatment of lip and columellar length, nasal cartilage position, and dental and skeletal deformities are performed from infancy to adulthood. A surgeon’s sincere commitment to excellence is required in the repair of a patient’s bilateral cleft deformity. The outcome of the surgery will impact this infant for a lifetime. The major obstacles to attaining consistent excellence in outcomes in bilateral cleft lip are a diminutive or short columella, a protruding premaxilla, and the typical flattened and amorphous nasal deformities. This manuscript will outline the author’s current management framework for a child born with a bilateral cleft, including the presurgical management, staging and timing of repair, nuances of repair differences, and postoperative care.
http://doi.org/10.1016/j.otot.2019.12.012 1043-1810/© 2020 Elsevier Inc. All rights reserved.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
2
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020 Table 1
Veau classification of clefts4
I. Clefts of the soft palate II. Clefts of the soft and hard palate, up to the incisive foramen III. Clefts of the soft and hard palate extending unilaterally through alveolus IV. Clefts of the soft and hard palate extending bilaterally through alveolus
Figure 1 (A) bilateral complete cleft lip and palate with (B) prolabium (blue) and lateral lip segment (green). (Color version of figure is available online.)
Definition/anatomy In a bilateral cleft lip (with or without cleft palate), the deformity involves both sides to a variable degree. In more severe presentations, the nose, alar sill, maxillary alveolus, dental structures, premaxilla, and prolabium are affected. The premaxilla is detached from the lateral maxillary segments and is often projected anteriorly. The columella can be extremely short and there may an absent or small anterior nasal spine. The lateral piriform apertures are posteriorly displaced, which contributes to the maxillary deficiency and typical underprojected bilateral cleft lip nasal deformity (Figure 1A). A bilateral cleft lip can present as complete, incomplete, microform or asymmetric. The central lip segments include the prolabium sitting atop the premaxillary segment (Figure 1B). The prolabium is derived embryologically from the paired median nasal processes, which do not contribute to the formation of the orbicularis oris. In a bilateral complete cleft lip, there is no muscle under the prolabial skin, whereas in an bilateral incomplete cleft lip
muscle can migrate into the prolabium. A complete cleft lip extends through the lip and nasal sill. An incomplete cleft lip has a gap in the orbicularis oris and skin but is intact for approximately three-fourth of the lip height. The microform cleft, also known as a form fruste, has a philtral skin groove, minor nasal alar hooding and alar base asymmetry, furrowing of the orbicularis oris muscle, and a notch at the vermilion-cutaneous junction. It does not extend more than one-fourth of the labial height as measured from the normal peak of Cupid’s bow to the nasal sill. The surgeon’s design of the philtral subunit is a key choice in the repair. The size and shape should balance being small enough to grow into a relatively normal size, while having adequate vascularity. The prolabium can vary greatly in size and shape in a bilateral cleft, and a small prolabium can be defined as being less than 6 mm of vertical height. The surgical plan in a case with a small prolabium must address the tendency for a short lip height, when compared to age-appropriate comparative norms.2 , 3 The initial assessment of an infant with bilateral cleft lip should include examination of the lip, premaxilla, prolabium, maxillary alveoli, columella, nasal tip and ala, and palate for symmetry and defect severity. To improve outcomes analysis and communication with others, a system of classification can be chosen. The Veau system defines type I (soft palate cleft), type II (soft and hard cleft palate), type III (unilateral complete cleft lip and palate), and type IV (bilateral complete cleft lip and palate; Table 1).4 The authors favor classifying the cleft severity (wide/typical/narrow), providing a description (complete/incomplete/microform), and stating the structures involved (nasal floor, lip, alveolar cleft, primary, and secondary palate). A wider cleft lip is more difficult to repair and can result in higher wound tension at closure. The severity of a cleft deformity can be defined by the distance between the premaxilla and the lateral maxillary segments. The amount of displacement affects the degree of tension on potential lip repair. Milder cases maintain contact between the premaxilla and the maxillary arches. A displacement greater than 1 cm is considered “wide” or “severe” and usually requires more extensive presurgical planning. A pinch test can help in determining the need for presurgical management, which is performed by pinching bilateral lateral segments of the lip together toward the prolabium in order to
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
3
determine if the closure can be reached without tension. If closure cannot be accomplished without tension, then presurgical orthopedics should be considered.
Presurgical planning Presurgical preparation of a wide bilateral cleft lip can include (1) lip taping, (2) PSIO, which can include NAM, (3) a staged repair or lip adhesion procedure, or (4) in only extreme settings and cases, a premaxillary vomerine osteotomy. Lip taping The goal of lip taping is to narrow the gaps between lip segments by applying pressure to the protruding premaxilla.5 Tape is placed across cleft lip segments with enough tension to encourage tissue expansion. A skin protectant such as Duoderm sheets (ConvaTec, Princeton, NJ) are applied to the cheeks before tape is applied. Lip adhesion Another method used to stage wide bilateral cleft lip repairs is to use a lip adhesion. This surgically brings together the prolabial and lateral lip segments, mucosa, and lip skin without dissection of the orbicularis oris muscle. A second, definitive stage of lip repair is then completed months later. Lip adhesion was originally described by Johanson and Ohlsson in 1960. It can be used to narrow the alveolar cleft, reduce cleft severity for second stage repair, and increase the thickness of the orbicularis oris.2 , 6 Bilateral cleft lip adhesion can thus increase prolabium size and facilitate definitive surgery.7 This procedure is typically performed between 1 and 3 months of age. After 3-4 months, the lip adhesion scar matures, and definitive surgery is then carried out. For infants with significant bilateral cleft lip asymmetry, lip adhesion and NAM are especially useful. Some have also advocated for the use of lip adhesion for wide bilateral cleft lip along with the application of orthopedic pressure to the maxillary and premaxillary arches prior to definitive repair to diminish wound tension.8 Nasoalveolar molding: a presurgical infant orthopedics An infant with a cleft lip-palate can be prepared with an adjustable oral appliance prior to surgery. This process, called NAM, was introduced by Grayson and colleagues, and utilizes a custom device to incorporate nasal molding with the traditional PSIO device. The device is adjusted by the specialized orthodontist to move the premaxilla and lateral alveolar segments.9 This appliance is secured with taping after fitting of the device to the maxilla with molding (Figure 2). The senior author is in favor of NAM in wide bilateral cleft lip patients whose family support structure is adequate for the repeated visits and additional care required for the NAM appliance.
Figure 2 Table 2
Presurgical treatment with NAM appliance.
Aims of the bilateral cleft lip repair
• Reconstruction of a concentric orbicularis oris • Formation of a midline lip tubercle (prominent central red lip fullness with variable presentation) • Creation of age-appropriate alar base width to account for differential widening with growth) • Construction of a philtral column that widens with growth • Establishment of symmetry with consideration of future growth
Premaxillary setback A surgeon must use all other options before considering a premaxillary vomerine osteotomy due to the potential adverse effects. In rare cases, closure of a severe bilateral cleft lip and palate can be enhanced by a premaxillary vomerine osteotomy and setback. This is only used as a technique of last resort and is usually done in older patients with unrepaired, severely wide bilateral cleft lip and palate.8 , 10 Repositioning is accomplished by osteotomy along the vomer after mucoperichondrial flaps are elevated off the septum. The premaxilla is then gently set back and fixated using sutures, wires, orthodontic brackets, or titanium plates and screws. One of the major complications of the procedure is devascularization and ischemia to the premaxillary segment.2 Thus, careful consideration of the risks and benefits of the procedure must be evaluated and discussed prior to proceeding.
Bilateral cleft lip repair technique The authors’ preferred approach to bilateral cleft lip repair is based on a set of aims (Table 2) and uses a 6-step process that involves design and execution, incisions and flap elevation, and closure (Table 3). The priorities of these steps will be outlined in a more detailed fashion.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
4
Table 3
1) 2) 3) 4) 5) 6)
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020 Steps of the bilateral cleft lip repair (TTT)
Markings and design Lateral lip dissection Medial/prolabium dissection Rhinoplasty and nasal septal dissection Closure of muscle layer Closure of mucosa and skin
Markings and design The design of the cleft lip repair is marked with methylene blue tattoos after vasoconstriction with a mixture of 1% lidocaine with 1/100,000 epinephrine. These injections help to minimize bleeding by vasoconstriction, but should be delivered in small amounts and slowly to avoid distorting the lip contours. Injections are performed in a supraperiosteal plane sublabially, into the lip commissures, nasal base, alar rims, and columella. The infraorbital nerve can be blocked with bupivacaine 0.25% with 1:100,000 epinephrine injections or delayed to the end of the procedure for postoperative anesthesia. The philtrum is designed from the prolabial skin of adequate size and vascularity. The designed philtrum will widen during wound healing and facial growth and this should be taken into consideration during the design process. A narrow 4-5 mm philtrum segment lends to a more natural appearance after facial growth. There is usually insufficient vermilion in the prolabium and a white roll that is indistinct or absent. In order to reconstruct the midline lip tubercle, vermilion flaps are derived from the lateral elements.2 , 11 , 12 The prolabium markings begin where the corresponding bilateral columella base points meet the lip-columellar junction. The midline in this junction is marked (subnasale). The lateral-most nasal ala points in the alar-cheek groove are marked (alare). Next, the central vermilioncutaneous junction in the midline of the philtrum is marked. Calipers are used to measure and record the bilateral philtral lip heights (approximately 8-10 mm) and alare-subnasale widths. The philtral column is designed with a slight central taper of around 4-5 mm wide. The design may resemble the shape of a standard necktie with the triangle pointing inferiorly (Figure 3). The lateral prolabial flaps are usually de-epithelized to leave bulk under the philtral column or discarded (Figure 4).
Figure 3 (A) Prolabium lip markings (B) Dots connected for prolabium planned incisions.
Figure 4 Blue shaded area to be de-epithelialized for recreation of philtral columns. (Color version of figure is available online.)
Lateral lip dissection The design of the lateral lip segments is based on the balance of 2 aspects: (1) inclusion of the cutaneous (aka white) roll prior to it tapering off as it approaches the nasal base, and (2) designing equal lip heights for both lateral lip segments and the philtral column height.
Transferring the lip height from the philtrum height to lateral lip height Using a caliper, the lateral lip segment height is measured to be equal to the philtral column height (usually 8-10 mm). The caliper can be started at the point at which the white roll fades from the commissure to the alar base, the
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
5
the alar crease. The skin and hair in this area can help identify this junction. The nasal vibrissae are above this line while the fine velum hairs of the cutaneous lip are inferior to this junction. The point is tattooed at the superior-most lateral lip segment on each side (Figure 5). Planning nasal floor closure The lateral lip incision design is extended into the nose along the piriform aperture to the midpoint of the insertion of the inferior turbinate. Release of the alar base soft tissues and abnormal insertion sites of the cleft musculature from the periosteum of the maxilla is imperative. The tension of the alar base and nasal floor is checked with a double prong retractor placed under the nasal alar base and by rotating/pulling medially. Additional release may be needed if tension is encountered. The planned closure of the nasal floor flaps is marked laterally and medially on the inferior-most septal mucosa. Designing the flaps to close the alveolar clefts and anterior palate are optional.
Incisions and flap elevation
Figure 5 (A) Complete lip markings (B) Dots connected for planned incision design.
Noordhoff point.13 The high point of the lateral lip height should be at the junction of the nasal alar-lip crease. This crease can be seen by pushing the lateral upper lip upward and the natural fold in the skin marked with a tattooing of
Figure 6
After sterilely prepping and draping, a small mouth pack is placed, and the design points are connected with a fine tipped pen (Figure 6). The lateral lip segment incisions are created first to avoid bleeding from the prolabium blurring the designs. The first incision is made perpendicular to the lip in the dry mucosa approximately 2-3 mm proximal to the distal point of the advancement flap incision. This has been designed for the cut-edge of the dry lip vermillion to unfold to fit into the triangular base of the necktie shaped philtral column. This dry vermillion flap is then back elevated in order to create a robust flap to form the central lip tubercle. Second, lateral lip height incision is made accurately and carried up to the inferior turbinate internally and down to the buccal sulcus sublabially. This creates an L-flap which when elevated reveals the orbicularis oris edges.
Illustration with labels and incision design color coded for skin edges to be reapproximated.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
6
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020
Figure 7 Prolabial flap retracted superiorly, demonstrating creation of a central sulcus over the premaxilla by suturing the prolabium to the periosteum of the premaxilla.
The lateral soft tissue segments are separated from the maxilla in a supraperiosteal plane using blunt dissection and scissors. A wider cleft lip requires a more extensive release to minimize wound tension. The double prong retractor is held by the nondominant hand and then placed into the incision. The ring finger on this hand palpates on the infraorbital foramen and orbital rim to protect during dissection. All muscular and soft tissue attachments should be released completely from the alar base to the piriform aperture to allow repositioning and a tension-free closure. This may require the incision to be extended to the midpoint of the insertion of the inferior turbinate with a cautery. These actions are repeated on the opposite side. Perialar incisions (alotomies) are usually unnecessary and pose scarring risks.
Medial prolabium dissection To create the philtrum, the philtral column is incised through the dermis at the planned incision sites while placing tension inferiorly with a small double prong hook on the prolabial mucosa and superiorly with a wide double prong under the nostrils. Next, the prolabial flap is bluntly dissected. The inferior prolabial mucosa is preserved as an inferiorly based flap to create a central labial sulcus (Figure 7). The orbicularis oris muscle is then dissected from the overlying skin of the lateral lip segments for 3-4 mm. Wider clefts will require more undermining. Muscle is minimally elevated from the underlying mucosa just deep to the minor salivary glands. The tension is tested by pushing the lip edges together.
Rhinoplasty and nasal septal dissection The extent and execution of primary rhinoplasty in the setting of bilateral lip repair is at the discretion of the
surgeon. The senior author prefers to perform primary rhinoplasty using a minimal access approach primary rhinoplasty with goals of addressing the (1) poor tip projection, (2) malpositioned lower lateral cartilages, and (3) alar hooding. The dysmorphic lower lateral cartilages are released from the overlying skin-soft tissue envelope with careful dissection through the prolabial dissection. This exposure can be augmented with minimal marginal or rim incisions to access the nasal cartilages. Repositioning of the lower lateral cartilages, if desired, can be achieved using a smaller absorbable suture (e.g. 5-0 Polydiaxone). Similarly, if needed, projection can be increased with use of intradomal suturing to extend columella length via a lateral crural steal effect. This was historically accomplished with the use of forked flaps or V-Y columellar advancement secondary surgery, which are now avoided. To reduce alar hooding, triangular fixation sutures can be placed through and through the alar crease skin. These suspend the lower lateral cartilages into a more anatomic position while retracting the nasal tip cartilages upwards with a Ragnal retractor in each nostril. Residual alar hooding of the soft tissue triangle can be addressed with a Tajima “reverse U” approach.14 Closure of the marginal incisions are done using 5-0 or 6-0 chromic or monocryl sutures. If these incisions are made, nasal conformers should be considered to prevent synechia. Circumferential intranasal incisions should be avoided due to the risk of nasal stenosis. How much septal mucosal dissection is safe? Preservation of the blood supply of the premaxillary segment and lip-columella soft tissue are a priority. On the other hand, guarded dissection of the anterior inferior septum can be done. The submucoperichondrial (septoplasty) plane can be entered and a cotton tipped applicator used to gently release septal flaps to be used in the nasal floor closure. Closure of the anterior alveolar cleft is not often possible.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson
Figure 8
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
7
Orbicularis oris muscle reconstruction with prolabium retracted superiorly.
An alar cinching suture using a 3-0 or 4-0 resorbable suture is then placed to narrow the alar base to approximately 22-25 mm in a 3-5-month old.2 , 3 Nasal conformers are placed and sewn into the membranous septum using a 3-0 prolene suture for 1 week, at which point they are removed, cleaned, and replaced with a looped tape onto the nasal tip. Parents are taught the nasal stent/ conformer placement/ taping and advised to continue for 6 weeks to assist in molding of the nasal cartilages during the period when the cartilages are still malleable from maternal hormones.15
Closure of orbicularis oris layer To begin closure, the prolabial mucosa is sutured to the exposed premaxilla to recreate the central gingivobuccal sulcus using 5-0 chromic absorbable sutures. Closure of the nasal floor is achieved using a 4-0 chromic, PDS, or vicryl suture to cinch the alar base width with an “alar-cinching suture.” The needle of this suture is placed from deep to superficial just under the lateral alar base soft tissues. Bilateral alar bases are captured with the suture and the knot tied over the nasal spine region of the premaxilla. A caliper is used to set the alare-alare width to approximately 25 mm, based on Farkas anthropometric normative data for age 5-6-month-old infant.2 , 3 This may seem too narrow, but will widen with time (Figure 8).
Closure of mucosa and skin The lip mucosa is closed with 5-0 chromic sutures in the sulcus. Using 3-0 or 4-0 vicryl or polydiaxone sutures, the muscle is closed starting from under the vermilion cutaneous junction moving superiorly. The sutures should be placed orthogonal to the muscle fibers in order to symmetrically orient the lateral muscle segments. Vertical mattress sutures are then placed to evert the orbicularis oris under the tubercle. To accentuate the nasolabial angle, a permanent suture is placed between the superior-most aspect of the orbicularis oris and secured to the periosteum of the nasal spine. The lateral prolabial aspect is de-epithelialized to allow the fullness of the lateral philtrum to add fullness to the philtral columns. The 2 triangular dry vermilion flaps are trimmed to create a tubercle with symmetry of the dry vermilion and closed with 7-0 vicryl on a spatula needle. The wet vermilion is then trimmed to fit into the triangular flaps and 5-0 chromic sutures are used for closure. Finally, the skin edges are closed with deep dermal sutures with 6-0 Monocryl sutures and cyanoacrylate surgical glue (Figure 9).
Controversies There remain areas of controversy in bilateral cleft lip repair. One concern is inhibition of maxillary growth with periosteal elevation which is only based on anecdotal evi-
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
ARTICLE IN PRESS
JID: YOTOT
8
[mUS6b;January 29, 2020;21:25]
Operative Techniques in Otolaryngology, Vol 000, 2020
Figure 9
Postoperative photograph (A) frontal (B) lateral views.
dence. There is also debate over the repair of the orbicularis oris muscle. Some surgeons advocate for the Millard, Mulliken, or Cutting techniques to create a concentric muscular sphincter while others advocate for the Manchester repair, in which the muscle edges are only joined to the soft tissue of the prolabium in an effort to avoid wound tension and dissection.2 , 8 , 12 , 16-18 The senior author typically avoids the Manchester technique17 in most cases.
Postoperative management The main objectives in the postoperative period include pain control, early feeding, prevention of infection, and avoidance of trauma. Our postoperative pain management protocol starts in the operating room with intraoperative use of bupivacaine infraorbital nerve blocks. Once the patient is out of the operating room, scheduled oral acetaminophen and ibuprofen is provided for pain management. Our practice incorporates use of catheter-tipped syringe feeding for the first 3-7 days to minimize tension on the repair. In addition, a small layer of bacitracin ointment is applied to the mucosal lip closure, but not over the surgical glue. Saline drops are applied to the silicone nasal conformers while they are in place to reduce crusting. These are removed and replaced at 1 week for a total duration of 6 weeks if the patient and parents can tolerate it. To avoid manipulation and trauma to the repair, soft arm restraints are utilized for up to 2 weeks.
over staging or lip adhesions except in severely wide, recalcitrant cases. The technique described is most influenced by a Mulliken-type repair with primary rhinoplasty, while secondary columellar advancement procedures are avoided (columellar forked-flaps and V-Y lip advancement techniques). Consistency of design and critical appraisal of results are essential for a cleft surgeon to give a child the best possible result.
Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-forprofit sectors.
Disclosures Senior Author (TT) has royalties for Thieme Publishing, Inc for textbook, Complete Cleft Care.
Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
References Conclusion The repair of the bilateral cleft lip and palate is a labor of love, requiring meticulous attention to measurements and creation of symmetry, while accounting for differential growth of the nose, lip, and maxilla. Beginner cleft surgeons will benefit from simulation and design practice as well as careful observation of the techniques described. The senior author prefers preparation with NAM
1. American Cleft Palate-Craniofacial Association. Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies, 2009. Revised edition https://acpa-cpf. org/ wp-content/ uploads/ 2017/ 06/ Parameters_Rev_2009_9_.pdf Accessed May 10, 2019. 2. Mulliken JB, Wu JK, Padwa BL: Repair of bilateral cleft lip: Review, revisions, and reflections. J Craniofac Surg 14:609–620, 2003. 3. Farkas LG, Posnick JC, Hreczko TM, et al: Growth patterns of the nasolabial region: A morphometric study. Cleft Palate Craniofac J 29:318–324, 1992.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012
JID: YOTOT
ARTICLE IN PRESS
Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson 4. Veau V, Récamier J. Bec-de-Lièvre: Formes Cliniques. Chirurgie. Paris: Masson, 1938. 5. Pool R, Farnworth TK: Preoperative lip taping in the cleft lip. Ann Plast Surg 32:243–249, 1994. 6. Johanson B, Ohlsson A: Osteoplasty in the late treatment of harelip and cleft palate. Langenbecks Arch Klin Chir Ver Dtsch Z Chir 295:876–880, 1960. 7. Hamilton R, Graham WP III, Randall P: The role of the lip adhesion procedure in cleft lip repair. Cleft Palate J 8:1–9, 1971. 8. Perlyn CA, Brownstein JN, Huebener DV, et al: Occlusal relationship in patients with bilateral cleft lip and palate during the mixed dentition stage: Does neonatal maxillary arch configuration predetermine outcome? Cleft Palate Craniofac J 39:317–321, 2002. 9. Grayson BH, Cutting CB: Presurgical nasoalveolar orthopedic molding in primary correction of the nose, lip, and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J 38:193–198, 2001. 10. Aburezq H, Daskalogiannakis J, Forrest C: Management of the prominent premaxilla in bilateral cleft lip and palate. Cleft Palate Craniofac J 43:92–95, 2006.
Nuance in bilateral cleft lip
[mUS6b;January 29, 2020;21:25]
9
11. Chen PKT, Noordhoff MS, Liou EJW: Treatment of complete bilateral cleft lip-nasal deformity. Semin Plast Surg 19:329–342, 2005. 12. Mulliken JB: Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg 108:181–194, 2001 195–196. 13. Noordhoff MS: Reconstruction of vermilion in unilateral and bilateral cleft lips. Plast Reconstr Surg 73:52–61, 1984. 14. Tajima S, Maruyama M: Reverse-U incision for secondary repair of cleft lip nose. Plast Reconstr Surg 60:256–261, 1977. 15. Matsuo K, Hirose T: Preoperative non-surgical over-correction of cleft lip nasal deformity. Br J Plast Surg 44:5–11, 1991. 16. Cutting C, Grayson B, Brecht L, et al: Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg 101:630–639, 1998. 17. Manchester WM: The repair of double cleft lip as part of an integrated program. Plast Reconstr Surg 45:207–216, 1970. 18. Hamamoto J: Bilateral cleft lip repairs: The Manchester method and presurgical orthodontic treatment. Cong Anom 24:421–428, 1984.
Please cite this article as: Tsung-Yen Hsieh, Mary Roz Timbang and Travis T Tollefson, Nuance in bilateral cleft lip repair, Operative Techniques in Otolaryngology - Head and Neck Surgery, https:// doi.org/ 10.1016/ j.otot.2019.12.012