Nasal deformity in association with a cleft lip is quite characteristic and remains to be a stigma for the individual with this deformity. As a newborn, a cleft lip is the most obvious deformity viewed by average individuals and parents, but in the long-term it is the nose. Most of the previously described corrective techniques for addressing the nasal deformity associated with a cleft lip have focused on the dermal approximation of the adjacent lip by securing the freed cartilages to the skin temporarily and repositioning the nasal tip cartilages. We developed a corrective technique in which the nose is effectively lifted and suspended internally to the radix in a semi-closed manner. Secondary corrections to the nose or lip in childhood should be avoided unless problems in these areas are causing psychological disturbances. In such situations, minimal incisions and/or old lip scars should be used for access. Introduction of scars to the columella must be avoided in children, as this procedure hinders future formal cleft rhinoplasty. Unilateral cleft-associated nasal deformity has been more challenging due to the relative asymmetry compared to the bi-lateral counterpart. Secondary cleft septo-rhinoplasty is considered a challenging operation requiring significant surgical expertise. In adults, an open tip approach is required in addition to the use of sturdy cartilage grafts to augment the columella, tip, and dorsum, and to address functional nasal issues. In cases of severe and or poorly treated bilateral cleft lips and nasal deformities in adults, the nose and columella are first to be reconstructed with prolabial flap followed by an Abbe flap to the lip.
- cleft lip
The nasal deformity in individuals with cleft lip (CLND) is a challenging and controversial topic that has been addressed with a diversity of surgical techniques. Most of the time, the esthetic outcomes have barely been acceptable or dissatisfactory to the surgeons worldwide [1, 2, 3].
Only after spending several years of practice with pediatric facial surgery can one appreciate the difficulty of achieving good results to the nose in cleft lip patients. Earlier in one’s medical practice, closing a wide cleft lip and achieving a good alignment were the main challenges. It has been truly stated: “Cleft lip surgery is essentially an operation to the nose” [4, 5]. Therefore, a diagnosis of “a case of cleft lip” is probably underestimating and inappropriately deficient and lacks the major challenging aspect of the anomaly, namely nasal deformity.
Although it may have a higher incidence in certain geographical and ethnic groups, the anomaly of a cleft lip is quite common globally, and it occurs regardless of maternal nutritional and/or socioeconomic status. Therefore, cleft patients are recognizable by both laypersons and medical professionals. It is interesting that in a certain languages and cultures, the term cleft lip has been translated in lay terms as the “Rabbit lip”.
CLND is the most likely stigma that remains visible despite vigorous and repeated attempts at correction; it has already been established that due to the several factors involved in the nasal cleft patho-anatomy,
We believe that with the currently available developments, it will be relatively easier for the cleft surgeon to obtain results in patients who have symmetric bilateral cleft lip nasal deformity (BCLND) compared to unilateral cleft lip nasal deformity (UCLND).
UCLND is a relatively more common presentation as a primary or secondary case, and it has been investigated and written about far more frequently than its bilateral counterpart BCLND, an observation that can be easily made upon reviewing this subject [3, 6, 7, 8, 9]. The rationale is not specific to either case, and the solutions applied to the former can-not simply be applied on both sides in case of the later or vice versa. The tilt of the tripod in the unilateral cases beginning with the infrastructure, the maxilla, and all of the above layers up to the skin and hence the
Secondary correction to the CLND in childhood or while facial growth is still in progress presents quite different strategies from the case of performing surgery on an adult with CLND. The intrinsic forces responsible for clefting in the first place continue to be active. Therefore, nasal correction in preschool up to teenage years must be considered more or less a symptomatic and a temporary measure for the child, his or her peers, and parents.
This chapter aims to provide an understanding of several aspects of this deformity: 1. The patho-anatomy of the cleft lip associated nasal deformity, 2. Evolution of the surgical techniques used for CLND as primary correction, 3. Concept of
2. Primary cleft lip-nasal correction
Cleft lip and CLND are the results of
Most of the cleft surgeons in the current era attempt to undertake a primary correction of the nasal deformity at the time of lip surgery; however, the techniques vary significantly. It has been a common practice that children undergo another correction at preschool age due to some recurrence of the original deformity. However, it is critical to remember the primary intervention to the nose must be considered as an interceptive procedure using the least number of external incisions as possible. The reason for this choice involves the significant changes that are expected to take place in the following years. Almost all adults who once had a cleft lip, end up having a formal cleft rhinoplasty. Less scarring from earlier interventions leads to a better final nasal shape, and esthetics can be achieved after the completion of facial growth. Many surgeons would have a good lip in results after primary surgery but less than average noses, rarely vice versa. This is also why secondary “cleft rhinoplasty” in adults is not combined with lip revision.
It is almost impossible not to incorporate the cleft lip repair technique or to discuss it while describing the primary nasal correction simply because lips and nose represent a continuation of the midface’s soft tissues. Poorly planned and or poorly executed cleft lip surgery will further compromise the nose and vice versa in addition to threatening long terms facial esthetics. Therefore, cleft lip surgery is considered surgery for life. One might argue that the preoperative severity of the case should be taken into consideration upon judging outcomes and esthetic results; however, no excuses for dehiscence, obvious stitch marks or scars crossing esthetic units, and/or gross lip malalignment exist (Figure 2). When it comes to the use of a specifically described technique, novice reconstructive surgeons generally tend to follow the methods that they were originally taught by their mentors. Since it takes a significant learning curve and duration to eventually master a technique, it is very difficult to change one’s way. The cleft lip is an area that does not permit experimentation
We initially used modified Millard’s techniques due to the fact it allows the surgeon to “cut as you go”. We were trained with Dr. Fisher; however, we were initially hesitant to apply the anatomic subunit principle-based technique since the margin for error is even more limited. However, with time, we gradually adopted this technique due to the appealing concept of limiting scars to the natural seams. In bilateral clefts, we tend to use Dr. Mulliken’s concept of recruiting the lateral crus and building the columella but with the exception that most of the philtral skin is saved. This skin is invaluable for future adult cleft rhinoplasty. We completely condemn the historical idea of initial columellar lengthening using forked flaps because of the associated unnecessary scars. No matter what or after whom the technique is named, the bottom line is that incisions and hence scars should not be placed in locations in which they are going to be obvious with time and will compromise permanent future esthetics for the child. An example is the peri alar incisions, which are routinely used at the time of primary surgery by many surgeons in an attempt to close a large cleft. Such scars are still seen in many adolescents and adults who underwent rhinoplasty, and unfortunately, they cannot be removed.
It is interesting that not too long ago, surgeons began giving more serious consideration to the early cleft nose approach. Historically, different suturing techniques have been suggested and described to secure the surgically dissected cartilages and free them both at the dome area and cephalically. To secure the repositioned LLCs, mattress sutures were used by Tajima in 1977 by holding the lower laterals to the triangular cartilages as part of their described approach to secondary correction of the cleft nose. Kernahan et al. presented their results using the same technique as Tajima and then presented their long-term results of the original approach with some additions. McComb used mattress sutures to reposition the nasal cartilages after undermining nasal skin, securing them externally as bolster sutures. Those mattress sutures depend on dermal resistance to maintain their traction and need to be removed in approximately 5 days. They initially demonstrated the technique in the UCLND and later presented their long-term follow-ups for both unilateral and bilateral clefts. Stenstrom, besides making rim incisions, added a small external incision on the dorsum to lift the affected alar cartilages and secure them to the septal cartilage with non-absorbable sutures. Mulliken’s idea of adding length to the columella mostly for bilateral cleft lip cases was a milestone in addressing the cleft nasal deformity. The technique proved to be equally useful for the UCLND (Figure 3).
Presurgical orthopedic correction, postoperative nostril splints, or the more recent naso-alveolar molding practice all aim at simplifying the repair and reducing tension to yield a better esthetic outcome. Our surgical approach of “
The timing of primary cleft lip surgery may vary from one surgeon to another, and it is also significantly dependent on logistics. The original rule of 10 (hemoglobin of 10 g, age of 10 weeks, and weight of 10 pounds) was only intended to convey these are the minimal prerequisites for undertaking the surgery to ensure relative safety and healing [10, 11]. However, many of these children have combined comorbidities or syndromes. Furthermore, lip-nasal surgery nowadays is a major undertaking in terms of expectations. Most surgeons prefer using diluted adrenaline infiltration to the lip and nose, and heart rate monitoring during surgical steps should be cautiously performed. Although, loupe magnification should be a routine practice for all surgical cases, tissue handling is relatively easier in an older infant. For those reasons, delaying the primary lip-nasal surgery for a few months is indeed worth considering. An older child at the primary surgery provides a sense of safety and reassurance for the surgeon .
In many busy referral centers, the waiting time for surgery might be quite lengthy, and it is not uncommon that even time-sensitive procedures, such as cleft lip and palate repairs, are often delayed. Although this delay is purely a logistical issue it is, however, a fact that one may have to face. In children with cleft lip and palate, we commonly perform an “All in one (AIO) procedure” during which the cleft lip, nasal deformity, cleft palate, and bilateral myringotomies with ventilation tubes are corrected in one longer surgery under anesthesia. This method is a well-known strategy and has been practiced especially in missionary cleft programs [13, 14]. In this process, the initial basic surgical care is taken care of with one admission and limited downtime. The safer age to do such an AIO procedure in our opinion is around 12 months or older. Even though we often do the primary correction to the lip and nose in toddlers (12–24 months) using the nasal lift approach, we can produce quite reproducible stable results to the nose with follow-up to the age of 10 years and without the need for secondary or preschool nasal correction (Figure 11) [15, 16].
3. Secondary nasal correction in childhood1
In children with CLND, preschool age (4–5 years) can be an extremely sensitive time of life in terms of psychological disturbances secondary to their physical distinct appearance and exposure to ridicule by their peers. Children begin to become self-conscious about any deformity they might have around this time. Some might refuse to go to kindergarten or attend school, and they even might not like to be seen in virtual classes. We occasionally have seen children as young as 2 or 3 years with facial anomalies who are upset about having their pictures taken. Their eye contact is usually negligible. Having a photo taken by a stranger makes them feel even more discriminated against from a facial appearance point-of-view. Preoperative photos are essential for planning reasons, documentation, and education; however, sometimes one has to develop the necessary skills of taking pictures especially for photo-sensitive children, and parents’ acceptance is a necessary factor for obtaining these photos.
Most cleft lip children who are brought for secondary or revisionary surgery at preschool age have undergone a primary correction or an attempt to the nose by a primary surgeon with some technique. However, for reasons explained below and due to the accelerated facial growth, a preschool nasal correction is still a frequent request and a frequently performed operation. It is important to point out to the parents that in case the lip requires revision or reconstruction, this process should be addressed at a separate surgery. The exception to this rule would be a revision to the red lip alone. If the surgeon feels and believes that it is the lip contributing to the deformity more than the nose at this stage (infrequent occurrence), it should then be given a priority.
It is crucial here that any nasal esthetic intervention in childhood or early teens should be considered in as closed an approach as possible. Unnecessary scars must be avoided, simply because this is an interceptive procedure. Columellar incisions or alar excisions mean burning your bridges at the time of definitive adult cleft rhinoplasty. It is critical to educate parents about this concept. In other words, one may express: “I can produce greater results now, but they are going to be temporary, and it will cause future definitive nose surgery complicated!”. For the same reasons, our primary approach for lip repair utilizes techniques that use minimal horizontal incisions or extension of incisions to the peri-alar crease regardless of the extent of alveolar gap or severity of the case.
Since the original surgery of primary cleft lip repair attempts at lengthening the lip height in caudal direction while lifting the nose in a cephalic direction or
4. Adult cleft rhinoplasty and reconstruction1
Adulthood for a cleft lip patient presents a different wave of psychosocial difficulty and struggle. Many such adults want to be in a serious social relationship but are often held back due to their facial esthetic dissatisfaction; both males and females are equally concerned with their esthetics. By this time, they have already been through several treatments, including orthognathic surgery. Few patients may have not had the privilege of proper cleft care or were somewhat neglected, and they might still be suffering from speech problems or poor dental alignment, for example. Such patients are not usually interested in taking care of each issue; instead, they might have specific goals, and nasal and lip appearances are the two most common. Therefore, patient priorities must be respected, and options should be given accordingly. However, it is important to educate the patient, and in cases in which orthognathic surgery is a possibility, nasal or lip surgery must be then postponed.
Adult cleft rhinoplasty has many different components, and it is far more complex and challenging compared to the conventional nose job. Very few esthetic rhinoplasty surgeons like to deal with cleft noses. When an adult patient with congenital anomaly consults a rhinoplasty surgeon, they usually have very high expectations. The surgeon in turn knows that he/she will not be able to produce a result anywhere close to their average cosmetic rhinoplasties [6, 17]. Ethnic factors play a major role in the strategy and planning process; however, in general, a more aggressive approach in rebuilding the cartilaginous framework is generally required. This type of surgery is especially true in the Middle-Eastern, Asian, Hispanic, and African noses.
In adults who are also unhappy about their lip shape and asking for it to be revised, this surgery must be deferred until after the nose surgery. It is very often that white lip tissue needs to be recruited to build the columella. Apart from that, alar repositioning often requires incisions extending onto the lip.
We cannot over-stress on the fact that patient’s expectations must be reasonable. The nose itself is an area generally considered prone to claims and conflicts; furthermore, the psychologic disturbance related to the congenital anomaly makes it even more prone to these aspects [18, 19].
Functional aspects need to be analyzed and addressed, apart from significant septal deformities, internal valves Collapse are quite frequent. A facial computed tomography (CT) scan is often helpful in cases in which the primary reason for consultation is a nasal airway, and a consultation with an oto-rhinologist is recommended. UCLND is usually a more challenging deformity due to its significantly more asymmetric nature. On the affected side, the maxilla is usually hypoplastic, the nasal bone often deviates, and the tip is tethered and under-projected. Nostril asymmetry is not fully visible except in a true worm’s eye view, but it is practically what most patients primarily express concern about (Figure 17). The operation aims at balancing the cleft side changes as closely as possible to the normal side while the patient is on the operating table, which obviously can be misjudged and under or overdone due to various factors, including the infiltration fluid, the intraoperative edematous skin, and oozing. The fact that the patient is seen in a supine position from a very short distance from an oblique view causes the decision to often be made while nasal skin is not fully re-draped and closed.
A rib cartilage graft is the most useful and practical type of cartilage that should be considered. It provides quite an ample amount for various areas (Figure 18). Septal cartilage is a second choice; however, it is usually sub-optimal. Conchal cartilage grafts are only useful for minor touch-up procedures. The other advantage of a rib graft is the fact that rectus fascia can also be taken if needed as a supplementary material to wrap around diced cartilage grafts, for the radix area. The following points are mostly made about to UCLND. An open tip approach with full exposure of upper laterals is required most of the time. An inverted V-type incision is recommended with the tip of the flap toward the nasal tip, and some columellar lengthening is achievable with the v–y effect. Septal work follows and then spreader grafts and flaps are used when indicated followed by possible osteotomies. Tip work usually needs a lateral crural steal maneuver to rebuild the columella, especially on the cleft side. Onlay grafting is commonly used for balancing the contour in addition to batten grafts to the alar rim. The final steps include alar repositioning or reshaping as well as the addition of diced cartilage to radix (Figures 19–21).
In BCLND, the main problem remains the symmetrically short columella with an overall under the projection of the entire nose on the profile view in addition to an increased nasal width and flaring of the alae on frontal and basal views. However, what helps to somewhat contribute to the result is the fact that the nose was relatively symmetric initially, indicating that performing symmetric work on the operating table is quite reliable. The milder cases of BCLND can be dealt with a standard esthetic open rhinoplasty approach (a strategy that cannot be applied for UCLND). The more severe cases of BCLND or those patients who have had several revisions to their lips and noses will have associated central lip deficiency usually more horizontally (tightness) in addition to atrophic vermilion and tubercle. This finding is secondary to their several previous surgeries (cleft lip crippled). Due to the significant fibrosis and poor tissue laxity, it becomes almost impossible to lengthen the columella at this stage without recruiting tissues from the lip. A forked flap would not be sufficient to accommodate a good length columellar strut graft. What makes the most sense is to use the entire philtral tissue as a nasally based flap (Prolabial flap) and use it in addition to a vascularized cover over the cartilage graft as the new columella with the complete nasal reshaping . The donor site is usually temporarily covered with a full-thickness skin graft. A second stage reconstruction involves an Abbe flap reconstruction to the philtrum, vermilion, and tubercle units (Figures 22–25). A similar strategy is used in patients who did not have the lip and/or nose repaired earlier in life, and at this point, they would still have an extremely short or non-existent columella with much stiffer nasal tissues (Figure 26).
Both procedures (prolabial flap with nasal reconstruction and Abbe flap reconstruction of secondary defect in the lip) can and have been combined in one surgical stage . This process would avoid the need for a temporary small skin graft but in return will also increase the burden to a quite limited region, especially at the nasolabial angle for which both flaps (prolabial and Abbe) distal edges are being inset or repaired to each other.
A cleft nose deformity correction, whether primary or secondary, is a daunting task to many cleft surgeons.
Parameters, such as presurgical orthopedic manipulation, strict collaborative programs, and compliance, play a major role in cleft surgery outcomes.
In the current era, the surgeon’s satisfaction with BCLND surgery is higher compared to UCLND due to the newer techniques enabling a surgeon to build a less scarred columella meanwhile with the advantage of a preexistent relative nasal symmetry.
The nasal correction aspect is the dominant part of surgery for a cleft lip, and this technique is less forgiving compared to the lip correction relatively speaking.
Cleft surgery should only be done by sub-specialized and dedicated surgeons in the field.
The nasal cantilever technique, which lifts the entire nasal collapsing “tent” and holds it into a fixed base (the nasion), is a new solid concept and promises to be an ultimate primary corrective approach to the “patho-anatomy” in children with cleft nasal–lip deformity.
Primary cleft lip surgery should be planned with a technique that utilizes the least incisions on the lip and nasal skin.
Secondary nasal shape correction in children must be aimed as a temporary interceptive measure to satisfy the child and his or her parents. Therefore, it should be done utilizing most of the existing scars for access with minimal added incision if any.
The cleft nasal–lip surgery tends to be more challenging with time because our earlier minor misjudgments tend to become more pronounced after several years of follow-up. New philosophies and approaches to primary surgery will always be evolving.
The cleft surgeon must possess a combination of pediatric and adult reconstructive facial skills and have a sense of esthetics.
All illustrations and artwork in this chapter are originally of the writer himself, signed by him and or protected with the copyright mark ©.
We would like to acknowledge our patients, their parents for agreeing in sharing their photos for the sake of advancement in research and education.*
Randall P. History of cleft lip nasal repair. The Cleft Palate-Craniofacial Journal. 1992; 29:527-530
Jou L, Wong F, Mardini S, Chen Y, Noordhoff S. Assessment of bilateral cleft lip nose deformity: A comparison of results as judged by cleft surgeons and laypersons. Plastic and Reconstructive Surgery. 2002; 110(3):733-738
Fisher DM, Mann RJ. Case reports, a model for the cleft lip nasal deformity. Plastic and Reconstructive Surgery. 1998; 101:1448-1456
Mulliken JB. Repair of bilateral complete cleft lip and nasal deformity: State of the art. The Cleft Palate-Craniofacial Journal. 2000; 37:342
Mulliken JB. Primary repair of bilateral complete cleft lip and nasal deformity. Plastic and Reconstructive Surgery. 2001; 108:181-194
Fisher MD, Fisher DM, Marcus JR. Correction of the cleft nasal deformity: From infancy to maturity. Clinics in Plastic Surgery. 2014; 41(2):283-299
Kernahan DA, Bauer BS, Harris GD. Experience with the Tajima procedure in primary and secondary repair in unilateral cleft-lip nasal deformity. Plastic and Reconstructive Surgery. 1980; 66(1):46-53
Abdulrauf B. The nasal lift technique in primary cleft lip surgery. The Cleft Palate-Craniofacial Journal. 2020; 58(7):919-924. DOI: 10.1177/1055665620964708
Wang T. Secondary rhinoplasty in unilateral cleft nasal deformity. Clinics in Plastic Surgery. 2010; 37(2):383-387
Wilhelmsen H, Musgrave R. Complications of cleft lip surgery. The Cleft Palate Journal. 1966; 3:223-231
Millard DJ, editor. Cleft Craft. Boston: Little, Brown; 1976. pp. 69-74
Fillies T, Homann C, Meyer U, et al. Perioperative complications in infant cleft repair. Head & Face Medicine. 2007; 3:9
Davies D. The one-stage repair of unilateral cleft lip and palate. Plastic and Reconstructive Surgery. 1966; 38:129-136
Guneren E, Canter HI, Yildiz K, et al. One-stage cleft lip and palate repair in an older population. The Journal of Craniofacial Surgery. 2015; 26:e426-e430
Abdulrauf B. An ultimate method for cleft nasal deformity correction at primary lip surgery: Innovative concepts and review. Archives of Oral and Maxillofacial Surgery. 2020; 3(1):36-49
Abdulrauf BMI, Mater ME. Single Stage Cleft Lip and Palate Repair In Toddlers: Retrospective Review of Feasibility and Operative Experience. Journal of Craniofacial Surgery. 2021 Sep 14. doi: 10.1097/SCS.0000000000008108. Epub ahead of print. PMID: 34519704
Abdulrauf B. The reconstructive plastic surgeon vs the aesthetic plastic surgeon: Perspective, International Journal of Surgery and Clinical Practice. 2020; 2(1):1-2
Abdulrauf B. Optional surgery? What a surgeon must not miss! Editorial. Online Journal of Otolaryngology and Rhinology. 2021; 4(4):1-4
Abdulrauf B. Consultation is about DRPPP. Plastic and Reconstructive Surgery Global Open. 2021; 9(4):Pe3521
Ujam A, Vig N, Nasser N. Combined correction of the nasal tip and upper lip in bilateral cleft lip patients: A novel approach. International Journal of Pediatric Otorhinolaryngology. 2019; 126:109593
Lun-Jou L, Alex K, Yu-Ray C. Simultaneous reconstruction of the secondary bilateral cleft lip and nasal deformity: Abbe flap revisited. Plastic and Reconstructive Surgery. 2003; 112(5):1219-1227
- None of the secondary correction or adult patients have shown had their initial infancy surgeries performed by the author, rather, they only presented for a second opinion concerning revisionary and reconstructive surgery.