Secondary Cleft Nasolabial Deformities

Abstract

Introduction:

Secondary or residual cleft lip and nasal deformities following primary unilateral or bilateral cleft lip repair are common. Many classification systems have been proposed to describe congenital cleft lip and palate deformities before repair. This article proposes a one-of-a-kind classification system for residual cleft deformities and describes its application to 136 cleft lip revision cases from cleft outreach missions worldwide.

Methods:

Patients’ demographics and deformities were classified preoperatively, and a database of the classification was created. Postoperatively, the type of surgery performed was added to the database and comparison was done using an independent t test.

Results:

Kappa coefficient was 0.92 and showed excellent agreement between the type assigned preoperatively to the patient and the type of procedure done.

Conclusions:

This system proves to provide good descriptions of the deformities, is user friendly, facilitates the planning of the corrective surgical procedure, and enhances the communicative lingo between surgeons and members of cleft multidisciplinary care teams. It is broadly applicable in outreach missions with limited resources and cleft referral centers with considerable load.

Keywords

classification cleft lip cleft revision congenital cleft deformities outreach missions residual deformities

The numerous attempts to find a universally accepted and broadly applicable classification for cleft lip and palate deformities have resulted in a multitude of proposed classification systems. It started with the simple classification by Davis and Ritchie in 1922, evolved to Kernahan's striped Y symbolic classification in 1971, and continued all the way to the complex RPL classification system developed by Schwartz and colleagues in 1993. However, all these published articles focus only on classifications of congenital deformities that have not been operated on and are en route for primary repair. Despite the presence of a multitude of techniques and classifications (Folk et al., 1997; Witt and Marsh, 1997; Smith et al., 2007;) used in the repair of residual palate deformities, little has been published on possible classification schemes for secondary (or residual) cleft lip and nasal deformities following primary unilateral or bilateral cleft lip repairs. A thorough literature review via Pubmed, Medline, Scopus, and EMBASE showed that few classifications relating to residual cleft lip deformities have been published. In 1975, Randall suggested a classification based on the indication for a secondary repair but did not address the anatomic nature of the deformity; eventually, he recognized the subjective nature of some aspects of his method of evaluation. In 1992, Assuncao proposed the VLS classification based on the anatomic nature of the defect. The aim of the VLS classification was to simplify and ease communication among cleft surgeons. Despite its detailed description of deformities, we found the latter classification complex and difficult to memorize and utilize.

Thus, given the lack of adequate literature concerning the classification of residual cleft lip deformities, and the need to have such a tool for planning and managing these conditions, especially in numerous cleft missions to underserved populations, we have devised a new and simple method to group these deformities in a manner that would make it easy to communicate with other cleft specialists in the field, triage patients, and plan a busy operative schedule in cleft missions and busy cleft referral institutions (Eberlin et al., 2008). This proposed classification system was introduced by the one of the authors in his textbook Video Atlas of Cleft Lip and Palate (Hamdan et al., 2013).

Four important factors guided the development of the proposed classification system:

•

The need for a simple and universal description for identifying and differentiating the various types of residual cleft lip/nasal deformities

•

The need for a system that facilitates the planning of the appropriate corrective surgical procedures

•

The lack of data in the literature for such a classification system

•

The need to enhance the communicative lingo among surgeons and other cleft health care providers at the institutional and international levels

Causative factors for residual cleft lip/nasal deformities following primary repairs include infections; hematomas; seromas; lip dehiscence; suture abscess occurring after 2 to 3 weeks of primary repair; technical deficiencies during primary repair, such as improper alignment of the Cupid's bow; insufficient release of the orbicularis oris muscle and inadequate suturing of all of its fibers; inadequate nasal repair; or postoperative trauma (Shaw et al., 2005; Schonmeyr et al., 2015).

Secondary cleft lip/nasal deformities include the following (Hamdan et al., 2013):

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Short skin flaps

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Wide or hypertrophied scars

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Asymmetry of the lip elements

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Lack of fullness of philtral tubercle

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Nasal asymmetry with widened, lateralized, or constricted nostril on the cleft side

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Underprojected nasal tip

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Shortened columella

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Thickened alar lobule

•

Alar valve constriction

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Obliteration of the alar crease

•

Alar rim notching

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Residual redundancy along the anterior soft tissue angle

Thus, this article proposes an original classification system for secondary or residual cleft lip and associated nasal deformities after primary surgical correction. It also presents a clinical application of this new system in 136 patients with cleft lip who had undergone primary repair and present with residual nasolabial deformities.

Materials and Methods

Description of the System

We propose an original classification system for residual or secondary nasolabial cleft deformities. This system is not based on anatomic landmarks, aesthetic subunits, or symbols but rather on the extent of tissue involvement in the residual deformity and correspondingly the extent of complexity of the corrective surgical procedure needed.

Residual cleft lip/nasal deformities have four major elements: skin and subcutaneous tissue, orbicularis oris muscle, vermilion mucosa, and nasal structures. Accordingly, this new classification groups the deformities in an increasing involvement of these lip/nasal elements and the complexity of their repair (Table 1).

Table 1

Classification System for Residual Cleft Lip and Nasal Deformities

Type	Deformity
I	Deformity involves only skin, subcutaneous tissues, and superficial dry mucosa
	Minor revisions requiring skin and subcutaneous tissue mobilization: Z-plasty, V-Y closure, linear excisions
II	Vermilion component orbicularis oris muscle deficiency: requires mucosal and muscle repair without skin or nasal correction.
	Deformity does not involve skin, subcutaneous, or nasal structure. The Cupid's bow is symmetrical in type II.
III	Lip revision involving muscle, skin, and mucosal repair; does not involve nasal repair.
IV	Major lip revision requiring muscle, skin, mucosal, and nasal repair

Following is a detailed description of the four types of residual nasolabial deformities and the corresponding set of possible surgical procedures used for each type:

Type I

Type I involves repair of asymmetries affecting the skin component of Cupid's bow, vermilion fold, upper lip contraction, and superficial dry mucosa. It does not address muscle or nasal repair. The range of deformities and their repair include vermilion border asymmetry (Z-plasty, V-Y flap, or straight-line repair); short upper lip (Z-plasty or diamond-shaped skin excision); long upper lip (excision of a horizontal strip of upper lip skin at its junction with the nostril sill crease with secondary shortening of the upper lip and elevation of the vermilion border to matching latitude with the noncleft vermilion border); philtral ridge defects, including the absence of a philtral column or a wide philtrum with no muscle abnormality (V-Y advancement flaps, Z-plasty, or excision of the excess skin with straight-line repair are three possible treatment options); and deficient gingi-vobuccal sulcus (Z-plasty, local buccal advancement flaps, or buccal mucosal grafts) (Fig. 1A).

Figure 1

A: Type I deformity involving only skin, subcutaneous tissues, and superficial dry mucosa. B: Type II with deficiency of the vermilion component of the orbicularis oris muscle. C: Type III with upper lip muscular, skin, and mucosal deficiency. D: Type IV with upper lip skin, muscular, and mucosal deficiency in addition to nasal deformity requiring repair.

Type II

Type II involves correcting orbicularis oris muscle and/or vermilion mucosal deficiencies without the need for skin or nasal repair. Cupid's bow is symmetrical.

It is very important to note that the building block of successful cleft lip revision is the restoration of the orbicularis oris muscle and its anatomic continuity along the upper lip. Attempts at correcting any muscle deficiency with dermal or fat grafts, AlloDerm (Life-Cell Corporation, Branchburg, NJ), or simple vermilion Z-plasty without simultaneous realignment of the deficient muscle fibers is not considered a functional repair.

Any procedure that does not address the underlying structural defect involving the muscle might enhance the cosmetic appearance of the lip for a few months but eventually results in residual defect due to the abnormal vector of pull created by the malrepaired muscle component.

This defect is commonly seen in the following situations:

•

When the vermilion component of the orbicularis oris muscle fibers is deficient along the repair line in unilateral and bilateral cleft deformities.

•

In V to Y repair, which involves raising a V-shaped mucosal flap with the two peaks of the V incision stopping at the wet-dry vermilion border. It is necessary to excise the scar between the orbicularis oris muscle fibers and repair the muscle in its anatomic position (Hamdan et al., 2013) (Fig. 1B).

Type III

Type III involves muscle, mucosal, and skin repair without any associated nasal work and therefore involves a combination of the techniques used in types I and II. Cleft rhinoplasty is not performed in type III repair because the residual deformity does not involve the nose, nasal repair might compromise the final lip revision repair outcome, or the patient requires a final cleft lip rhinoplasty at a later stage (Hamdan et al., 2013). Depending on the deformity, type III might also be corrected with the use of deepithelialized, medially based submucosal flaps tunneled across the midline to augment the defects (Mulliken, 2000) or with an Abbe flap when there is severe soft tissue loss and scarring at the philtrum (Fig. 1C).

Type IV

Type IV involves muscle, skin, mucosal, and nasal repair. It entails a complete lip/nasal revision similar to all the steps used in the primary cleft repair. The procedure corrects deficiencies in the orbicularis oris muscle; contractures in the mucosa and skin; asymmetries of the nasal tip, alar lobule, and nostril axes; and irregularities of the vermilion border and Cupid's bow (Fig. 1D).

Study Methodology

The study included 136 patients with residual cleft lip or cleft lip and palate deformities, who presented for correction between 2013 and 2015. Patients with acquired noncongenital lip deformities were excluded. All patients were evaluated preoperatively by the authors and by several other surgeons involved in cleft missions worldwide. A total of six to eight missions per year are organized, and five to eight different surgeons are present in every mission. Each surgeon plays an independent role in evaluating and classifying cleft deformities.

The deformities were categorized into one of the four types of the proposed classification system and entered into a cleft database before their revision surgeries. Patients who satisfied the inclusion criteria underwent surgical correction of their residual deformities by the authors as part of several missions for cleft lip repair in specialized and outreach settings. Preoperative and postoperative standardized photographic records were available for all patients. The patients were also triaged according to the proposed classification, and the operating schedule was designed according to the type and complexity of the deformity. With no exceptions, the patients or their guardians gave consent preoperatively.

This study was approved by both the American University of Beirut Institutional Review Board and the Tufts University Institutional Review Board, and the patient data used in this study was anonymized. A database with no identifiers was created for the 136 patients. It included age, sex, mission site (Lebanon, Peru, Guatemala, El Salvador, or Ecuador), anesthesia type (general anesthesia versus local with or without sedation), anesthesia time, and surgical time. Most importantly, the database included the surgical type assigned to each patient during preoperative assessment (called the preoperative type) and the type assigned to each patient postoperatively according to the surgical procedure performed for optimal correction of the deformities (called the “operative intervention type”).

Statistical Analysis

Continuous variables were expressed as mean ± standard deviation and compared using an independent t test or Mann-Whitney U test based on normality. Categorical variables were expressed as counts (percentages) and compared using a χ² test. Measure of agreement between preoperative evaluation and surgical intervention was assessed with the kappa statistic. A two-sided P < .05 was used uniformly to indicate significance. Statistical analysis was conducted with SPSS version 23.0 software (SPSS Inc., Chicago, IL).

Results

Across the four surgical categories, patient demographics and surgical variables differed substantially (Table 2). Ages ranged from 3 months to 45 years old. Sixty-two patients were females (45.6%), and 74 patients were males (54.4%). Of the patients, 47 had their procedure in Lebanon, 32 in Peru, 30 in Ecuador, 17 in Guatemala, and 10 in El Salvador. The surgical time was observed to progressively increase from type I to type IV, and type IV was the longest in duration (P = .003) (Table 3). Younger patients tended to receive general anesthesia; the average age of the general anesthesia group was 8.1 ± 5.6 years. The older patients tended to receive local anesthesia; the average age of the local anesthesia group (with or without sedation) was 19.6 ± 5.7 years (P < .001). Lastly, the kappa coefficient was used to assess the agreement between preoperative evaluation-type assignment and the surgical intervention type assigned and showed an excellent agreement (kappa = 0.92, P < .001) (Fig. 2A through 2D).

Table 2

Type Designated According to the Operative Intervention

Patient Factors	Type I	Type II		Type III		Type IV
Continuous Variables	Mean ± SD	Mean ± SD	P Value^*	Mean ± SD	P Value^*	Mean ± SD	P Value^*
Age (year)	11.8 ± 8.0	14.2 ± 9.4	.400	11.9 ± 7.8	.970	15.1 ± 12.8	.373
Surgery time (hours)	0.7 ± 0.5	1.3 ± 0.8	.035	1.4 ± 0.5	.001	1.6 ± 0.8	.003
Categorical Variables	Counts (%)	Counts (%)	P Value^*	Counts (%)	P Value^*	Counts (%)	P Value^*

Type 1 is the reference category.

Table 3

Correlation Between the Classification Type Assigned Preoperatively and the Surgical Time

	Type I		Type II		Type III		Type IV
	Mean	Standard Deviation	Mean	Standard Deviation	Mean	Standard Deviation	Mean	Standard Deviation
Surgical time (in decimals)	.72_a	.51	.1.29_{a, b}	.84	.1.36_b	.52	.1.58_{b, c}	.84

Figure 2

Preoperative and postoperative results of A: type 1 deformity, B: type II deformity, C: type III deformity, and D: type IV deformity.

Discussion

Residual cleft lip and cleft nasal deformities are not uncommon. Considering the wide range of these secondary deformities, cleft lip revision surgeries remain a challenge to cleft surgeons worldwide. Revision procedures are very common and are performed in an attempt to optimize the structural and/or functional residual deformities manifesting after inadequate primary repair. This makes it worth looking into a classification system that is comprehensive, user friendly, easy to document, and descriptive.

Moreover, it is noticed that inadequate repair of congenital cleft deformities is more prevalent in areas where proper surgical expertise, patient referral, and multidisciplinary approach are absent (Patel et al., 2014). This is clearly manifested in developing and economically challenged countries where health care systems are disorganized and nonspecialized surgeons have to handle these highly technical and specialized procedures.

The authors are involved with year-round cleft missions in underserved areas of the world where surgical expertise and resources, including equipment, funding, and even allocated time per surgical repair, are limited. Grouping deformities plays a pivotal role in planning these missions and allocating resources from a distance. It also helps makes it easier to communicate with specialized and nonspecialized personnel.

This suggested classification is not based on any classification previously published in literature but instead is based on the analysis of the authors’ vast experience of a large number of cleft lip and nose revision of residual deformities. Their personal experience stems from finding the proposed classification to be particularly useful in situations where time and resources are limited.

The system has been applied to a wide spectrum of cases encountered on cleft missions. All residual deformities were adequately described and classified, and the planned surgical correction fitted properly into the proposed set of corrective procedures within each type. It was applied to patients of both sexes, patients ranging in age from a few months old to 45 years old, and those who belong to different ethnic groups. In only 7 of 136 patients did the type assigned on preoperative examination differ from the type corresponding to the surgical procedure performed. In these seven patients, type IV was almost always involved. This is probably because type IV involves nasal repair, and the decision of whether or not to operate on the nose is made intraoperatively in most circumstances.

Patients who received general anesthesia were, on average, 8.1 years old, and those that received local anesthesia were, on average, 19.6 years old. In general, patients younger than 12 years received general anesthesia, and those older than 12 years received only local anesthesia with or without sedation.

The trend of increasing surgical time required from type I to IV is obviously justifiable because the elements involved in the deformity demand more meticulous and prolonged surgical repair with increasing complexity. Therefore, assigning a type to each patient in every mission during the preoperative assessment screening helps give valuable information about the number of patients of each type that will be treated and therefore the average time required. Factoring in the patients’ ages and type of anesthesia required, as previously discussed, generates invaluable information for allocating both the needed personnel and operating room space. This will help to optimize the limited time resources in busy missions.

Conclusion

This article proposes a new classification system for secondary cleft lip and nasal cleft deformities. The system is based on grouping the lip/nasal anatomic subunits into four elements: skin and subcutaneous tissue, vermilion mucosa, orbicularis oris muscle, and nose. Accordingly, this system consists of four types as previously described. This grouping system could be applied to a wide variety of cleft lip revisions. Such an accurately and properly descriptive system facilitates communications among cleft specialists across different countries, in addition to multiple personnel constituting a multidisciplinary cleft team. Each of the four types comprises a set of deformities that require the utilization of specific operative procedures for secondary cleft lip repair. This helps in organizing the approach for cleft lip revisions and allocating the appropriate surgical time and personnel needed per procedure. The system is applicable in outreach cleft programs and large institutional referral centers. It also decreases the financial burden on national health systems and private third-party payers.

Footnotes

Conflict of interest: None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.

Funding resources: No funding was received during the writing or submission of this article.

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