Abstract
Objective
Accurate classification of cleft lip plays an important role in communication, treatment planning, and comparison of outcomes across centers. Although there is reasonable consensus in defining cleft types, the presence of Simonart's band can make classification challenging. Our objective was to survey cleft care providers to determine what all consider to be Simonart's band, how its presence effects cleft lip classification, and to provide recommendations for standardized nomenclature.
Design
A multiple-choice survey was e-mailed to 1815 members of the American Cleft Palate–Craniofacial Association, assessing each respondent's definition of Simonart's band and its effect on cleft classification. Cleft classification was drawn from the ICD system diagnosis billing codes. Descriptive analysis was performed.
Results
Three hundred seventy-three providers completed the survey (20.5% response), the majority of whom were surgeons (61.5%); 87.1 % agreed with the definition that a Simonart's band is “any soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges.” However, only 41.8% felt that the presence of a Simonart's band rendered a cleft lip incomplete; 54.4% felt that an alveolar cleft was the defining difference between a complete and an incomplete cleft lip. When asked to define the child with a cleft involving the upper lip that extends into the naris but interrupted by a soft tissue bridge located only at the base of the nostril or more internally, without a cleft of the alveolar ridge and palate, 61.4% classified this as an incomplete cleft lip, 32.7% as a complete cleft lip, and 5.9% as an unspecified cleft lip.
Conclusions
Responses revealed wide discrepancy in the classification of cleft phenotypes and in the interpretation of the significance of anatomical components in the classification of a cleft lip. We discuss the difficulty in aligning classification based on unclear definition of terms and variable anatomic parameters. We highlight this issue in the face of a need for comparability in clinical evidence-based practices. To ensure precision and uniformity in cleft classification, we recommend that use of the term “Simonart's band” be abandoned while incorporating a notation of the integrity of the nasal sill into the LAHSHAL system. We propose a uniform definition of incomplete versus complete cleft lip, wherein a cleft lip will be classified as complete in the presence or absence of narrow bands of tissue present at the base of the nasal sill or more internally.
Accurate classification of the cleft lip deformity is critical to the stratification of disease, treatment planning, and comparison of surgical outcomes data over time and across centers. Additionally, assessing the degree of deformity is important in effective communication to other care providers. Whereas consensus often exists with respect to the definition of cleft types, the presence of a Simonart's band can often confuse this classification.
The origin of the term Simonart's band remains unclear. By one report, Simonart's bands are attributed to the German surgeon Gustav Simon, who described a bilateral lip adhesion procedure (Gibson, 1977). The term “Simonart's” may be derived from “Simonartz,” an amalgam of Simon and Artz, a German word for doctor, further supporting this attribution. By another report, however, a 19th-century Belgian surgeon named Simonart described what appeared to be an intrauterine (amniotic) band in a fetus that was born with a cleft lip. He did not, however, specifically describe the bridge of tissue at or within the nasal sill as seen in some cleft lips (Mulliken et al., 2013). The etymology of the term is therefore difficult to ascertain.
Today, the term Simonart's band is used by many to describe “any soft tissue bridge” located “at the threshold of the nostril” or more internally, between the segmented alveolar ridges (Millard, 1976, p. 68, 86). With this definition in mind, it has been suggested that Simonart's bands are the result of incomplete fusion of the oral and nasal epithelium to form the bucconasal membrane and/or subsequent incomplete apoptotic rupture of this membrane to give continuity between the nasal and oral cavities during the 5th to 8th week of embryonic development. These soft tissue bridges have varying constituents and may include epithelial remnants called Epstein's or epithelial pearls, aberrant muscle fibers, neurovascular structures, and/or epithelial mucosal tissue (Kraus et al., 1966; Ross and Johnston, 1979; Sperber, 2001).
Not only is the etymology and definition of the term unclear, its effect on cleft classification is equally inconsistent. Many textbooks appear to adhere to the premise that the presence of a Simonart's band renders a cleft lip incomplete, with most referencing Millard's Cleft Craft and noting, “Anatomically, the cleft lip (
Published manuscripts, however, appear to argue that the presence of a Simonart's band does not result in classifying a cleft lip as incomplete. Da Silva Filho et al. (2006) set out to investigate the prevalence of a Simonart's band in patients with a complete cleft lip and alveolus and complete cleft lip and palate (da Silva Filho et al., 2006). By the manuscript's title and the study's objective, the presence of a Simonart's band does not result in a cleft being classified as incomplete; in fact, the same group previously described their distinction between complete and incomplete cleft lip to be dependent upon whether there is a skeletal cleft of the alveolus (da Silva Filho et al., 1994).
Given this wide discrepancy and the significant implications of inconsistent classification, our objective was to survey cleft care providers to determine their understanding and use of the term Simonart's band, to see how the presence of a Simonart's band affects cleft classification, and to propose a potential standardization of nomenclature.
Methods
A multiple-choice survey questionnaire was e-mailed to the active members of the American Cleft Palate–Craniofacial Association (IRB# PRO14010292). The survey was sent three times over a period of 5 weeks to optimize response rate. Instructions were included in a cover letter, with a link directing participants to the survey. Participation in the survey was voluntary, and participants could exit the survey without submitting their responses at any time. No participant compensation was provided. The survey contained multiple-choice questions as well as free-text responses (Fig. 1). The free-text responses allowed participants to expand upon their responses should they disagree with the premise of the question or feel that their response warranted further explanation. Classification of clefts was drawn from the currently utilized iteration of the International Classification of Disease (
Survey e-mailed to members of the ACPA.
Results
A total of 1815 members of the American Cleft Palate–Craniofacial Association received the questionnaire, with 373 cleft care providers completing the survey (20.5% response rate). Respondents included surgeons (61.5%), orthodontists (21.7%), and speech pathologists (6.2%) (Table 1).
Respondent Role in Cleft Care: Which of the Following Best Describes Your Role in Cleft Care?
Responses revealed wide variation in the classification of cleft phenotypes and demonstrated confusion regarding the definition of a Simonart's band. Respondents were first asked if they agreed with the definition that a Simonart's band represents “any soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges”; 87.1% agreed with this definition. Those who disagreed with this definition were asked to elaborate as to why. Two arguments were most common: (1) that a Simonart's band should not contain muscle and therefore cannot be defined as “any soft tissue bridge,” and (2) that only a band that is greater than a certain width (two respondents noted a definition of greater than 3 mm or greater than 5 mm) makes the cleft an incomplete cleft lip. Free-text responses included: “We have used a size of <5 mm as the cut off, but this is less clear in practice, with some bands being oriented more transversely than in a craniocaudal direction.”
When specifically asked if the presence of a Simonart's band was the defining difference between an incomplete cleft and a complete cleft lip, 41.8% responded affirmatively.
Respondents were then asked if the presence of a skeletal cleft of the alveolus was the defining difference between an incomplete cleft and a complete cleft lip. A slight majority (54.4%) noted that it was; 45.6% disagreed, believing that a complete cleft of the lip can exist without an alveolar cleft, that there exists a spectrum of skeletal clefts, and that a soft tissue bridge defines an incomplete cleft lip.
When asked to “define the child who has a cleft involving the upper lip that extends into the naris but is interrupted with a soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges, and is in combination with a cleft of the alveolar ridge and palate,” 63% classified this as a unilateral complete cleft lip with cleft palate (Fig. 2a). The remainder of respondents classified this phenotype as either a unilateral incomplete cleft lip with cleft palate (28.4%) or as an unspecified cleft lip with cleft palate (8.6%). Moreover, when asked to “define the child who has a cleft involving the upper lip that extends into the naris but is interrupted by a soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges, without a cleft of the alveolar ridge and palate,” 61.4% classified this as an incomplete cleft lip, with the remainder of respondents classifying this phenotype as either a complete cleft lip (32.7%), or an unspecified cleft lip (5.9%) (Fig. 2b).
Distribution of responses to survey questions describing the cleft lip. a: Please define the child who has a cleft involving the upper lip that extends into the naris but is interrupted with a Simonart's band, and is in combination with a cleft of the alveolar ridge and palate, b: Please define the child who has a cleft involving the upper lip that extends into the naris but is interrupted with a Simonart's band, WITHOUT a cleft of the alveolar ridge and palate.
Discussion
Simonart's Band: Effect on Classification
The presence of a so-called Simonart's band confounds the classification of oral clefts. There is historical confusion in regards to the etymology and definition of a Simonart's band (Gibson, 1977; Mulliken et al., 2013). Such confusion persists today. We find that not only is there weak consensus as to the definition of Simonart's band, but there is also no agreement as to how the presence of such a tissue bridge affects the classification of oral clefts. We concede that our survey response rate was low and was provided by a wide range of providers; however, cleft care is a multidisciplinary service, and our goal was to gain input from all members of the team.
Our findings echo the same confusion that exists within the literature as to what is a Simonart's band and how does its presence effect classification of clefts (Wang et al., 2014). Logical arguments may be made as to why such variability exists in the classification of the cleft lip that contains a Simonart's band. From an anatomic perspective, one may argue that any tissue bridge that exists would render a cleft lip incomplete. From the treatment perspective, one might argue that the presence of a Simonart's band does not change the protocol of care. That is, the lip would still be managed surgically as though it were completely cleft and, therefore, should be classified as such. Some argue that the presence of a Simonart's band does not impact the need for alveolar bone grafting; rather, the presence of an alveolar cleft directly impacts the need for such; therefore, the cleft should be classified as complete or incomplete based on this parameter. However, few if any current classification systems utilize the presence of an alveolar cleft to differentiate between a complete and incomplete cleft of the lip (Wang et al., 2014). With incomplete cleft lips, presurgical infant orthopedics (
Review of Current Classification Systems
Wang et al. (2014) recently published a review article that evaluated over 20 cleft lip classification systems. They found that not all cleft lip classification systems distinguish between complete and incomplete clefts, and those who do often base their classification upon a subjective assessment of the extent of clefting rather than an anatomic measurement or landmark, and many fail to provide a definition as to what constitutes one or the other (Santiago, 1969; Kriens, 1989; Friedman et al., 1991; Ortiz-Posadas et al., 2001; Liu et al., 2007). The Elsahy, Harkins, Natsume, and Expression systems all similarly define complete cleft as one where the cleft that involves the nostril floor (Elsahy, 1973), extends into the nostril (Harkins et al., 1962), nasal cavity (Natsume et al., 1984) or proboscis (Koul, 2007). The authors go on to specifically state that it is unclear how some of these systems would classify a patient with a Simonart's band, which they describe as a residual bridge of epithelial tissue superficially connecting the lip or alveolus. Definitions of incomplete cleft classifications also vary in specificity: from “where functional tissue is partially affected and there is insufficient function of affected layers” (Koch et al., 1995) to “those that are less than half of the lip” (Liu et al., 2007). An improved classification system would therefore provide a clear distinction between incomplete and complete cleft lips, provide an objective anatomic basis for such distinction, and be able to be incorporated into the most commonly used existing classification systems.
Recommendations for Standardized Nomenclature and Classification
Clinically, there are over 240 possible combinations of cleft lip with or without cleft palate (Mooney et al., 2015). These include less commonly encountered combinations such as a microform cleft of the alveolus with a partial cleft of the lip on the left side, or a partial bilateral cleft lip with a complete bilateral cleft of the alveolus associated with a complete soft palate cleft.
Our goal is to optimize consistency in classification and increase comparability in the collection, processing, and presentation of orofacial cleft statistics and outcome data. We recommend that the first step would be to abandon use of the term “Simonart's band.” There is no accurate historical evidence regarding its definition, and despite our finding that the majority of respondents agree with Millard's 1976 definition, there is wide variation and no clear trend in the literature regarding its interpretation and its effect on the classification of cleft lips as complete or incomplete (Allori et al., in press-a; Wang et al., 2014).
Next, we propose an objective delineation using anatomic landmarks to distinguish between tissue that bridges the lip versus tissue that bridges the nostril sill. We propose using a line from the alar-facial groove to the columellar-philtral junction. For current classification systems that do distinguish between complete and incomplete cleft lips, this region is most often the watershed between the two (Wang et al., 2014). Any tissue bridge at or above this line would be within the nasal sill (
Examples of complete and incomplete cleft lip defined by soft tissue above and below the nasal sill, a: Complete Cleft Lip. This patient has a soft tissue bridge that lies at or above the line between alar-facial groove to the columellar-philtral junction and, therefore, has a complete cleft lip. b: Incomplete Cleft Lip. This patient would be described as having an incomplete cleft of the lip, using our proposed anatomic landmarks to distinguish between tissue that bridges the lip versus tissue that bridges the nostril sill. This patient has a soft tissue bridge that lies below the line between alar-facial groove to the columellar-philtral junction, and therefore has an incomplete cleft lip.
Integrating this standard into a commonly used existing classification system would be the next step. Perhaps the most thorough, clinically applicable, and simplest classification system is the L-A-H-S-H-A-L system (Kriens, 1989). However, neither the L-A-H-S-H-A-L nor any other current classification scheme addresses tissue variability within the nasal sill (Mooney et al., 2015). Modification of this system to L-NS-A-H-S-H-A-NS-L, wherein NS represents the nasal sill and is used to describe the presence of a soft tissue bridge in this location (at or above a line from the alar-facial groove to the columellar-philtral junction), may allow for more accurate description of this cleft variant. In this schema, it is possible to describe a complete cleft of the lip proper, with or without the presence of tissue bridging the nasal sill.
Finally, there needs to be crossover to the ICD-10 system, which is utilized to code and bill for a cleft lip diagnosis. While ICD-10 has improved significantly from the ICD-9 in terms of increased detail in coding with the purpose of creating a more searchable database for study, this is truer for acquired diagnoses (especially, for example, in the area of fractures of the hand where codes are unique for individual digits and fracture types), but is less true for congenital diagnoses such as cleft lip and palate. With this in mind, we propose modifying the ICD codes.
Currently, the ICD-10 codes for cleft palate and cleft lip are divided within the supergroup Q35 to 37 (Table 2). They may be categorized as “cleft palate” (Q35), “cleft lip” (Q36), or “cleft palate with cleft lip” (Q37). Like ICD-9 (supergroup 749.0 to 749.2), ICD-10 allocates only 16 possible cleft lip and/or palate combinations (Taub and Silver, 2016). However, ICD-10 does not address incomplete versus complete cleft lips, which is a departure from the previous iteration, which did distinguish between incomplete (749.12, 749.14) and complete (749.11, 749.13) clefts of the lip. We concede that classifying a cleft lip and/or palate into one of the 16 options offered by the current ICD classification system for cleft palate and cleft lip is something that varies greatly with user, despite primers on their utilization (Allori et al., in press-b). Therefore, we propose modifying the Q35 to 37 supergroup (Table 3). Importantly, our proposed modification works within the frame of the current code (Q35 to 37), filling current numerical gaps, and would therefore be easy to implement, and increases the options from 16 to 27 which would significantly reduce variability.
ICD-10 Codes for Cleft of the Lip and/or Palate
Proposed Modification to ICD-10 Codes for Cleft of the Lip and/or Palate
Standardization of cleft classification among care providers will enable us to more accurately describe the anatomy, communicate, and compare clinical outcomes in cleft management. By no means would our proposals eliminate all current issues. Rather they would improve upon the most commonly used current systems of classification and coding/billing. Eliminating the use of ambiguous, historical terms such as “Simonart's band,” while utilizing standardized, descriptive classification schema—such as those proposed herein—will allow for more analysis and comparison of evidence-based practices.