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Journal of Cleft Lip Palate and Craniofacial Anomalies 78 July-December 2014 / Vol 1 / Issue 2 Classification of cleft lip and palate: An Indian perspective Karoon Agrawal surgery. CLP has many variations and combinations. This makes it difficult to express correctly. To describe a group of anomalies, classification system or grading system serve as an effective tool of communication. Good classification system allows us to organize a large amount of data into a compreh
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  Journal of Cleft Lip Palate and Craniofacial Anomalies78 July-December 2014 / Vol 1 / Issue 2 Classi fi cation of cleft lip and palate: An Indian perspective Karoon Agrawal surgery. CLP has many variations and combinations. This makes it difficult to express correctly. To describe a group of anomalies, classification system or grading system serve as an effective tool of communication. Good classification system allows us to organize a large amount of data into a comprehensive system and simplifies treatment planning and record keeping. This was realized by Davis and Ritchie in 1922 and presented the first classification of CLP. [1]  Since then many cleft surgeons have presented various classifications based on anatomy, morphology and embryology. There are many based on diagrammatic representation. In this article, attempt has been made to trace the chronological development of classification systems. Balakrishnan, [2]  presented Indian classification in 1975. This still remains a popular cleft classification system in India.The Indian classification has not been published in the format in which it is being used presently. The published article emphasizes that clefts could occur at nine sites and this could be coded for ease of computerization, in the format prevalent then. [2]  We have encountered many children with cleft, which could not be rightly placed in this descriptive classification. Hence, the srcinal classification has been modified and presented with additional features. EVOLUTION OF CLEFT CLASSIFICATIONS Davis and Ritchie presented the first classification for congenital CLP. The alveolar process formed the foundation for groupings: Group I — Prealveolar, Group II - Postalveolar, Group III — Unilateral alveolar cleft and Group IV — Bilateral alveolar cleft. They suggested that the term “hare lip” should be discarded. [1]  This was neither an anatomical nor an embryological classification. Around the same time Brophy (1921-1923) classified the clefts in 16 distinct morphological forms. [3]  However, this was considered too difficult and impractical. Director Professor and Head, Department of Burns, Plastic and Maxillofacial Surgery, Safdarjung Hospital and VMMC,  New Delhi, India Address for correspondence: Dr. Karoon Agrawal,T-23, First Floor, Green Park Main, New Delhi - 110 016, India.E-mail: karoonaparna@gmail.com ABSTRACT Classi fi cation of the cleft has evolved over a century. Many descriptive, diagrammatic, and coding systems have been proposed to be used. However, there are only few which have stood the test of time. One of them is Indian classi fi cation. Indian classi fi cation of cleft lip (CL) and palate proposed in 1975 is a popular classi fi cation in India presently. There are numerous combinations of cleft deformities, and we found that some of them could not be classi fi ed appropriately with the srcinal classi fi cation. The clefts are classi fi ed in three groups: CL as Group 1, cleft palate as Group 2 and Group 3 for combined CL, alveolus and palate in continuity. Originally right, left, midline, and alveolus were abbreviated. To make the classi fi cation wholesome, the srcinal classi fi cation has been revisited and presented with additional features. The basic classi fi cation in three groups remains as srcinal. Additional abbreviations have been added to classify the special situations. Partial, submucosal, Simonart’s band, protruding premaxilla, and microform have been added to the list of abbreviations. This classi fi cation has been used for over 30 years by the author in over 4000 cleft patients. We fi nd it simple to use, versatile enough to classify almost all possible cleft combinations, easy for communication during discussion and convenient to write as diagnosis in patients’ fi les. Easy computer archiving and ef fi cient retrieval of the data are the special features of this classi fi cation. Key words:  Archiving, classi fi cation, cleft lip, cleft palate, coding, Indian, nomenclature, terminology Review Article INTRODUCTION Cleft lip and palate (CLP) is common and interesting craniofacial anomaly in plastic and reconstructive Access this article online Website:  www.jclpca.org DOI:  10.4103/2348-2125.137894 Quick Response Code: [Downloaded free from http://www.jclpca.org on Tuesday, October 07, 2014, IP: 114.121.132.178] || Click here to download free Android application for this journal  Agrawal: Classification of cleft lip and palate 79 July-December 2014 / Vol 1 / Issue 2 Journal of Cleft Lip Palate and Craniofacial Anomalies Veau’s classification in four groups was also far from anatomical and is not in use today. Cleft lip (CL), CL with alveolus, midline clefts and many more were not included in Veau’s classification. [4]  Kernahan and Stark designated the incisive foramen as the dividing point between primary and secondary palates. This correctly described the deformity. [5] Vilar-Sancho classified and coded them based on Greek nomenclature. Lip was represented by “K” (keilos), alveolus by “G” (gnato), hard palate by “U” (urano) and soft palate by “S” (stafilos). Complete cleft was represented in capitals and partial in small letters. “2” was used to represent bilateral, “d” indicated right, “l” indicated left, an “I” indicated incomplete and “o” indicated operated. Being in Greek, it could not be adapted by the rest of the world. It also could not classify many of the clefts. [6]  Harkins et    al  . were appointed by American cleft palate association (ACPA) to design a classification of CLP. They proposed a classification in six groups based on the concept of Kernahan and Stark. This included rare clefts along with the usual clefts. Harkins et al  . divided the groups further based on the extent and sides. [7]  This made the classification quite elaborate and difficult to remember for an average cleft surgeon. Hence, it did not become popular.Dahl divided the clefts in four groups: CL, cleft palate (CP), and unilateral CLP and bilateral CLP. [8]  Spina modified the ACPA classification with incisive foramen as a reference point. Clefts were divided into four groups: Group I — Preincisive foramen clefts, Group II — Transincisive foramen clefts, Group III — Postincisive foramen clefts and Group IV — Rare facial clefts. Each group had unilateral, bilateral and median; each was further subdivided into total and partial. This was adapted by the International Society for Plastic and Reconstructive Surgery. [9]  Sandham added type 5 as “other types of clefts” over Dahl’s proposed classification. [10]  All these classifications are descriptive and not convenient for routine communication. It is difficult to archive in computer and hence data retrieval also may not be easy.Kernahan proposed “Y” classification in nine boxes with nasopalatine foramen as the central point. [11]  It was further modified by Elsahy, Millard, Friedman et    al  ., Smith et    al  . and many more. [12-15]  The modified Kernahan’s “Y” classification represents the cleft deformity exactly as it exists and is very versatile. This has unquestionable utility for the clinicians. [16]  This classification is a diagrammatic or symbolic representation of the cleft deformity and used for documentation or charting very effectively. It cannot be used for writing the diagnosis in the case file, for verbal communication or description in the text format nor can it be used for computer archiving. In the true sense, it is not a classification. It is a symbolic representation of the various cleft deformities as Kernahan himself stated. [17] There have been many attempts to code the various types of CLP. McCabe used electronic data processing system for punching cards. [18]  Santiago coded the CLP for machine recording, [19]  Schwartz et    al  . introduced an RPL system for numerical coding with 0-3 numbers to simplify the representation of the clefts, [20]  Ortiz-Posadas et al.  developed mathematical expression in numerical scores reflecting complexity of clefts, [21]  Castilla and Orioli presented ECLAMC system for numeral coding, [22]  Liu et    al  . published five-digit numerical recording system for CLP [23]  and many more. However, these methods of classification or coding systems did not gain popularity because of its complexity and difficulty in remembering.Kriens, 1989 proposed LAHSHAL, an abbreviated documentation system. Lip (L), alveolus (A), hard palate (H), and soft palate (S) were used to form LAHSHAL. [24]  Later, it was modified to LAHSAL on the recommendation of Royal College of Surgeons UK in 2005. [25]  This was a simplified version of Kernahan’s “Y” classification and had similar shortcomings and limitations.A clock diagram for CLP was introduced by Rossell-Perry, to describe the pathology based on the severity of distortion of nose, lip, and palate. The surgical treatment has been described based on this classification. The author claims to have observed the relationship with the severity and the outcome. [26]  Most of the cleft surgeons may not agree with this observation.A classification based on the (patho-)embryology of the primary and secondary palates has been presented by Luijsterburg et al  . in   2014. The classification is based on the patho-embryological events resulting in various sub-phenotypes of common oral clefts. Patients within the three categories CL/alveolus (CL/A), CL/A and palate, and CP were divided into three subgroups: Fusion defects, differentiation defects, and fusion and differentiation defects. This classification provides new cleft subgroups that may be used for clinical and fundamental research. [27]  However, this classification has little role in clinical practice. [Downloaded free from http://www.jclpca.org on Tuesday, October 07, 2014, IP: 114.121.132.178] || Click here to download free Android application for this journal  Agrawal: Classification of cleft lip and palate Journal of Cleft Lip Palate and Craniofacial Anomalies80 July-December 2014 / Vol 1 / Issue 2 INDIAN CLASSIFICATION AND ITS MODIFICATION The srcinal Indian classification and brief notations as published by Balakrishnan are given in Table 1. Using these groups and brief notations, he described 12 types of possible cleft deformities. Sixteen possible intergroup combinations in a series of over 1000 cleft patients have been described. He used “/” to describe combinations. [2]  While using the classification and dilemma over “/” sign, we replaced it with “+” sign as it was more appropriate sign to express the combination. In srcinal classification system completeness of the cleft is not specified. To mark the partial cleft “P” notation was evolved. If there is no specific notation, it is considered as complete cleft. Thereafter “S” was added to represent “submucosal” cleft. Abbreviations for Simonart’s band, protruding premaxilla (Pmax), and microform, “sb,” “Pmax” and “micro” respectively were added over the years [Table 1]. The abbreviation part of this classification has four parts. Group is abbreviated as “Gp” in the first part. Other parts are well-depicted diagrammatically [Figure 1].Clefts occur in innumerable combinations. It is not possible to enlist all the combinations. However, some of the common clefts along with their short forms based on modified Indian classification are given in Table 2 [Figures 1-6]. CLINICAL EXPERIENCE Balakrishnan’s Indian classification has been used by us in more than 4000 cleft patients over a period of 30 years. With the addition of more abbreviations, it has become more versatile. It is now possible to classify almost all the combinations of the clefts encountered, and it can be represented by a brief notation. On a rough estimate, more than 1000 cleft surgeons in India are using this classification for decades as a testament to its validity.This classification is taught to our residents during 1 st  month of their training. The author has personally taught to more than 40 residents over past 30 years. All of them are able to use this classification very effectively, with a short learning curve though. Table 1: Indian classi fi cation as presented by Prof. Balakrishnan (1975) Cleft typeOriginal abbreviation Cleft lipGp 1Cleft palateGp 2Cleft lip, alveolus and palateGp 3RightR LeftLMidlineMAlveolusAAdditional abbreviationsPartialPSubmucosalSSimonart’s bandsbMicroformmicro Table 2: Abbreviations of different cleft types Type of cleftRightLeftMidlineBilateral (right+left)* CLGp 1RGp 1LGp 1MGp 1R+LCL partialGp 1PRGp 1PLGp 1PMGp 1P R+LCL microformGp 1 micro RGp 1 micro LGp 1 micro MGp 1 micro R+LCL and alveolusGp 1ARGp 1ALGp 1AMGp 1A R+LCL and alveolus with protruding premaxillaGp 1A R+L Pmax [Figures 3a and b]CPGp 2CP partialGp 2PCP submucosalGp 2SCL, alveolus and palateGp 3RGp 3LGp 3MGp 3 R+LCL, alveolus and palate with protruding premaxillaGp 3 R+L Pmax [Figure 6]CL, alveolus and palate with Simonart’s bandGp 3sb RGp 3sb LGp 3sb M**Gp 3sb R+LCL and CP with intact anterior palateGp 1R+Gp 2Gp 1L+Gp 2Gp 1M+Gp 2**Gp 1R+L+Gp 2CL partial and complete CP with intact anterior palateGp 1PR+Gp 2Gp 1PL+Gp 2Gp 1PM+Gp 2Gp 1P R+L+Gp 2CL complete and partial CP with intact anterior palateGp 1R+Gp 2PGp 1L+Gp 2PGp 1M+Gp 2P**Gp 1R+L+Gp 2P *Bilateral cleft has been given as if both sides have similar cleft deformities. There can be different clefts on two sides; which can be classified accordingly using “+” sign, **Author has not come across such clefts, however these are possible situations. [Downloaded free from http://www.jclpca.org on Tuesday, October 07, 2014, IP: 114.121.132.178] || Click here to download free Android application for this journal  Agrawal: Classification of cleft lip and palate 81 July-December 2014 / Vol 1 / Issue 2 Journal of Cleft Lip Palate and Craniofacial Anomalies DISCUSSION The Indian classification has anatomical and embryological basis. This is more logical version of srcinal Davis and Ritchie (1922) [1]  and Dahl classifications. [8]  Incisive foramen is the demarcation between primary and secondary palate. Any cleft anterior to incisive foramen is Group 1 and cleft behind incisive foramen is labeled as Group 2 (Gp 2). When it is involving incisive foramen, both primary, and secondary palate will be cleft. Hence, it has been rightly grouped as Group 3.Clinically too, this classification is quite relevant. All elements of the primary palate, that is, lip, alveolus, anterior palate and nose are repaired together as a single entity. The elements of the secondary palate, that is., hard and soft palate and uvula are repaired together, hence grouped together as Gp 2.This grouping system follows the clinical severity of the cleft. CL is considered simple as it causes mainly Figure 4: Photograph of a child with complete cleft lip on right side with cleft lip, alveolus and palate on left side with Simonart’s band on left side. This has been classi fi ed as Gp 1R + Gp 3L sb. Figure 5: Photograph of a child with complete cleft lip on right side with cleft lip, alveolus and palate on left side. This will be classi fi ed as Gp 1R + Gp 3L. Figure 6: Intraoral picture of a child with bilateral complete cleft lip, alveolus and palate with protruding Premaxilla. This is coded as Gp 3 R + L Pmax. Figure 2: (a and b) Front and intraoral picture of a patient with bilateral complete cleft of lip with complete cleft of hard and soft palate up to incisive foramen. The abbreviated code is Gp 1R + L + Gp 2. ba Figure 3: (a and b) Front and intraoral photographs of a child with bilateral cleft lip and alveolus with protruding Premaxilla. This is coded as Gp 1A R + L Pmax. ba Figure 1: (a and b) Front and intraoral picture of a patient with right sided partial cleft lip with cleft of soft palate. This has been coded as Gp 1PR + Gp 2P. ba [Downloaded free from http://www.jclpca.org on Tuesday, October 07, 2014, IP: 114.121.132.178] || Click here to download free Android application for this journal
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