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Classification of Cleft Lip/Palate: Then and Now

Article  in  The Cleft Palate-Craniofacial Journal · September 2015


DOI: 10.1597/14-080 · Source: PubMed

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The Cleft Palate–Craniofacial Journal 00(00) pp. 000–000 Month 2015
Ó Copyright 2015 American Cleft Palate–Craniofacial Association

ORIGINAL ARTICLE

Classification of Cleft Lip/Palate: Then and Now


Alexander C. Allori, M.D., M.P.H., John B. Mulliken, M.D., John G. Meara, M.D., D.D.S., M.B.A., Stephen
Shusterman, D.M.D., Jeffrey R. Marcus, M.D., on behalf of the CleftKit Collaboration

Cleft lip and/or palate (CL/P) is phenotypically diverse, making classification difficult. This article
explores the evolution of ideas regarding CL/P classification and includes the schemes
described by Davis and Ritchie (1922), Brophy (1923), Veau (1931), Fogh-Andersen (1943),
Kernahan and Stark (1958), Harkins et al. (1962), Broadbent et al. (1968), Spina (1973), and others.
Based on these systems, a longhand structured form is proposed for describing CL/P in a way
that is clear, comprehensive, and consistent. A complementary shorthand notation is also
described to improve the utility and convenience of this structured form.

KEY WORDS: classification, CleftKit, cleft lip, cleft palate, nomenclature, nosology, ontology,
taxonomy, terminology

As part of the journal’s ‘‘Then & Now’’ series reflecting has never been greater. Modern medicine’s complex
on the development and maturation of cleft care over the landscape requires a common language understand-
past 50 years, this article explores the rich history of able by clinicians, patients, hospital administrators,
classification systems used to describe and categorize the payors, government agencies, and other stakeholders.
diverse phenotypes collectively called cleft lip and/or As a step toward a universal vocabulary, this article
palate (CL/P). concludes with a contemplation of the lessons learned
Inaccurate and inconsistent classification continues from each historic attempt at cleft classification and
to be a problem today. Arguably, the need for a with a synthesis of the central precepts that have
universal, simple, and practical classification scheme emerged from this experience. Based on this, we
present a detailed, longhand structured form to help
guide accurate and complete phenotypic description,
Dr. Allori is Title, Division of Plastic, Maxillofacial & Oral Surgery, as well as a corresponding shorthand notation to
Duke University Hospital & Children’s Health Center, Durham, improve practicality and versatility in implementation.
North Carolina; Title, Department of Plastic & Oral Surgery, Boston
Children’s Hospital, Harvard Medical School, Boston, Massachusetts;
and Director, Duke Program for Effectiveness, Quality, Innovation & SPLITTING HARES
Policy in Health Care (EQUIP), Duke University School of Medicine,
Durham, North Carolina. Dr. Mulliken is Title, Division of Plastic, It is difficult to trace the etymologic history of CL/P. The
Maxillofacial & Oral Surgery, Duke University Hospital & Children’s
Health Center, Durham, North Carolina. Dr. Meara is Title, condition is as old as mankind itself, and it has been
Department of Plastic & Oral Surgery, Boston Children’s Hospital, described in different ways by the people of every era. Many
Harvard Medical School, Boston, Massachusetts. Dr. Shusterman is
Title, Department of Dentistry, Boston Children’s Hospital, Harvard
of the early written accounts compare cleft lip with animal
Dental School, Boston, Massachusetts. Dr. Marcus is Title, Division features, most popularly ‘‘harelip’’ (labium leporinum) due
of Plastic, Maxillofacial & Oral Surgery, Duke University Hospital & to the resemblance of the mouth of the hare (genus Lepus).
Children’s Health Center, Durham, North Carolina.
Related funding support: Plastic Surgery Foundation 2013 Pilot
French Renaissance surgeon Pierre Franco (1505 to
Research Grant 273938 (‘‘Development of Cleft Lip and Palate 1578)—famed colleague and rival of Ambroise Paré (c.
Outcome Measures’’). 1510 to 1590)—used both terms levre fendu (‘‘split lip’’) and
Presented at the 71st Annual Meeting of the American Cleft Palate–
Craniofacial Association, Allori AC, et al., ‘‘Toward Realtime Patient-
dents de lièvre (‘‘hare teeth’’) in his works Petit Traité (1556)
Centered Outcomes Assessment and Continuous Quality Improve- and the Traité des Hernies (1561), notably the first surgical
ment: A CleftKit Progress Report,’’ Indianapolis, Indiana, March 29, textbooks printed on a Gutenberg press (Franco, 1976).
2014.
Submitted April 2014; Revised January 2015; Accepted March Three centuries later, Joseph-François Malgaigne (1806 to
2015. 1865) still referred to cleft lip as bec-de-lièvre (‘‘beak of the
Address correspondence to: Dr. Alexander C. Allori, Program for hare’’; Malgaigne and Ivy, 1976).
Effectiveness, Quality, Innovation & Policy in Health Care (EQUIP),
Assistant Professor of Surgery, Duke Cleft & Craniofacial Center, A clarion call for abandoning the use of the term
Division of Plastic, Maxillofacial & Oral Surgery, Duke University harelip was sounded at the meeting of the American
Hospital & Children’s Health Center, DUMC 3974, Room 110 Baker Medical Association in St. Louis in 1922, where John
House, 200 Trent Drive at Erwin Road, Durham, NC 27710. E-mail
alexander.allori@duke.edu. Staige Davis of Baltimore and Harry P. Ritchie of St.
DOI: 10.1597/14-080 Paul declared, ‘‘the term ‘harelip’ should be discarded,

0
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

and [instead] the deformity thus named should be (irrespective of the integrity of the lip or palate) should
called ‘congenital cleft of the lip’’’ (Davis and Ritchie, be categorized exclusively as group III. Reading further,
1922). Terminological change came slowly; the con- it is apparent that Davis and Ritchie recognized this as a
vention of referring to cleft lip as ‘‘harelip’’ persisted point of uncertainty (if not weakness) in their classifi-
well into the 20th Century in many languages, e.g., cation scheme:
Victor Veau and Jacques Récamier’s Bec-de-Lièvres
In the third group, there is usually an associated cleft of the
(1938), Victor Spina’s ‘‘Tratamento cirurgico do labio [alveolar] process, lip and palate of many degrees anatomically;
leporino’’ (1961), and Blair O. Rogers’s ‘‘Harelip but there must be included here that case, of variable
Repair in Colonial America’’ (1964). frequency, in which a cleft lip and cleft process are present
with a normal palate, or, if such cases occur, of a cleft palate
ANATOMIC AND MORPHOLOGIC PERSPECTIVES and cleft process with a normal lip. The proper classification of
these cases is a subject for debate. . . . It will be more
practicable to record them in the third group as there the
Davis and Ritchie (1922)
opportunity is given for the direct description of the three
structures. (Davis and Ritchie, 1922, p. X)
Davis and Ritchie went beyond merely recommending
discontinuation of the term harelip. They were among Davis and Ritchie considered the alveolar process to
the first to advocate for a standard system of be of central importance to the surgical understanding
classification. They argued, of the problem and therefore described cleft phenotypes
So far, there have been no generally accepted standard terms
in relation to this structure. In their words, ‘‘the alveolar
for describing congenital clefts of the lip and palate, and, in process forms the foundation for a surgical grouping’’
consequence, it is often difficult to understand the (Davis and Ritchie, 1922, p. X); the classification system
descriptions in some of the numerous articles written on therefore ‘‘separates the cases according to their special
the subject. One author may use a set of terms to describe
surgical requirements, and it indicates in every case,
certain of these conditions, while another may use the same
terms to describe those conditions which are surgically directly instead of by implication, the most important
different. Then again an author may have a set of terms condition—that of the alveolar process’’ (Davis and
which, it appears on investigation, he alone uses, and the Ritchie, 1922, p. X).
reader may have to depend on the illustrations to find what Their supposition that the alveolus formed the basis
is really meant by the text. In fact, the terminology is
considerably confused. (p. XX) of an ‘‘intelligent classification’’ was not universally
accepted and was cause for much debate. Nevertheless,
Davis and Ritchie proposed a simple three-group Davis and Ritchie’s proposal was received favorably by
system that allowed separate description of the lip, contemporary surgeons such as James Thompson of
alveolus, and palate, using the alveolar process as a Galveston, Texas, who went on record to say that ‘‘there
dividing line for their categorization: is great need of improvement in classification and
 Group I: Prealveolar process cleft (clefts affecting the nomenclature and that the scheme [by Davis & Ritchie]
lip) is a step in advance’’ (Davis and Ritchie, 1922, p. X).
1. Unilateral (right/left: complete/incomplete) Vilray Blair of St. Louis was cautiously reserved in his
2. Bilateral (right: complete/incomplete; left: com- endorsement of the new scheme:
plete/incomplete) I am thoroughly in sympathy with and heartily approve of the
3. Median (complete/incomplete) whole idea, but we are attempting to project something for
 Group II: Postalveolar process cleft (clefts affecting universal adaptation. If it is not universally adopted, it is not
the palate) worth while; and, to assure its adoption, it must be the best
1. Soft palate plan. If it is not, some one will present something a little better,
upset the one we have adopted and go on. (Davis and Ritchie,
2. Hard palate
1922, p. X)
 Group III: Alveolar process cleft (any cleft involving
the alveolar process) Blair’s major criticism of the classification system was
1. Unilateral (right/left: complete/incomplete) its basis on contemporary surgical perspectives (that
2. Bilateral (right: complete/incomplete; left: com- were subject to change) rather than on anatomy alone
plete/incomplete) (which is immutable):
3. Median (complete/incomplete)
Dr. Ritchie puts forth . . . that the classification shall be along
Because CL/P phenotypes may involve multiple surgical lines, abandoning the anatomic lines. Granting that . .
structures, Davis and Ritchie allowed overlap of the . the surgical classification has the broader appeal, the anatomic
basis is more fixed. To my mind, . . . [we may one day be left
categories. Specifically, they prescribed that cleft lip with] a classification based on a surgical conception that had
with cleft of the secondary palate (but intact alveolus) be not stood the test of time. . . . I am not sure we are sufficiently
recorded as both group I and group II; however, any advanced to make a permanent, logical classification on a
and every case involving a cleft of the alveolus surgical basis. (Davis and Ritchie, 1922, p. X)
Allori et al., CLASSIFICATION OF CL/P 0

Brophy (1921 to 1923) line between the prealveolar clefts and postalveolar
clefts was arbitrary. Poul Fogh-Andersen of Copenha-
Truman W. Brophy of Chicago echoed Blair’s sentiment gen was one such surgeon who considered the incisive
that a suitable classification should be based on a proper foramen, rather than alveolar process, to be a better
understanding of anatomy; he was quite vocal in criticizing dividing line from an embryological perspective.
the Davis and Ritchie system as being insufficiently In his monograph, Inheritance of Harelip and Cleft
detailed in this regard. In 1921 and 1923, Brophy Palate (1942), Fogh-Andersen proposed an alternative
published his own exhaustive study of the forms of CL/P to the Davis and Ritchie classification that was
based on an incredible 5076 operations to repair cleft composed of four groups:
palate and 2676 operations to repair cleft lip (Brophy,
1921). His intention was to account for ‘‘every muscle and 1. Harelip (single or double)
bone involved in this deformity’’ (Davis and Ritchie, 2. Harelip with cleft palate
1922). Accordingly, his classification included 16 distinct 3. Isolated cleft palate
morphological forms of cleft palate with/without cleft lip 4. Rare atypical clefts, e.g., median cleft lip
(Table 1). Brophy’s classification was lauded by the Annals
of Surgery (McWilliams, 1924), but many surgeons Like Davis and Ritchie, Fogh-Andersen’s group 1
considered the system overly complex and impractical. clefts (‘‘harelip’’) were anterior, but for Fogh-Andersen,
this meant the cleft was anterior to the incisive foramen
Veau (1931) rather than to the alveolar process. Importantly, Fogh-
Andersen noted in his large epidemiologic investigations
In 1931, Victor Veau published his landmark Division that some degree of labial clefting was invariably
Palatine, which described his approach to evaluation observed in association with division of the alveolar
and management of cleft palate. Veau was respectful of process, suggesting that cleft lip and alveolus were
Brophy but openly critical of his classification system, associated and possibly etiologically related. Thus,
stating that it specified a great many ‘‘variétés de la Fogh-Andersen’s group 1 (‘‘harelip’’) effectively in-
même forme’’ (‘‘varieties of the same form’’) that should cludes cleft lip alone and cleft lip with cleft alveolus,
be grouped together. and his group 2 (‘‘harelip with cleft palate’’) encom-
Veau’s greatly simplified classification of palatal clefts passed cleft lip and alveolus with cleft palate (e.g., Veau
consisted of four morphological forms (Table 1): III and Veau IV) as well as cleft lip and palate with
I. Clefts of the soft palate intact alveolus (e.g., cleft lip with a Veau I or Veau II
II. Clefts of the soft and hard palate, up to the incisive cleft palate).
foramen Fogh-Andersen further refined the definition of an
III. Clefts of the soft and hard palate extending isolated cleft palate (his group 3) as a defect that is
unilaterally through alveolus ‘‘always median [which] never reaches further than the
IV. Clefts of the soft and hard palate extending incisor foramen’’ (Fogh-Andersen, 1971). This descrip-
bilaterally through alveolus tion is equivalent to a Veau I cleft of the soft palate only
or a Veau II cleft of the secondary hard and soft palate.
Of note, while Veau discusses the most intricate of Veau III and Veau IV cleft palate were necessarily
anatomical findings in Division Palatine, he purposefully
included in group 2 because Fogh-Andersen believed
chose to exclude ‘‘confounding’’ details (such as severity)
some degree of labial clefting to be present whenever the
from the classification system itself, preferring simple
alveolar process was violated.
groupings. In his subsequent work dedicated to cleft lip,
Finally, Fogh-Andersen added group 4 to capture the
Bec-de-Lièvres (Veau and Récamier, 1938), Veau eschews
taxonomical groupings altogether and instead advocates a median cleft lip, which was previously regarded only as
clear and concise description of the labial defect (including a rare defect rather than as an atypical but distinct
laterality [unilateral/bilateral/median] and extent [simple/ category of cleft lip.
total]). The minimalism, morphologic basis, and clinical
relevance of Veau’s approach to classification made it very Kernahan and Stark (1958)
attractive to contemporary surgeons.
Desmond A. Kernahan and Richard B. Stark of New
EMBRYOLOGIC PERSPECTIVES York were also staunch advocates of basing a cleft
classification system on developmental anatomy. In
Fogh-Andersen (1942) 1958, they provided support for Fogh-Andersen’s use of
the incisive foramen as the embryologically sound
One of the criticisms of the Davis and Ritchie dividing line by citing evidence from the most current
classification was that the alveolar process as dividing understanding of facial embryogenesis:
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

TABLE 1 Comparison of the Morphologic Classification Schemes of Brophy (1924) and Veau (1932)*

The ‘‘primary palate’’ extends as far posterior as the incisive palatal processes subsequently assume a position above the
foramen; its development is completed by the seventh week of tongue. . . . They meet in the midline and fuse, a process which
intra-uterine life. From this ‘‘primary palate’’ are subsequently begins at the incisive foramen and progresses posteriorly during
formed the central portion of the upper lip and the premaxilla. the eight to twelfth weeks of intra-uterine life. (Kernahan and
Failure of this mesodermal penetration leads to breakdown of
Stark, 1958, p. X)
the ectoderm and formation of clefts, although rarely a small
bridge of ectoderm may persist in the form of a Simonart’s band
Kernahan and Stark challenged the use of morphol-
at the nostril floor.
ogy alone as a basis for classification: ‘‘The embryologic
From one to five weeks later, (i.e. from 7-12 weeks of events leading to the deformity, viz., the separate nature
intrauterine life) formation of the secondary palate (hard and and time formation of the primary and secondary
soft palate) occurs through the growth medianward and fusion
of two laterally placed palatal shelves. . . . Hanging downward palates, are well understood;’’ and therefore, ‘‘cleft lip
and lying laterally alongside the tongue at first, these two and palate . . . ideally should be classified on that basis’’
Allori et al., CLASSIFICATION OF CL/P 0

TABLE 1 Continued
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

TABLE 1 Continued
Allori et al., CLASSIFICATION OF CL/P 0

TABLE 1 Continued

* Brophy’s (1923) system was very detailed but arguably too complex for practical use. Veau himself commented that Brophy’s classification featured a great many ‘‘variétés de la
même forme’’ (‘‘varieties of the same type’’) and aimed to simplify the classification into one that was more utilitarian and clinically significant. Veau I encompasses Brophy classes 1 to
3, Veau II encompasses Brophy classes 4 to 6, Veau III corresponds with Brophy class 7, and Veau IV corresponds with Brophy class 8 (and arguably 13). Differences among the
Brophy classes within these groupings are really matters of severity. Brophy classes 9 to 16 are other combinations of CLþP that are not considered by the Veau classification, which
was limited to describing clefts of the palate (CP) only. According to Veau, ‘‘Pour ma part, je m’y refuse absolument . . . ils sont opérés comme des becs-de-lièvre ordinaire et non
comme des divisions palatines’’ (‘‘For my part, I absolutely refuse [to classify them] . . . they are operated upon like ordinary harelips and not like palatal divisions.’’) Figures
reproduced from Veau V. Division Palatine. Paris: Masson; 1931. Permission requested from Elsevier-Masson press; pending approval.

(Kernahan, 1991, p. X). Based on this understanding, Modifiers were added to describe laterality (unilat-
Kernahan and Stark proposed three groups: eral/bilateral/median) and severity (total/subtotal).
1. Clefts of structures anterior to the incisive foramen This nosological framework is nearly identical to that
2. Clefts of structures posterior to the incisive foramen of Fogh-Andersen, although the ordering of the
3. Clefts affecting structures anterior and posterior to groups is different. Kernahan and Stark encompassed
the incisive foramen median cleft lip in their group 1 (anterior clefts)
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

TABLE 2 Criteria for an ‘‘Ideal’’ Classification Scheme* (Table 2; Harkins et al., 1962). The Committee
emphasized precise terms with clear definitions, rejecting
I. Concise, clear definitions of terms; hence,
A. Rejection of the meaningless, the ambiguous, and the irrelevant ‘‘the meaningless, the ambiguous, and the irrelevant.’’
B. Preference for simple, descriptive English terms Specifically, the Committee encouraged the use of Greek
C. Retention of established customary terms, where possible, in order
and Latin terms,2 which they believed to be concise,
to avoid duplication and confusion
D. Formation of new terms only where necessary specific, and universally understandable. The Commit-
E. Indication of synonomous [sic] terms, especially those of wide usage tee also advocated for a system that would respect
such as ones based on Latin or Greek, to facilitate comprehension
and use of the system proposed here
embryology and surgical anatomy, such that it would be
II. Convenience of use through: useful to both clinicians and academicians.
A. Economy of expression Implementation of this framework required two
B. Logical arrangement of classification conformable with
1. Normal topographic (spatial) relationship of anatomical principal anatomical demarcations: the ‘‘prepalate,’’
structures anterior to the incisive foramen and thus including lip,
2. Normal sequence in embryologic advent and union
C. Standardized methods of measurement
alveolar process, and primary hard palate, and the
III. Stimulation of scholarly and clinical research by: ‘‘palate,’’ posterior to the incisive foramen and thus
A. Standardized procedures for observation and reporting including secondary hard and soft palate. Of note, the
1. Meaningful in terms of embryologic antecedents
2. Meaningful in describing tissue relationships used to evaluate alveolus was included with the primary hard palate as a
need and method of therapy singular unit (prepalate), per Kernahan and Stark;
B. Provision for rare conditions however, this practice was still a point of contention.
* In its 1962 report, the Nomenclature Committee of the American Association for The Committee believed it was important to clarify the
Cleft Palate Rehabilitation published its criteria for an ideal classification scheme
(Harkins et al., 1962). These guiding principles are still very valid. rationale for this recommendation based not only on
embryologic evidence but also on epidemiologic obser-
rather relying on a separate catch-all category for vations by Fogh-Andersen and others:
atypical clefts.1 Defects seem to appear in accordance with these groupings.
Clefts of the lip occur alone, [as] do clefts of the lip and alveolar
REFINEMENT process, not involving the palate. Clefts of the palate occur in
varying degrees from the uvula to the incisive foramen; however,
once past this point, they usually continue through the alveolar
American Cleft Palate–Craniofacial Association
process and lip. (Harkins et al., 1962, p. X)
Classification (Harkins et al., 1962)
Anatomic segmentation into the prepalate and the
In 1960, the American Association for Cleft Palate palate thus permitted separation into four major
Rehabilitation appointed a Nomenclature Committee to categories of orofacial clefts in the American Cleft
formulate a classification system. The Committee was Palate–Craniofacial Association (ACPA) classification:
chaired by Cloyd S. Harkins and included Asa Berlin,
Robert J. Harding, J. J. Longacre, and Richard M. 1. Clefts of the prepalate (cleft of lip and embryologic
Snodgrasse. The committee recognized that ‘‘a major primary palate)
impediment to communication and understanding in a. Cleft lip (cheiloschisis)
any realm of human endeavor exists when and wherever b. Cleft alveolus (alveoloschisis)
terms are inadequately defined and consensus does not c. Cleft lip, alveolus, and primary palate (cheiloal-
obtain in the classification’’ (Harkins et al., 1962). The veoloschisis)
need for a standardized terminology was becoming 2. Clefts of the palate (cleft of the embryologic
increasingly important with the advent of multidisci- secondary palate)
plinary cleft palate teams, which included not only a. Cleft of the hard palate (uranoschisis)
surgeons and dentists but also new colleagues from b. Cleft of the soft palate (staphyloschisis or velo-
other fields such as speech and language pathology, schisis)
genetics, and developmental biology. c. Cleft of the hard and soft palate (uranostaphylo-
In its 1962 report, the Nomenclature Committee schisis)
published its criteria for an ideal classification scheme

2
The Greek roots used included veíko1/cheı́los (‘‘lip’’); otqamó1/
1
A modern appreciation of fetal facial development clarifies that uran ós (‘‘sky’’), signifying the hard palate; rsáutko1/stáfilos
a median premaxillary cleft is due to incomplete fusion of the medial (‘‘grapes’’), representing the uvula and soft palate; and rvírilo/
nasal prominences to form the premaxillary segment, whereas the schı́simo (‘‘tear’’ or cleft). Latin roots included alveus (‘‘trough’’ or
more common lateral cleft lip with/without cleft alveolus occurs ‘‘hollow’’) for the tooth-bearing alveolar process and velum (‘‘veil’’
from aberrant fusion of the right/left maxillary prominences with or ‘‘sail’’) for the soft palate. Combinations yielded very specific
the premaxillary segment. In this light, a system organized strictly terms, such as uranostaphyloschisis (a cleft affecting the hard and
according to embryologic principles would indeed classify median soft palate) and alveolocheilopalatoschisis (a cleft affecting the lip,
labial clefts separately from lateral labial clefts. alveolus, and palate).
Allori et al., CLASSIFICATION OF CL/P 0

3. Clefts of the prepalate and palate (alveolocheilopala- B. Oblique clefts (oro-orbital)


toschisis) C. Transverse clefts (oroauricular)
4. Facial clefts other than prepalatal and palatal D. Clefts of the lower lip, nose, and other very rare
a. Cleft of the mandibular process clefts
b. Naso-ocular clefts
Clefts within each of groups 1 to 3 received
c. Oro-ocular clefts
descriptions of all involved structures (lip, alveolus,
d. Oroaural clefts
hard palate, and soft palate) by laterality and severity
Each cleft could be further characterized by laterality (using the terms total or partial). The International
and severity. Laterality was simply denoted as left, right, Subcommittee published its classification in 1969 in the
bilateral, or median. With regard to severity, the Transactions of the International Confederation (Broad-
Committee chose to use quantitative measurement of bent et al., 1969).
the width of the cleft and semiquantitative description
of the extent of the cleft. Extent was denoted as 1/3, 2/3, Spina (1973)
or 3/3 the length or area of the involved structure.
Specific criteria were attached to these descriptors for In 1973, Victor Spina of São Paulo suggested a minor
each condition on a case-by-case basis, but they may be revision of the International Classification’s first tier
thought of as corresponding roughly with ‘‘minor with the intent to make the terminology more precise.
form,’’ ‘‘incomplete,’’ and ‘‘complete,’’ respectively. Spina argued that the terms anterior and posterior
Interestingly, unlike prior surgical classifications, the describing palatal defects in group 1 and group 3,
Committee ‘‘views all hard palate clefts as midline respectively, raised the question, ‘‘anterior or posterior
(therefore not needing specification [as right, left, or to what?’’ (The answer, of course, is the incisive
bilateral]), but encourages the identification of vomer foramen.) Accordingly, Spina renamed group 1 as pre-
attachment as a separate observation.’’ The Committee foraminal clefts, group 2 as trans-foraminal clefts, and
recommended that certain details that are relevant and group III as post-foraminal clefts. Spina maintained that
noteworthy to surgeons do not necessarily warrant basing the terminology on Latin with reference to a
adding complexity to the classification. specific anatomic structure would make the classifica-
tion system easier to understand, simpler to teach, more
International Classification (Broadbent et al., 1969) manageable to memorize, and more applicable to
interdisciplinary and international communication.
In 1967, the International Confederation for Plastic
and Reconstructive Surgery convened the 4th Interna- TOWARD CONSENSUS
tional Congress in Rome. The Subcommittee on No-
menclature and Classification was chaired by T. Ray Striped-Y
Broadbent and included Fogh-Andersen, Berlin, Karfik,
David N. Matthews, Gerhard Pfeifer, Gustavo Sanve- The extent of adoption of either the ACPA or
nero Rosselli, Karl Schuchardt, and Stark, many of International schema is not known, but certainly the
whom brought years of nosological and taxonomical Kernahan and Stark classification persisted in wide-
experience to the table. The International Subcommittee spread use well beyond their introduction. This can
based its framework on the prior work of Fogh- probably be explained in large part by Kernahan’s
Andersen, Kernahan and Stark, and Harkins et al. They translation of the Kernahan and Stark classification
devised a two-tier system that categorized clefts of the lip, system into pictographic form (Kernahan, 1971; Fig. 1).
alveolus, and palate based on embryologic principles and The Kernahan ‘‘striped-Y’’ diagram was designed
rare facial clefts based on topographic findings: principally to simplify record keeping, but it also made
Classification of the lip, alveolus, and palate (based classification a visual process rather than an abstract,
on embryologic principles): cognitive exercise. Kernahan’s striped-Y and the subse-
quent modifications by Elsahy (1973) and Millard
1. Clefts of the anterior (primary) palate (1976) served to secure the position of the Kernahan
2. Clefts of the anterior (primary) and posterior and Stark classification as the predominant system used
(secondary) palates in daily clinical practice.
3. Clefts of the posterior (secondary) palate
Classification of rare facial clefts (based on topo- ACPA Reclassification Committee (Whitaker et al.,
graphical findings): 1981)

A. Median clefts of the upper lip, with/without In 1981, the ACPA Ad Hoc Committee on Nomen-
hypoplasia or aplasia of the premaxilla clature and Classification of Craniofacial Anomalies,
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

could be used, and it recommended the striped-Y


diagram as a useful notational tool that was compatible
with both systems.

TO INCLUDE OR EXCLUDE FACIAL CLEFTS?

A discussion of classification of CL/P (or orofacial clefts)


would not be complete without acknowledging the equally
important work devoted to classification of other facial
clefts. Some clinicians thought that there should exist a
unifying classification that included all facial clefts; the
practical constraint, however, was that this usually took the
form of a generic catch-all category that did not adequately
describe the atypical clefts. Other clinicians thought that
facial clefts required their own classification system. For
further discussion of these considerations, the reader is
referred to the following resources: Harkins et al. (1962),
Tessier (1976), and Whitaker et al. (1981).

DISCUSSION: A SYNTHESIS AND PROPOSAL

‘‘If the future is to bring any increase in our understanding of


the cleft lip and palate problem, a uniform method of
classification is essential.’’

—Kernahan and Stark (1958)

Where Are We Now?

In reflecting on the rich history of classification in CL/


P, we see that there has been a stepwise progression of
FIGURE 1 The striped-Y logo designed by Kernahan was as a thoughts and ideas—sometimes complementary and
pictographic representation of the cleft phenotype and served as a sometimes discordant—that has led to a state that is
symbolic correlate of the Kernahan and Stark classification system. more advanced yet still incomplete.
Millard (1976) in Cleft Craft reviewed the history of
chaired by Linton A. Whitaker, was charged with the classification systems for CL/P and concluded that
reclassification of craniofacial anomalies. In review of ‘‘none has been universally accepted because of lan-
classification of CL/P, the Committee listed numerous guage differences, inaccuracies, omissions and lack of
criticisms of the International Classification of 1969: simplicity’’ (p. X). While he acknowledged the need for
First, the ordering and numbering of the groups in the detail in the medical record (for which he employed his
International Classification was confusing and conflict- modification of a striped-Y diagram), he firmly believed
ed with those of other classifications. Second, the that classification systems per se should remain uncom-
Committee disagreed with the terms anterior and plicated and useful. This sentiment was echoed in chorus
posterior palate, preferring the embryologically precise by Davis and Ritchie, Veau, Fogh-Andersen, Kernahan
primary and secondary palate (as used by Kernahan and and Stark, and others.
Stark) or prepalate and palate (as used in the ACPA Reading through Millard’s work, one notices that he
Classification by Harkins et al.). A third criticism preferred to simply use the descriptive terms cleft lip,
concerned listing median cleft lip under rare facial cleft palate, and cleft lip and palate. Veau and Récamier
clefts, when it made more clinical sense to group it with (1938) also, in Bec-de-Lièvres, chose to describe the cleft
the other orofacial clefts in the first tier (clefts of the lip, lip rather than to categorize it. Were Veau and Millard
alveolus, and palate). on the right track? Is there really a need to further
The 1981 Committee determined that the Kernahan classify and categorize, subdivide, and characterize?
and Stark classification of 1955 and the ACPA What is the utility of a label such as group 1 or type B?
Classification of 1962 were better systems, based on Yet most clinicians and academicians would agree
their syntheses of current knowledge and simplicity and wholeheartedly that there must exist a way to describe
clarity of presentation. It reaffirmed that either system the cleft phenotype, if not to separate data into different
Allori et al., CLASSIFICATION OF CL/P 0

subgroups for taxonomical purposes, then at least to 1. As posited by Fogh-Andersen, Kernahan and Stark,
facilitate accurate communication among practitioners. and others, the incisive foramen is an embryologi-
CL/P is phenotypically diverse. The lip, alveolus, cally correct and surgically useful dividing point that
primary palate, secondary hard palate, and soft palate separates preforaminal structures (lip, alveolus, and
may each be affected in varying degrees, or not at all, primary hard palate) from postforaminal structures
and the total number of possible combinations is in the (secondary hard palate and soft palate).
tens of thousands. The challenge is to record this 2. Per Harkins et al., a complete description of the CL/
information in a way that is sufficiently detailed, P phenotype requires specifying if the cleft involves
accurate, intelligible, and consistent. Moreover, it is the lip, alveolus (and primary palate), and/or
desirable that the method also be adopted universally, secondary palate. Moreover, as stressed by Davis
such that data could be shared among groups and and Ritchie, the state of the integrity of the alveolar
instantly understood. process is a critical detail that must not be omitted. It
Detailed awareness of the history of CL/P classifica- has implications for choice of therapy (e.g., dento-
tion allows one to understand the elements that must be facial orthopedics, nasolabial adhesion, gingivoper-
incorporated into an ideal schema. Most enlightening is iosteoplasty, alveolar bone grafting, etc.) as well as
the contrast between Brophy and Veau in their potential outcomes (e.g., effects on dentition, occlu-
classifications of cleft palate. Whereas Brophy sought sion, and articulatory speech).
to describe every intricate detail, Veau surmised that 3. The laterality and severity of the labial cleft and the
many were mere variations on a theme that did not morphology of the palatal cleft should be specified to
warrant separate groups in the classification. Rather, the degree necessary to describe morphologic fea-
Veau sought to develop a system that remained as tures relevant to treatment planning or outcome
simple as possible yet could adequately describe as many assessment.
of Brophy’s classes as possible. a. Labial description: As argued by Veau and
The importance of a balance between comprehensive- Millard, it is more practicable and more easily
ness and simplicity was also underscored by the ACPA understood to describe the cleft lip rather than to
Nomenclature Committee in 1965. The committee categorize it into taxonomical groups.
emphasized that the terms in the classification needed b. Palatal description: For simplicity and clinical
to be clear, concise, logically arranged, and convenient utility, we advocate use of Veau’s numbering for
and also accurate from both embryologic (etiologic) and morphologic description of cleft palate. To
surgical (therapeutic) perspectives. Veau’s four types, we would add only ‘‘bifid
An additional challenge is that cleft care is interdis- uvula’’ and ‘‘submucous cleft palate’’ (with/
ciplinary and complex; there are many perspectives to without bifid uvula), which covers the minor
consider. A researcher may prefer a system with an forms of cleft palate. Thus, there are only six
embryologic, genetic, or biochemical foundation; the forms to consider, which are easily learned by
clinician, one that is simple and correlates with clinicians of any specialty and level of experi-
treatment goals and principles; the epidemiologist, one ence. In our review of clinical records from
that is comprehensive and versatile; and the adminis- several well-known cleft centers (data not
trator, one that is practical and easily implemented. An shown), use of the Veau classification has been
ideal system needs to serve these diverse needs. more consistent than descriptive terms such as
complete, incomplete, partial, unilateral, bilateral,
Where Do We Go From Here? and central. We suggest that these inexact terms
lead to confusion and add little value to a
classification; moreover, we propose that such
Proposal for a Universal Structured Form for Description terms should be discarded.
of CL/P Phenotypes 4. For simplicity, high-level morphological details (such
as severity of alveolar clefting, protrusion of the
With great admiration for those giants upon whose premaxilla, and collapse of the alveolar segments)
shoulders we stand, the authors respectfully propose a should be excluded from the classification. Of course,
unifying synthesis as a standardized method for these details are of clinical interest and should be
description of CL/P phenotypes. This schema is based sufficiently described in each patient’s medical
on the most enduring elements of the historical record. As Kernahan keenly emphasized, such
schemata and represents the next step in the natural clinical features ‘‘lie outside the scope of a classifi-
progression toward a comprehensive and clinically cation’’ (Friedman et al., 1991). Too many details
useful classification system. Specifically, the system is make a classification system so utterly encumbered
based on the following precepts: and complex that it becomes unusable.
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

FIGURE 2 CLAP notation is an acronymic shorthand for the longhand structured form. Uppercase letters summarize the part of the anatomy involved
(L, lip; A, alveolus; P, palate). A lowercase prefix describes the laterality and severity of the preforaminal component (lip) (laterality ¼ u, unilateral; b,
bilateral; med, median; severity ¼ c, complete; i, incomplete; m, minor-form, microform, or mini-microform; a, asymmetric). A lowercase suffix designates
morphology of the postforaminal component (secondary palate; bu, bifid uvula; sm, submucous; v1, v2, v3, and v4, Veau I-IV, respectively). CLAP
notation is read from left to right and translates directly to the structured form. For example, ‘‘right ucCLAPv3’’ translates as ‘‘right unilateral complete
cleft of the lip and alveolus with a palate that is Veau III.’’

Therefore, a complete description of a CL/P pheno- longhand notation does not lend itself easily to grouping
type should include the laterality and severity of the for research or administrative purposes.
labial defect, acknowledgment of an alveolar defect, and To improve the practicality of the system (and
a morphological characterization of the palatal defect hopefully its rate of adoption and fidelity in implemen-
(Fig. 2). Examples are given below: tation), we developed a complementary shorthand
notation, which is illustrated in Figure 2. The impor-
 Veau-I cleft palate tance and usefulness of parallel longhand and shorthand
 Left unilateral incomplete cleft lip (with intact forms was underscored by Balakrishnan (1975).
alveolus and secondary palate) The proposed shorthand notation is visibly similar to
 Right unilateral complete cleft lip and alveolus with the informal abbreviations that many clinicians intuitively
Veau-III palate use. (A review of clinical notes from several well-known
 Bilateral asymmetric cleft lip (right complete and left cleft teams [data not shown] revealed that the rather
microform) with right Veau-III palate common ‘‘right unilateral complete cleft lip and alveolus
 Median cleft of prolabium and premaxilla with Veau-III palate’’ was recorded with great variability
as ‘‘RUCCL/P,’’ ‘‘RUCLP,’’ ‘‘UCCLP,’’ ‘‘UCCL&P,’’
This longhand structured format has been successfully and ‘‘CUCLP.’’) Unlike these casual abbreviations,
employed by many experienced clinicians. When such a however, our proposed shorthand notation is strictly
system is used routinely, it leaves very little to structured according to a few rules and definitions.
question—diagnoses are always clear, treatment proto- Briefly, the uppercase C serves as the central reference
cols can be anticipated from the diagnosis, and even point and merely stands for the word cleft. The
administrative coding is more straightforward. Unfor- anatomic parts are listed acronymically in capital letters
tunately, many clinicians are inconsistent in describing a (L for lip, A for alveolus, and P for palate). Therefore,
phenotype so thoroughly. We encourage all teams to the notation for a cleft lip alone would feature only the
adopt this standardized structure for describing CL/P letters CL; a cleft lip and alveolus with intact palate,
phenotypes. CLA; a cleft lip, alveolus, and palate, CLAP; and a cleft
lip with cleft palate but intact alveolus, CLP. These
Practical Implementation: Shorthand CLAP Notation uppercase letters specify anatomic involvement.
Preceding this acronym is a lowercase prefix serving
Admittedly, writing out this longhand notation every as a preforaminal descriptor of laterality (u, unilateral;
time the patient is evaluated would be tedious. Also, the b, bilateral; med, median) and severity (c, complete; i,
Allori et al., CLASSIFICATION OF CL/P 0

TABLE 3 Standardized ‘‘Superset’’ Groupings for Phenotypic Combinations*

Superset Abbreviation Definition Includes Excludes

{CP} Cleft palate only CPbu CPv1 {CL6A}


CPsm CPv2 {CLþP}
CPv3
CPv4
{uCL} Unilateral cleft lip only (intact alveolus) ucCL {uCLþA}
uiCL {uCLþP}
umCL
{bCL} Bilateral cleft lip only (intact alveolus) bcCL {bCLþA}
biCL {bCLþP}
bmCL
baCL
{uCL6A} Unilateral cleft lip, with/without alveolus {uCL} ucCLA {uCLþP}
uiCLA
umCLA
{bCL6A} Bilateral cleft lip, with/without alveolus {bCL} bcCLA {bCLþP}
biCLA
bmCLA
baCLA
{CL6A} Cleft lip, with/without alveolus {uCL6A} {CLþP}
{bCL6A}
{CLþP} Cleft lip and palate ucCLPv1 ucCLAPv3 {CL6A}
uiCLPv1 uiCLAPv3 {CP}
umCLPv1 umCLAPv3
ucCLPv2 bcCLAPv4
uiCLPv2 biCLAPv4
umCLPv2 bmCLAPv4
baCLAPv4
etc.
{CL6P} Cleft lip, with/without cleft palate {CL6A} {CP}
{CLþP}
{CL/P} Cleft lip and/or cleft palate {CL6A} (no exclusions)
{CP6A}
{CLþP}
* These standardized groupings are proposed for data aggregation and analysis. To specify more detailed phenotypes, the CLAP notation itself may be used. (For clarity, supersets
may be enclosed in optional curly braces, whereas CLAP notation does not.)

incomplete; m, lesser-form [minor-form, microform, or using the longhand structured form described above
mini-microform]; and a, asymmetric, which is only (e.g., ‘‘right unilateral complete cleft lip and alveolus
possible for the bilateral case). For unilateral cases, the with Veau-III palate’’). The longhand notation should
terms right and left may be specified, but they are not be summarized parenthetically into the standard short-
abbreviated. This separates the ‘‘side’’ (right/left) from hand CLAP notation (e.g., ‘‘right ucCLAPv3’’). All
the ‘‘type’’ (unilateral). For example, both ‘‘right ucCL’’ notes at subsequent encounters may simply reference the
and ‘‘left ucCL’’ belong to the group ‘‘ucCL,’’ unilateral shorthand notation.
complete cleft lip. (To attempt to abbreviate this as Any other details that the team feels to be clinically
‘‘rucCL’’ and ‘‘lucCL’’ adds unnecessary complexity and relevant (e.g., width of the alveolar gap, degree of
makes the notation harder to read.) premaxillary protrustion, etc.) should be recorded in the
Succeeding the acronym is a lowercase suffix serving a medical record in the typical fashion. Teams may
postforaminal descriptor of the morphology of the cleft continue to employ any ancillary method of detailed
palate: bu (bifid uvula), sm (submucous cleft palate, with/ note taking (e.g., pictographic notation such as Kerna-
without bifid uvula), v1 (Veau-I cleft of soft palate), v2 han striped-Y diagram, or text-based notation such as
(Veau-II cleft of secondary soft and hard palate, up to LAHSHAL) to which they are accustomed, if they feel
incisive foramen), v3 (Veau-III unilateral cleft of second- these add value.
ary soft and hard palate through primary palate and
alveolus), and v4 (Veau-IV bilateral cleft of secondary soft Application: Aggregating Groups for Research and
and hard palate through primary palate and alveolus). Administration

Application: Patient-Specific Description for Medical For research and quality improvement, it is desirable
Documentation to sort patients into ‘‘meaningful’’ groups for data
aggregation and analysis. The choice of groups depends,
Our recommendation is that the medical note at the of course, on the particular needs of the researcher,
time of initial encounter describe the CL/P phenotype clinician, or administrator. The CLAP notation is both
0 Cleft Palate–Craniofacial Journal, January 2016, Vol. 53 No. 1

simple and versatile. It greatly facilitates grouping into Broadbent TR, Fogh-Andersen P, Berlin AJ, Karfik V, Matthews DN,
both general supersets as well as very specific subsets, Pfeifer G. Report of the Subcommittee on Nomenclature and
Classification of Clefts of Lip, Alveolus and Palate and Proposals
which for clarity may be enclosed within curly braces. for Further Activities. Newsletter of the International Confederation
Recommended groupings are based on the standard for Plastic and Reconstructive Surgery [Monograph]. Amsterdam,
abbreviations endorsed by Cleft Palate–Craniofacial 1969.
Journal and are shown in Table 3. Note the addition Brophy TW. Cleft Lip and Palate. Philadelphia: P. Blakiston’s Son and
of the plus sign (þ) to mean ‘‘and,’’ that is, the Co.; 1923.
Brophy TW. Cleft palate and harelip procedures. Int J Orthod Oral
combination of two features; the plus-or-minus sign
Surg. 1921;7:319–330.
(6) to mean ‘‘with/without,’’ signifying the optional Davis JS, Ritchie HP. Classification of congenital clefts of the lip and
inclusion of a second feature; and the virgule (forward palate with a suggestion for recording these cases. JAMA.
slash, or /) to mean ‘‘and/or.’’ Note specifically that 1922;79:1323–1327.
{CL/P} means ‘‘cleft lip and/or cleft palate,’’ which is Elsahy NI. The modified striped Y—a systematic classification for cleft
lip and palate. Cleft Palate J. 1973;10:247–250.
more strictly defined than in casual usage. By conven-
Fogh-Andersen P. Epidemiology and etiology of clefts. Birth Defects
tion, most statistical reports combine cleft lip alone Orig Artic Ser. 1971;7(7):50–53.
together with cleft lip and alveolus; thus, we list the Franco P. The cure of cleft lips in 1561. Plast Reconstr Surg.
‘‘cleft-lip’’ superset as {CL6A} rather than as the less- 1976;57:84–85.
accurate ‘‘CL.’’ To specify more detail in the description Friedman HI, Sayetta RB, Coston GN, Hussey JR. Symbolic
of a particular phenotype, the compete CLAP notation representation of cleft lip and palate. Cleft Palate Craniofac J.
1991;28:252–260.
should be used. Harkins CS, Berlin A, Harding RL, Longacre JJ, Snodgrasse RM. A
classification of cleft lip and cleft palate. Plast Reconstr Surg
CONCLUSION Transplant Bull. 1962;29:31–39.
Kernahan DA. On symbolic representation of cleft lip and palate.
Earlier, the rich history of classification of CL/P Cleft Palate Craniofac J. 1991;28:259–260.
Kernahan DA. The striped Y—a symbolic classification for cleft lip
phenotypes was explored. Whereas prior classification
and palate. Plast Reconstr Surg. 1971;47:469–470.
systems were firmly rooted in taxonomical divisions, Kernahan DA, Stark RB. A new classification for cleft lip and cleft
consisting of groups and subgroups, we propose a system palate. Plast Reconstr Surg Transplant Bull. 1958;22:435–441.
that is descriptive rather than hierarchical. The clinician Malgaigne J-F, Ivy RH. Du bec-de-lièvre. Plast Reconstr Surg.
describes the cleft phenotype in words; the requirements of 1976;57:359–363.
McWilliams CA. Book reviews: Cleft Lip and Palate, by Truman W.
the longhand structured form ensure that this description is
Brophy. Ann Surg. 1924;79:154–157.
clear, comprehensive, and consistent. Simple rules guide the Millard RD Jr. The naming and classifying of clefts. In: Cleft Craft.
conversion of this longhand structured form into an Vol. I. Boston: Little, Brown, and Co.; 1976:41–55.
intuitive and readable acronymic shorthand that improves Rogers BO. Harelip repair in colonial America: a review of 18th
its utility and convenience. This system focuses on clinically century and earlier surgical techniques. Plast Reconstr Surg.
meaningful anatomic differences in cleft types and should 1964;34:142–162.
Spina V. A proposed modification for the classification of cleft lip and
be sufficient for most use cases. A follow-up article will also cleft palate. Cleft Palate J. 1973;10:251–252.
describe the great compatibility of this method of Spina V. Tratamento cirurgico do labio leporino total uni e bilateral:
classification and documentation with coding systems, for Estudo evolutivo pela craniometria e modelagem. Revista Latino
example, ICD-10-CM and SNOMED-CT. We hope that Americana de Cirurgia Plastica. 1961;5:38–79.
cleft teams everywhere will find this system helpful for Tessier P. Anatomical classification of facial, cranio-facial and latero-
facial clefts. J Maxillofac Surg. 1976;4(2):69–92.
clinical care, research, and administration.
Veau V. Division Palatine: Anatomie, Chirurgie, Phonétique. Paris:
Masson; 1931.
REFERENCES Veau V, Récamier J. Bec-de-Lièvre: Formes Cliniques, Chirurgie. Paris:
Masson; 1938.
Balakrishnan C. Indian classification of cleft lip and palate. Ind J Plast Whitaker LA, Pashayan H, Reichman J. A proposed new classification
Surg. 1975;8:23–24. of craniofacial anomalies. Cleft Palate J. 1981;18:161–176.

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