New Classification Scheme of Proboscis Lateralis Based on a Review of

50 Cases
Yoshiaki Sakamoto, M.D., Junpei Miyamoto, M.D., Hideo Nakajima, M.D., Kazuo Kishi, M.D.

Background: Among congenital nasal deformities, proboscis lateralis is one

of the rarest. Boo-Chai classified proboscis lateralis into four groups. Recently,
we encountered a new case of proboscis lateralis with median cleft lip. We
noticed that this classification had not been considered according to
convalescence and embryologics, and further refinement seemed to be
needed.
Methods: We reviewed all cases of proboscis lateralis reported in English
through 2009 and classified them by intercanthal distance.
Results: A total of 34 studies involving 50 cases were reviewed. Six cases
were identified as having normal intercanthal distance. Three of them
presented nose abnormalities and fit Boo-Chai group II category. The other
three were consistent with group I. Hypertelorism was observed in 27 cases
and was further divided into two groups based on the occurrence of a frontal
encephalocele. Seventeen cases without a frontal encephalocele were
compatible with Boo-Chai groups III and IV. The other 10 cases associated
with a visible encephalocele had encephalopathy; most died at an early age,
and long-term survival cases suffered developmental delay and mental
retardation. Seventeen cases were defined as hypotelorism, and all cases also
presented as holoprosencephaly.
Conclusions: The redefined classification contains two new groups: group V
as hypertelorism with encephalocele and group VI as hypotelorism. A new
classification scheme is proposed as not only convenient for clinical
application but also embryologically accurate.

KEY WORDS: classification, cleft lip, holoprosencephaly, proboscis lateralis

Proboscis lateralis is a rare congenital anomaly related to
a facial fusion defect. It occurs in fewer than 1 of every
1,000,000 live births (English, 1988). It frequently appears
with holoprosencephaly, as shown in DeMyer’s classifica-
tion (Table 1) (DeMyer et al., 1964) .
Boo-Chai classified proboscis lateralis into four groups
according to the associated anomalies (Table 2) (Boo-Chai,
1985). Group I consists of a normal nose with proboscis
lateralis and is the least common type. In group II, nasal
abnormalities accompany the proboscis on the same side,
with no other anomalies. Group III is the most common
type, in which eye and/or adnexial defects occur with the
ipsilateral nasal defects and proboscis. In group IV, there is
a cleft lip and/or palate in addition to the nasal and ocular
defects.
Dr. Sakamoto is Assistant Professor, Dr. Miyamoto is Assistant
Professor, Dr. Nakajima is Associate Professor, and Dr. Kishi is
Professor, Department of Plastic and Reconstructive Surgery, Keio
University School of Medicine, Tokyo, Japan.
Submitted June 2010; Accepted November 2010.
Address correspondence to: Dr. Yoshiaki Sakamoto, Department of
Plastic and Reconstructive Surgery, Keio University School of Medicine,
35 Shinanomachi, Shinjuku-ward, Tokyo 160-8582, Japan. E-mail
ysakamoto@z8.keio.jp.
DOI: 10.1597/10-127.1
Recently, we reported a new case of proboscis lateralis
with median cleft lip (Fig. 1) (Sakamoto et al., 2010). We
noticed that although the classification scheme is useful, it
has two problems. The first is that the scheme does not take
holoprosencephaly into account, although it is often
important to distinguish holoprosencephaly from other
cases with good prognosis. The second problem is that the
groups are not completely classified embryologically.
Therefore, a clearer and more comprehensive classifica-
tion scheme is needed in order to take into account the
clinical complexities and embryology of proboscis lateralis.
MATERIALS AND METHODS
We reviewed all cases of proboscis lateralis reported
in English through 2009. Hypertelorism is one of the
distinctive features of holoprosencephaly. Boo-Chai (1985)
did not include intercanthal distance as a classification
item, and we added this as a variable. When there was a
mention of intercanthal distance for the case in the
literature, we adopted it.
On the other hand, if there was no mention, we reviewed
intercanthal distance based on the frontal photo of the
patient. Diagnosing hypotelorism and hypertelorism was
based not only on clinical visual impression but also the

201
202 Cleft Palate–Craniofacial Journal, March 2012, Vol. 49 No. 2

TABLE 1 Boo-Chai’s Classification of Proboscis Lateralis

Group Nose Eyes and Adnexa Cleft Lip/Palate

I Normal Normal None

II Defect Normal None
III Defect Abnormal None
IV Defect Abnormal Complicated

intercanthal index. Usually, the intercanthal index (100 3

inner intercanthal distance in centimeters/outer inter-
canthal distance in centimeters) is calculated from the
subject data. In this case, however, the frontal photograph
appearing in the literature was scanned, and an analysis of
the ratio of inner and outer intercanthal distance was
performed on a Macintosh computer using the public
domain NIH Image program developed at the National
Institutes of Health (2010). In keeping with the literature
(Farkas and Munro, 1987; Igarashi and Kajii, 1988;
Evereklioglu et al., 2002), an index less than 32 was defined
as hypotelorism and greater than 43 as hypertelorism.
This research is not subject to institutional review board
approval at our institution. This study followed the
Declaration of Helsinki.
RESULTS
Fifty-seven case studies were collected. Some reports that
did not describe intercanthal distance or did not provide
frontal photographs were excluded from the study. As a
result, our review included 34 studies involving 50 cases
(McLaren, 1955; Gitlin and Behar, 1960; Kirkpatrick,
1970; Dasgupta et al., 1971; D’assumpção, 1975; Rontal
and Duritz, 1977; Schinzel et al., 1984; Boo-Chai, 1985;
Bowe, 1986; Nyberg et al., 1987), including our report
(Sakamoto et al., 2010). They were classified into one of the
following three types based on the intercanthal distance:
hypotelorism, normal, and hypertelorism. Subsequently,
the relationship between intercanthal distance and Boo-
Chai’s (1985) classification scheme was examined.
Seventeen cases were identified as hypotelorism, and all
cases also presented as holoprosencephaly (Table 3). These
cases were all stillborn or died at an early age (Gitlin and
FIGURE 1 Our case of proboscis lateralis with median cleft lip (Sakamoto
et al., 2010).
Behar, 1960; Schinzel et al., 1984; Nyberg et al., 1987;
Souza et al., 1990; Galguera et al., 1996; Chen et al., 1999;
Situ et al., 2002; Boahene et al., 2005; Cho et al., 2005;
Granto et al., 2005).
Normal intercanthal distance was observed in six cases
(Table 4). Three of these presented nose abnormalities
(Dasgupta et al., 1971; Kayikçioğlu, 2000; Mutaf et al.,
2006), and the other three did not present any eye or other
abnormalities (Jost et al., 1995; Acarturk et al., 2006).
Therefore, the three cases with abnormal noses fit the Boo-
Chai group II category, and the other three cases were
consistent with his group I. The nose abnormality of the
three cases was heminasal hypoplasia, and each case had
two nostrils.
Hypertelorism was observed in 27 cases, which could be
further divided into two groups based on the occurrence of
a frontal encephalocele. There were 17 cases without a
frontal encephalocele, but all had anomalies of eye and
adnexa (Kirkpatrick, 1970; D’assumpção, 1975; Rontal
and Duritz, 1977; Bowe, 1986; Belet et al., 2002; Eroğlu and
Uysal, 2003; Abou-Elhamd, 2004; Guion-Almeida et al.,
2004; Acarturk et al., 2006; Thorne et al., 2007), and six
had cleft lip/palate (Boo-Chai, 1985; Belet et al., 2002;
Eroğlu and Uysal, 2003; Uğurlu et al., 2005; Sakamoto et

TABLE 2 DeMyer’s Classification of Holoprosencephaly

Type Type of Face Facial Features Cranium and Brain

I Cyclopia Single eye or partially divided eye in single orbit Microcephaly

Arhinia with proboscis Alobar holoprosencephaly
II Ethmocephaly Extreme orbital hypotelorism but separate orbits Microcephaly
Arhinia with proboscis Alobar holoprosencephaly
III Cebocephaly Orbital hypotelorism, proboscis-like nose Microcephaly
No medial cleft of lip Usually has alobar holoprosencephaly
IV With median cleft lip Orbital hypotelorism, flat nose Microcephaly and sometimes trigonocephaly
Usually has alobar holoprosencephaly
V With median philtrum-premaxilla Orbital hypotelorism, bilateral lateral cleft of lip with Microcephaly and sometimes trigonocephaly
Anlage median process representing philtrum-premaxilla Semilobar or lobar holoprosencephaly
Anlage, flat nose
 Sakamoto et al., NEW CLASSIFICATION SCHEME OF PROBOSCIS LATERALIS 203

TABLE 3 Proboscis With Hypotelorism

No. Authors Age Sex Group* Side{ Remarks

1. Gitlin and Behar (1960) Infant M III Bilateral Died immediately after birth
2. Schinzel et al. (1984) Infant M III Midline Stillbirth, cyclopia
3. Infant Unknown II Midline Died 2 h after birth
4. Infant F III Midline Died shortly after birth, cyclopia
5. Infant M III Midline Stillbirth
6. Nyberg et al. (1987) Fetus Unknown III Midline Termination of pregnancy, cyclopia
7. Infant Unknown III Midline Stillbirth
8. Souza et al. (1990) Infant F III Midline Stillbirth
9. Galguera et al. (1996) Infant M IV Midline Died 27 d after birth, cyclopia
10. Chen et al. (1999) Fetus Unknown III Midline Termanation of pregnancy, cyclopia
11. Situ et al. (2002) Fetus M III Midline Stillbirth, cyclopia
12. Granto et al. (2005) Infant F III Midline Died 9 mo after birth
13. Infant M III Midline Died 40 d after birth
14. Cho et al. (2005) Fetus Unknown III Midline Termanation of pregnancy, cyclopia
15. Boahene et al. (2005) Fetus M IV Midline Termanation of pregnancy, cyclopia
16 Lee et al. (2006) Fetus F II Midline Termanation of pregnancy
17. Capobianco et al. (2007) Infant M III Midline Died just after birth. cyclopia
* Group according to Boo-Chai’s Classification.
{ Position of proboscis.

TABLE 4 Proboscis With Normal Intercanthal Distance

No. Authors Age Sex Group* Side{ Remarks

1. Dasgupta et al. (1971) 19 yr M I Midline Normal nose and loss of vision

2. Kayikçioğlu (2000) Infant M I Left Otherwise completely normal
3. Mutaf et al. (2006) 1 mo M I Right Otherwise completely normal
4. Jost et al. (1995) 6y F II Left Defect of left ala nasi
5. 17 y M II Right Atrophy of right nostril
6. Acarturk et al. (2006) 3 mo M II Left Defect of left ala nasi
* Group according to Boo-Chai’s Classification.
{ Position of proboscis.

TABLE 5 Proboscis Plus Hypertelorism Without Encephalocele

No. Authors Age Sex Group* Side{ Remarks

1. Kirkpatrick (1970) 1/2 mo M III Right Coloboma palpebral

2. D’assumpção (1975) 8 mo M III Left Coloboma of left lower lip
3. Rontal and Duritz (1977) Infant M II Right Disrupted medial wall of orbit
4. Bowe (1986) 5 mo Unknown III Left Coloboma of the upper palpebrum
5. Belet et al. (2002) Infant M III Right Coloboma of right lower lip
6. Eroğlu and Uysal (2003) Infant M III Right Coloboma of right lower lip
7. Abou-Elhamd (2007) Infant M III Left Coloboma of left upper lip
8. 7 mo M III Left Coloboma of left lower lip
9. Guion-Almeida et al. (2004) 1 mo M III Left Dislocation of left orbit
10. Acarturk et al. (2006) 1y F III Right Coloboma of right lower lip
11. Thorne et al. (2007) 1y F III Left Defect of left ala nasi, coloboma iris
12. McLaren (1955) 2 mo F IV Left Coloboma iris, cleft lip and palate
13. Boo-Chai (1985) 6 mo F IV Left Microphthalmia, cleft lip and palate, coloboma
of lower lid
14. Belet et al. (2002) Infant M IV Right Absence of nasolacrimal apparatus, cleft lip
15. Eroğlu and Uysal (2003) Infant M IV Right Atresia of nasolacrimal duct, cleft lip
16 Uğurlu et al. (2005) 8y F IV Right Ocular deformities, cleft lip and palate
17. Sakamoto et al. (2010) 6 mo F IV Right Atresia of nasolacrimal duct, cleft lip and palate
* Group according to Boo-Chai’s Classification.
{ Position of proboscis.

al., 2010). Therefore, the six cases with cleft lip/palate were
compatible with Boo-Chai group IV, and the other 11 were
compatible with group III (Table 5). Eight of 11 cases
classified as group III and four of six cases classified as
group IV had a single nostril. The other five cases had two
nostrils, but the nostrils on their affected side were
micronostrils (Kirkpatrick, 1970; Bowe, 1986; Guion-
Almeida et al., 2007).
The other 10 cases associated with a visible encephalocele
had encephalopathy and most died at an early age; long-
term survival cases suffered developmental delay and
mental retardation (Table 6) (Antoniades and Baraister,
1989; Gilbert-Barness et al., 2001; Harada and Muraoka,
2001; Guion-Almeida et al., 2007). Some of these were
diagnosed as cerebro-oculo-nasal syndrome (Chen et al.,
1999).
204 Cleft Palate–Craniofacial Journal, March 2012, Vol. 49 No. 2

TABLE 6 Proboscis Plus Hypertelorism Plus Visible Encephalocele

No. Authors Age Sex Group* Side{ Remarks

1. Antoniades and Baraister (1989) 2.6 y F IV Right Developmental delay at 2.6 y

2. Gilbert-Barness et al. (2001) Infant M IV Bilateral Died 5 min after birth
3. Harada and Muraoka (2001) 0.5 mo M IV Bilateral Suggested severe functional brain damage
4. Guion-Almeida et al. (2007) 8 mo F IV Bilateral Severe developmental delay at 22 mo
5. 5 mo F III Bilateral Died 17 months after birth
6. 6 mo M III Left Died 7 mo after birth
7. 20 mo M IV Bilateral Died 3 y after birth
8. 1 mo F III Bilateral Developmental delay at 5 mo
9. 4 mo F III Bilateral Severe developmental delay at 3.6 y
10. 6 mo M IV Left Died 7 y after birth
* Group according to Boo-Chai’s Classification.
{ Position of proboscis.

TABLE 7 New Classification of Proboscis Lateralis

Group Interorbital Distance Nose Eyes and Adnexa Cleft Lip/Palate Cranium and Brain

I Normal Normal Normal None

II Normal Defect (Usually defect of ala nasi) Normal None
III Hypertelorism Defect (Usually defect of ala nostril) Abnormal None
IV Hypertelorism Defect Abnormal Complicated
V Hypertelorism with Defect Abnormal Either Cerebro-oculo-nasal
encephalocele syndrome
VI Hypotelorism Defect (Usually defect of nose) Abnormal (cyclopia) Either Holoprosencephaly

DISCUSSION hypertelorism. Therefore, we considered that a more

A New Classification Scheme of Proboscis Lateralis Based
on Intercanthal Distance
To differentiate holoprosencephaly from other anomalies
with good prognosis, DeMyer (1967) suggested that the
face predicts the brain. Given that holoprosencephaly
shows significant hypotelorism, he emphasized the impor-
tance of intercanthal distance for differentiating brain
defects. On the other hand, Rontal and Duritz (1977)
suggested that one cause of proboscis lateralis is distur-
bance in the growth of the maxillary process. Because
underdevelopment of the maxillary process leads to
hypoplasia of the medial canthal region, an appreciable
number of individuals with proboscis lateralis show
reliable classification scheme may be based on intercanthal
distance.
Based on comparison of our results with those of the Boo-
Chai scheme, we propose a new classification scheme of
proboscis lateralis (Table 7). In addition to the four groups
of Boo-Chai’s classification are two others: Group V with
encephalocele and group VI with holoprosencephaly. All
high-risk cases were excluded from these two groups.
Hypothesis of the Development of Proboscis Lateralis
The precise embryologic mechanism responsible for the
development of proboscis lateralis has not been defined.
Popular theories were based on the nasal placode and
maxillary prominence (Rontal and Duritz, 1977), but these

FIGURE 2 In the fourth week, a pair of nasal placodes developed on the frontonasal prominence. Mesenchyme proliferates to produce horseshoe-shaped
elevations that are medial nasal prominence and lateral nasal prominence. The medial nasal prominences fuse to form the columella, and ala nasi is formed from
the lateral nasal prominences. Each maxillary prominence begins to fuse with the globular prominence to form the philtrum.
 Sakamoto et al., NEW CLASSIFICATION SCHEME OF PROBOSCIS LATERALIS 205

FIGURE 3 The proposed hypothesis regarding the genesis of various patterns of proboscis lateralis.

theories could not identify or explain all cases. Therefore,
we propose a new hypothesis of how proboscis lateralis
develops based on our new classification scheme.
To consider the formation of this abnormality it is first
necessary to outline the development of the face. This
begins at an early embryonic stage, at approximately the
fourth week. The five facial primordia appear as promi-
nences: the single frontonasal prominence, the paired
maxillary prominences, and the paired mandibular prom-
inences. These five facial prominences are active centers of
206 Cleft Palate–Craniofacial Journal, March 2012, Vol. 49 No. 2
growth in the underlying mesenchyme. By the end of the
fourth week, a pair of nasal placodes, which are an elliptical
thickening of the surface ectoderm, has developed on the
inferolateral parts of the frontonasal prominence. Mesen-
chyme in the margins of the placodes proliferates,
producing horseshoe-shaped elevations that are the medial
and lateral nasal prominences. As a result, the nasal
placodes lie in depressions called nasal pits. These pits are
the nasal sacs, the primordia of the anterior nostrils and
nasal cavities (Fig. 2). During expansion of the nasal sacs,
the medial nasal prominences fuse to form the columella.
By the end of the sixth week, each maxillary prominence
has begun to merge with the globular prominence to form
the philtrum, the intermaxillary segment of the upper jaw,
and the primary palate. Also, the lateral nasal prominences
form the ala nasi. By week 14, facial development is
complete (Moore, 2003).
The following is our hypothesis of the development of
proboscis lateralis based on our classification (Fig. 3).
First, in group II, it is supposed that during the
proliferation of mesenchymal cells in the medial nasal
prominence, a fissure or concavity appears accidentally in
this area, and thereby divides this medial nasal prominence
into two segments. The medial part merges into the
unaffected side and forms the columella. However, the
lateral nasal prominence of the affected side merges into the
adjacent lateral part and forms a proboscis lateralis instead
of ala nasi. The development of the maxillary prominence is
normal in this group.
Groups III and IV are associated with the degree of the
hypoplasia of the maxillary prominence advocated by
Rontal and Duritz (1977).
The development of group I is different from other
groups, and its development has been proposed to resemble
that of a double nose (Nakamura and Onizuka, 1986). The
pair of nasal placodes is formed in a vertical arrangement
on one side. The lower placode adheres to the other side
and forms a normal nose; whereas, the upper placode
develops into a proboscis lateralis because there is nothing
to which to adhere on that side. Such a development is
likely to be very rare, which explains why group I has the
least number of cases.
If there is a swelling like a tumor or encephalocele at the
center, growth of the prominences is disturbed. Each
prominence fuses in various forms dictated by the balance
of right and left growth, and sometimes proboscis lateralis
appears as a case in group V.
Group VI (type I or II holoprosencephaly) is caused by a
median deficiency of tissue, including aplasia of the median
nasal prominence. As a result, lateral nasal prominences
adhere to each other and form a proboscis lateralis
presenting with hypotelorism.
Moreover, the definition of ocular anomalies become
clearer by taking into account intercanthal distance.
Intercanthal distance increases from group I to group IV,
and other accompanying deformities also become more
severe. We believe our classification scheme to be not only
convenient for clinical application but also embryologically
accurate.
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