Surgical Correction of Bifid Nose Due to Tessier’s No. 0 Cleft

doi:10.4236/mps.2014.41001

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Modern Plastic Surgery, 2014, 4, 1-4

Published Online January 2014 (http://www.scirp.org/journal/mps)

http://dx.doi.org/10.4236/mps.2014.41001

OPEN ACCESS MPS

Surgical Correction of Bifid Nose Due to Tessier’s No. 0

Cleft

Hiroki Shibayama1, Takatoshi Yotsuyanagi2, Tamotsu Saito2, Ken Yamashita2, Makoto Yamauchi2,

Tetsuya Suda3

1Department of Plastic and Reconstructive Surgery, Fujisawa City Hospital, Kanagawa, Japan; 2Department of Plastic and Recon-

structive Surgery, Sapporo Medical University School of Medicine, Hokkaido, Japan; 3Department of Plastic and Reconstructive

Surgery, Sunagawa City Medical Center, Hokkaido, Japan.

Email: [email protected]

Received October 18th, 2013; revised November 15th, 2013; accepted November 23rd, 2013

cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In

ABSTRACT

Bifid nose is a rare congenital abnormality and its surgical correction is difficult. We represent a case of bifid

nose with mild nasal deformity without cerebral or lip deformity. We performed rhinoplasty with a kind of the

forked flap at one year of age and the appearance improved.

KEYWORDS

Bifid Nose; Rhinoplasty; Forked Flap; Tessier’s No. 0 Cleft

1. Introduction

Bifid nose is one of the features of Tessier’s No. 0 or No.

14 craniofacial clefts [1], and is caused by the failure of

the medial and lateral nasal processes to achieve contact

with each other during the first eight weeks of fetal life.

There are few reports of the surgical correction of bifid

nose, and we describe here our experience of bifid nose

due to Tessier’s No. 0 cleft. We performed rhinoplasty

with a type of the forked flap [2] at one year one month

of age and achieved relatively good results.

2. Case Report

A one-month-old boy presented with congenital nasal

disfigurement and a subcutaneous tumor on the dorsum

of the nose. He had no anomalies of other organs, and his

delivery was normal. His close relatives had no conge-

nital anomaly. Grooving of the nasal tip and columella

was observed. His nose was relatively short. The width

of the columella was increased and the distance between

the nostrils was long. Canthal index was 47. There was

an elastic soft subcutaneous tumor on the dorsum of the

nose. Median cleft lip, cleft palate and duplicated labial

frenulum were not present (Figure 1). Computed-tomo -

graphy revealed that the nasal bone was flattened but the

nasal septum was normal. No central nervous system

anomalies such as cranium bifidum or a frontonasal en-

cephalocele were recognized. The tumor was suspected

to be a lipoma (Figure 2).

Figure 1. First visit to our hospital. There is a groove at the

nasal tip and the dorsum of the nose and the columella were

wide.

Surgical Correction of Bifid Nose Due to Tessier’s No. 0 Cleft

OPEN ACCESS MPS

We perfomed rhinoplasty and tumor excision at one

year one month of age under general anesthesia. The

design was similar to a forked flap, to elevate the nasal

tip (Figures 3 and 4). After elevation of the columella,

we found that the anterior nasal spine was wide and both

alar cartilages were displaced laterally (Figure 5). The

cartilages were moved medially and sutured to each other

after excision of redundant subcutaneous tissue, then the

flap forming the philtrum was fixed. Lastly, the flaps

forming the columella were trimmed and sutured (Fig-

ures 6 and 7). Pathological examination of subcutaneous

tissue showed lipoma, with no malignancy. Six months

after the operation, the appearance of his nose was rela-

tively improved and all scars were inconspicuous (Fig-

ures 8 and 9).

3. Discussion

Bifid nose has been called by several names for a long

time. In 1889, Trendelenburg [3] was described this fin-

dings as “Doggennase”, and this is re cog nize d as the first

report about bifid nose. In 1910 Wilkinson [4] firstly

used the term “bifid nose”. In 1967 DeMyer [5] sug-

Figure 2. Computed-tomography showed the flattened nasal

bone, however the nasal sept um was normal.

Figure 3. Design: We performed rhinoplasty with a kind of

“forked flap”.

Figure 4. Lateral view before surgery.

Figure 5. The anterior nasal spine was wide and the alar

cartilages were separated. There was redundant tissue be-

tween the cartilages.

Figure 6. Immediately after the surgery: Front view.

Surgical Correction of Bifid Nose Due to Tessier’s No. 0 Cleft

OPEN ACCESS MPS

Figure 7. Immediately after the surgery: Lateral view.

Figure 8. Six months after the surgery: Front view.

Figure 9. Six months after the surgery: Lateral view.

gested “median cleft face syndrome” for congenital de-

formities of the medial area of the face. However Sedano

[6] rejected this suggestion because DeMyer’s definition

was inadequate, and presented a new concept, “fronto-

nasal dysplasia”. After that, the concept of congenital

deformities of the medial area of the face became com-

plicated. In 1976 Tessier reported a classification of cra-

niofacial cleft, and now, this classification is most widely

accepted. In the classification, bifid nose is associated

with No. 0 and No. 1 4 cl e ft s.

The cause of bifid nose is known to be the failure of

the medial and lateral nasal processes to achieve contact

with each other during the first eight weeks of fetal life.

Many theories and states such as central disorganization

of the neural crest, external pressure, oligohydramnios,

amniotic bands, and hematoma have been proposed as

etiological mechanisms of th is congenital anomaly.

Tessier’s No. 0 or No. 14 cleft often presents with bi-

fid nose, median cleft lip and hypertelorism. The degree

of disease is varies in each patient, and the associated

brain malformation generally limits the life span to in-

fancy. There are some reports of associated congenital

anomalies of other than the face, but their relationship

with these clefts is unclear [7]. In bifid nose, some ver-

tical skin grooves are generally observed in the nasal tip

or dorsum and lower nasal tip. The dorsum and root of

the nose are broad and flattened. The alar and lateral car-

tilages are displaced laterally, and the nasal bone and

septum become separated or thick. In No. 0 cleft, the

manifestations are deformities of the nose and nasal sep-

tum. This cleft is often associated with median cleft lip

and cleft palate, and some authors have reported patients

with lipoma, epidermoid cyst and teratoma [8]. The ma-

nifestations of No. 14 are deformities of the orbit, and

associated brain or cranial deformities such as holopro-

sencephaly or microcephaly are often found. In the

present case, the patient presented with nasal deformities,

hypertelorism and lipoma without brain or cranial defor-

mity, and we considered this to be Tessier’s No. 0 cleft .

The treatment of bifid nose was first reported by Jo-

seph [9] in 1931, who described composite grafting for

mild cases, a VY advancement flap for moderate cases,

and a forehead flap with free bone grafting for severe

cases. For mild or moderate cases, some authors sug-

gested modifications of Joseph’s technique. In 1949

Webster [10] described osteotomy of the widened nasal

bone and maxilla, and he replaced the nasal cartilage

medially by suturing them to each other. After bone

and/or cartilage surgery, soft tissue reconstruction was

performed, such as excision of the redundant skin or V-Y

plasty. In 1958 Peer [11] reported suture of the alar car-

tilages for mild cases. In 1987 Ortiz [12] suggested ac-

tive osteotomy in 59 cases in which the patient had a

bony cleft. However, in 2005 Tayfun [13] stated that

Surgical Correction of Bifid Nose Due to Tessier’s No. 0 Cleft

OPEN ACCESS MPS

active ostetomy should not be perfomed, in order to

avoid an adverse effect on nasal growth.

In this case we planned replacement of the alar carti-

lages and designed a forked flap to narrow the columella

and heighten the nasal tip because the nasal deformity

was mild. After incision, we removed the redundant soft

tissue between the alar cartilages, and then sutured them

to each other. In view of the patient’s age, we did not

perform osteotomy or cartilage grafting. Although the

timing of surgery is controversial, we think it should not

be performed before one year of age in consideration of

the risk of general anesthesia.

4. Conclusion

We performed rhinoplasty with a type of forked flap for

bifid nose patient at one year one month of age and

achieved relatively good results. Patients with bifid nose

are classified into No.0 or 14 cleft by Tessier’s classifi-

cation and we should plan a surgery for each patient in-

dividually because nasal malformations are different in

each case.

REFERENCES

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