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| LETTER TO THE EDITOR |
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| Year : 2014 | Volume
: 7
| Issue : 1 | Page : 104-105 |
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A rare occurrence of Tessier's 0 cleft in siblings
Dinesh Singh Chauhan, Yadavalli Guruprasad
Department of Oral and Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur, Karnataka, India
| Date of Web Publication | 10-Dec-2013 |
Correspondence Address:
Yadavalli Guruprasad
Department of Oral and Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur - 584 103, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-2870.122808
How to cite this article:
Chauhan DS, Guruprasad Y. A rare occurrence of Tessier's 0 cleft in siblings. Med J DY Patil Univ 2014;7:104-5 |
Sir,
Craniofacial clefts are among the most disfiguring of all facial anomalies. They exist in a multitude of patterns and with varying degrees of severity; some patients may demonstrate a faint, cutaneous manifestation or microform, whereas another will display more complete skeletal and soft-tissue manifestation. Craniofacial clefts are rare and occur sporadically. According to Tessier classification, the no. 0 cleft is a median craniofacial dysraphia. It may be also referred to as internasal dysplasia, median cleft face syndrome and frontonasal dysplasia. We report a rare case of Tessier's 0 cleft occurring in siblings.
Two boys aged 6 years and 3 years were referred to Department of Oral and Maxillofacial Surgery for evaluation of deformed nose. On detailed history, the deformity was congenital and delayed reporting to the hospital due to their socio-economic background and rural origin. There was no history of consanguinous marriage or drug intake during pregnancy of both the children. Clinical examination revealed midline bifid nose deformity in both the siblings with a broad columella and a wide midline furrow with thick tissue of fibromuscular band [Figure 1], [Figure 2], [Figure 3]. Clinical diagnosis of Tessier's 0 cleft was made with no other significant craniofacial findings. Staged surgical treatment was planned, initially resecting excess tissue in the midline with domal suturing followed by more refined rhinoplasty using cartilage grafting after post-adolescence. | Figure 1: Clinical photograph showing siblings with Tessier's 0 cleft involving nose
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 | Figure 2: Frontal view photograph of elder sibling showing Tessier's 0 cleft thus giving bifid nose appearance
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 | Figure 3: Frontal view photograph of younger sibling showing Tessier's 0 cleft thus giving bifid nose appearance
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The most accepted and purely utilitarian categorization of facial clefts was given by Ozaki and Kawamoto and Tessier. [1],[2] The clefts are numbered from 0-14 starting in the midline and using the orbit as the reference point. According to this scheme no.0 and no.14 constitute median craniofacial dysrhaphia. The cleft goes through the frontal bone with duplication of the crista galli, the midline of nose with nasal septal duplication, the columella, midline of upper lip and maxilla. The developmental origin of Tessier no. 0 is not clearly understood; however, it is known that midline facial defects can be traced to a period corresponding to the 3 rd week of gestation. The no. 0 cleft is the most common of craniofacial clefts and can be present either as a widening or duplication of the midline structures, or as agenesis/hypoplasia of these structures. The nose is often bifid, with a broad columella and a wide midline furrow. The nasal septum is thickened and often duplicated. The alar and upper nasal cartilages are hypoplastic and often displaced laterally. [3],[4] A thick subcutaneous fibromuscular band from the alar cartilages to the frontal bone can pull the columella upward and make the nose appear shorter. [4] Treatment is often a staged surgical procedure, resecting excess tissue in the midline with domal suturing initially followed by more refined rhinoplasty using cartilage grafting after post-adolescence. [5],[6] Tessier's 0 cleft occurring in siblings is very rare and very few cases of craniofacial clefts occurring in siblings are reported in the literature.
| References | |  |
| 1. | Ozaki W, Kawamoto HK Jr. Craniofacial clefting. In: Lin K, editor. Craniofacial Surgery: Science and Surgical Technique. Philadelphia, PA: WB Saunders; 2001. 
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| 2. | Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92.
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| 3. | Fogh-Andersen P. Rare clefts of the face. Acta Chir Scand 1965;129:275-81.
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| 4. | Krikun LA. Clinical features of median cleft of nose. Acta Chir Plast 1972;14:137-48.
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| 5. | da Silva Freitas R, Alonso N, Shin JH, Busato L, Ono MC, Cruz GA. Surgical correction of Tessier number 0 cleft. J Craniofac Surg 2008;19:1348-52.
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| 6. | Jhamb A, Mohanty S. A chronicle of Tessier no. 0 and 1 facial cleft and its surgical management. J Maxillofac Oral Surg 2009;8:178-80.
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[Figure 1], [Figure 2], [Figure 3]
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