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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2007  |  Volume : 11  |  Issue : 2  |  Page : 80-82
 

Lateral facial cleft, accessory maxilla and hemifacial microsomia: An uncommon triad


Department of Oral and Maxillofacial Surgery, Meenakshiammal Dental College and Hospital, Alappakkam Main Road, Maduravoyal, Chennai - 600 095, India

Correspondence Address:
P Anantanarayanan
Department of Oral and Maxillofacial Surgery, Meenakshiammal Dental College and Hospital, Alappakkam Main Road, Maduravoyal, Chennai - 600 095
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.37389

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   Abstract 

This is a report of a unique case of lateral facial cleft (Tessier 7 soft tissue) combined with a posterior maxillary cleft (Tessier 6 skeletal) and mandibular hypoplasia presented in an eight-year-old boy. The varied clinical findings, along with the rarity of the occurrence, mandate documentation of this entity and a thorough review of literature.


Keywords: Hemifacial microsomia, lateral facial cleft, macrostomia, maxillary cleft, tessier cleft


How to cite this article:
Anantanarayanan P, Manikandhan R, Thomas TK, Satish Kumar M. Lateral facial cleft, accessory maxilla and hemifacial microsomia: An uncommon triad. J Oral Maxillofac Pathol 2007;11:80-2

How to cite this URL:
Anantanarayanan P, Manikandhan R, Thomas TK, Satish Kumar M. Lateral facial cleft, accessory maxilla and hemifacial microsomia: An uncommon triad. J Oral Maxillofac Pathol [serial online] 2007 [cited 2019 Aug 18];11:80-2. Available from: http://www.jomfp.in/text.asp?2007/11/2/80/37389



   Introduction Top


Macrostomia, commissural or lateral facial clefts (Tessier 7 soft tissue) are unusual lesions that result from failure of the embryonic mandibular and maxillary processes of the first branchial arch to fuse properly and form the corners of the mouth. It may be seen alone or in combination with other anomalies, accompanied by varying degree of severity and extent. It may range from a simple presentation in the form of a wide oral aperture with loss of commissural anatomy to a full-thickness defect of the face extending from the mouth to the tragus. Associated anomalies may also be present in the form of pre-tragal skin tags, defective development of the auricle and external ear, absence of the  Eustachian tube More Details, abnormalities of the temporomandibular joint, zygomatic arch, eyelids and polydactyly. [1],[2],[3],[4],[5],[6] There may also be varying degrees of skeletal hypoplasia and discontinuity of bone between maxilla and zygomatic bone, frontal and zygomatic bone and between the temporal and zygomatic bone. These lesions are usually unilateral and rarely bilateral. [7] Lateral clefts are the most common oro-facial clefts, second to the more common isolated cleft lip, with an incidence of 1 in 3,500 to 5,000 live births; while hemifacial microsomia is reported at 1 in 3,500 to 26,550 live births. [8] The occurrence of lateral clefts and hemifacial microsomia is even believed to be a part of the same spectrum of congenital anomalies and has been documented and discussed in detail.

The incidence of alveolar cleft is more common and generally ranges from 1 in 1,000 to 1 in 1,200 live births. The anterior maxillary alveolar cleft (Tessier 2 skeletal) is the most common form, while the posterior maxillary cleft (Tessier 5, 6 or 7 skeletal) is much rarer. [6] Earlier documentations of these posterior maxillary clefts have used the term 'maxillary duplication.' Duplication of jaws carrying teeth may occur in the mandible or maxilla, either as an accessory process or more rarely, a complete jaw. [2] The combination of a lateral facial cleft and a posterior maxillary skeletal cleft, however, has negligible documentation, making this presentation significant.

The embryological origin of these entities is less understood and still evokes controversies. Five developmental processes aid in the formation of the mouth: the paired mandibular processes that form the lower segment, the frontonasal process that contributes to the middle of the upper part and the paired maxillary processes that form the lateral segments of the upper part. The primitive mouth, or stomodeum, is larger in relation to the face during the early stage of facial development. The maxillary and mandibular processes grow forward and merge laterally and complete the development of the mouth. A failure of the maxillary and mandibular processes to merge or a disruption in the processes after merging can result in a transverse lateral facial cleft. [1] However, Gorlin [4] and others claim that the lateral facial cleft more often represents a post-merging disruption because there is considerable clinical variability in expression. During the development of a lateral facial cleft, no distinct etiological factor is found, but it may represent a multifactoral congenital anomaly.


   Case Report Top


An eight-year-old boy reported to the outpatient clinic with a chief complaint of a deformed face and a wide mouth since birth. His perinatal history was normal and uneventful. His milestones were recorded to be normal. He showed good psychological development, abilities of comprehension, cognitive skills and was of average intelligence, attending primary school.

Examination revealed gross facial asymmetry with a lateral facial cleft on the right side, extending from the commissure (which was absent) up to the anterior border of masseter. A linear depression present from the end of the cutaneous component of the cleft to zygomatic arch, demonstrated a subcutaneous component of the cleft with disruption of the underlying muscles. There was also a reduction in the size of the right external ear. An epicanthal fold was seen on the right eye, while the left was normal. The lip presented with good morphology on the unaffected side with satisfactory competence. Clinically, there was a reduction in the mandibular dimensions on the right side when compared with the left [Figure - 1],[Figure - 2].

Intraoral examination revealed mixed dentition in class I occlusion with a 10 mm overjet. There was crowding of the right posterior maxillary teeth behind the 1 st deciduous molar, and the teeth appeared clustered and not in normal form, showing definitive discontinuity from the maxillary dental arch [Figure - 3]. There was no functional impairment noted during mastication or other mandibular movements. There was a mild resonance impairment affecting his speech, which was otherwise intelligible.

Conventional radiographs and CT imaging confirmed the right-sided mandibular hypoplasia and the presence of a cleft in the posterior maxilla on the same side, due to which the erupted and unerupted teeth appeared in a cluster within bone not attached to the dento-alveolar process. This was originating from the root of the zygomatic arch and temporal process of the zygomatic bone [Figure - 4],[Figure - 5].

Management of this deformity was planned in two stages, with primary correction of macrostomia using a modified W plasty with muscle reconstruction and management of the hard tissues later.


   Discussion Top


This case concurs with the findings of Rushton and Walker [2] (1937) and Cheung et al., [2] who postulate that the phenomenon of dual manifestation of accessory maxilla and transverse facial clefts is perhaps not coincidental, but rather a distinct syndrome, with its pathogenesis different from either of the deformities when occurring isolated.

The variable presentation of the hypoplastic mandible with the facial clefts represents the broad spectrum of hemifacial microsomia and has been documented. [8] Similarly, the presentation of an accessory maxilla with a lateral cleft is also reported. [2] This unique presentation manifests all the three entities mentioned above.

The pathogenesis of lateral cleft, as well as the posterior maxillary cleft, is not firmly established. There are two main theories of its development. His (1892) [4] proposed that the cleft occurs due to fusion between different processes forming the face (the maxillary and mandibular and the maxillary and zygomatic processes). This was challenged by proponents of the mesenchymal penetration theory, which holds that there is deficiency in mesenchymal penetration of this embryonic region, resulting in epithelial breakdown and subsequent facial cleft formation. The etiological causes of the lateral facial cleft appear to be heterogeneous: (1) vascular cause, attributable to embryonic hematoma formation from disruption of the stapedial artery stem, the severity of the condition depending on the size of the hematoma and its time of resolution as brachial arch development occurs, which is similar to hemifacial microsomia; and (2) environmental factors, including an increasing number of infectious diseases, drugs or chemical agents capable of inducing embryonic dysmorphogenesis.

Mulliken et al. proposed cleft lip, palate and facial clefts occur in about 10% of hemifacial microsomia cases and that the entire spectrum of facial clefts can manifest as a component of the broad grouping of the 'Hemifacial Microsomia Syndrome.' This can be based on the definition that a syndrome is a recurrent pattern of multiple anomalies pathogenically related and occurring in embryologically noncontiguous regions. [8] He also reports that the pathogenic mechanism for cleft lip/palate is similar to hemifacial microsomia when they are associated and is different when isolated.

Cheung et al. [2] postulated that in the deep tissue between embryonic maxilla and mandible, a late compensatory growth from the embryonic temporal bone could have penetrated into this region before the complete breakdown of the overlying ectoderm. The ectodermal cover of this mesodermal budding will form the tooth germs in the future accessory maxillae.

We would conclude that this case is a rare presentation of three entities - (1) lateral facial cleft, (2) accessory maxilla and (3) hemifacial microsomia - making it an extended expression of the 'Hemifacial Microsomia Syndrome.'

 
   References Top

1.Askar I, Gurlek A, Sevin K. Lateral facial clefts (macrostomia). Ann Plast Surg 2001;47:355-6.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Cheung LK, Samman N, Tidemann H. Bilateral transverse facial clefts and accessory maxilla-variant or separate entity. J Craniomaxillofac Surg 1993;21:163-7.  Back to cited text no. 2    
3.Eppley BL, van Aalst JA, Robey A, Havlik RJ, Sadove AM. The spectrum of orofacial clefting. Plast Reconstr Surg 2005;115:101e-14e.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Gorlin RJ, Cohen MM Jr, Levin L. Orofacial clefting syndromes: General aspects. In : Motulsky A, Harper P, Bobrow M, Scriver C, editors. Syndromes of the Head and Neck . Oxford University Press: New York; 1990. p. 697-84.  Back to cited text no. 4    
5.Yaneha MW. Congenital macrostomia. Otolaryngol Head Neck Surg 2001;124:353-4.  Back to cited text no. 5    
6.Tessier P. Anatomical classification of facial, craniofacial and laterofacial clefts. J Maxillofac Surg 1976;4:69-92.  Back to cited text no. 6  [PUBMED]  
7.Hunt JA, Hober PC. Common craniofacial anomalies: Facial clefts and encephaloceles. Plast Reconst Surg 2003;112:606-15.  Back to cited text no. 7    
8.Fan WS, Mulliken JB, Padwa BL. An association between hemifacial microsomia and facial clefting. J Oral Maxillofac Surg 2005;63:330-4.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]



 

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