Home | About Us | Editorial Board | Current Issue | Archives | Search | Instructions | Subscription | Feedback | e-Alerts | Login 
Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
 Users Online: 355  
 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size


 
CASE REPORT
Year : 2007  |  Volume : 25  |  Issue : 1  |  Page : 39-42
 

Crossed hemifacial hyperplasia: A diagnostic dilemma


Department of Pedodontics and Preventive Dentistry, Manipal College of Dental Sciences, Manipal, Karnataka, India

Correspondence Address:
R Nayak
Department of Pedodontics and Preventive Dentistry, Manipal College of Dental Sciences, Manipal, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.31989

Rights and Permissions

 

   Abstract 

Crossed hemifacial hyperplasia is a rare condition producing facial asymmetry and overgrowth of the extremities of the opposite side. Very few cases are reported in literature. A case of an 11-year-old female is presented here to supplement existing clinical knowledge with many of the reported clinical and orofacial findings. Emphasis is placed on diagnosis of the condition by thoughtful elimination and the necessity of a multi-disciplinary approach to its management.


Keywords: Crossed hemifacial hyperplasia, asymmetry, alveolar process


How to cite this article:
Nayak R, Baliga M S. Crossed hemifacial hyperplasia: A diagnostic dilemma. J Indian Soc Pedod Prev Dent 2007;25:39-42

How to cite this URL:
Nayak R, Baliga M S. Crossed hemifacial hyperplasia: A diagnostic dilemma. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2019 Nov 18];25:39-42. Available from: http://www.jisppd.com/text.asp?2007/25/1/39/31989



   Introduction Top


Subtle, asymmetric variations of the contralateral structures of the head and face occur commonly in the general population and are an accepted feature of morphogenesis. [1] Occasionally, a gross asymmetry easily perceptible to the eye may occur either due to physiologic reasons or due to a growth abnormality. [2] One such entity, characterized by marked unilateral over development of hard and soft tissues of head and face is a rare congenital malformation. This has been termed variously in literature as facial hemihypertrophy, partial/unilateral gigantism, hemifacial hyperplasia. [1],[3]

Hemi hypertrophy was first documented by Meckel. [4] Gessel [5] described hemihypertrophy as 'essentially a developmental anomaly antedating birth and arising in some way as a partial deflection of the normal process of birth'. Ward and Lerner [6] stated that asymmetric enlargement could be manifested in a unilateral/crossed configuration and may involve all the body tissues in the area (i.e., total) or a single tissue (i.e., limited)

Crossed hemifacial hyperplasia is a rare condition that produces facial asymmetry along with a co-existent enlargement of the opposite lower extremity. [7] Case reports of this entity are rare in literature. Pollock and Colleagues [3] reviewed 192 cases of facial hemihypertrophy and found only five reported cases of crossed hyperplasia Tommasi et al . [7] reported another case.

The purpose of this report is to present the case history of a child with crossed hemifacial hyperplasia to supplement existing clinical knowledge.


   Case Report Top


An 11-year-old female patient was referred to Department of Pedodontics, for opinion regarding enlargement of the gums on the right side of her lower jaw. Patient presented to our clinic with a complaint of enlargement of gums noticed 3 years back, which was also associated with simultaneous appearance of skin lesions and a bony swelling on the left side of the head since 6 months. The child's father and aunt were the informers. The child was the second of the two siblings born of a non-consanguineous marriage, at full term by normal delivery. No significant postnatal illness was reported. The developmental milestones were normal. Child was of normal intelligence, doing well at school. There was no history of similar complaints in the family. The child was of average build, moderately nourished with the following anthropometric measurements: Height: 136 cm, Weight: 24 kg, Head circumference: 52 cm and was at SMR stage 11 Patients left extremities were larger than the right extremities [Figure - 1],[Table - 1].

A bony swelling was observed on the left parietal bone measuring 4x3 cm in size and was non-tender. Skin over swelling was mobile with no discharge from the area. Skin showed evidence of maculopapular lesions with increased hair growth on the left side of the body and back.

Extra oral examination of the patient revealed an obvious asymmetry of the face with the right side appearing larger than that of the left side. The asymmetry was evident only on the lower half of the right side of the face. The right half of the lower lip was hypertrophied. Maculopapular skin lesions were seen on the right malar region [Figure - 2].

Intra-oral examination revealed diffuse enlargement of gingiva on the right side of the lower jaw, which abruptly stopped at the midline, especially marked in the canine, premolar and incisor region. The surface of the enlarged gingival tissue was rough, stippled, pigmented, and firm in consistency with no evidence of any inflammatory component. Anteriorly the tissue almost covered the labial aspect of the lower incisors sparing the incisal edges [Figure - 3]. The right half of the tongue was enlarged with polypoid excrescences representing enlarged fungiform papillae. An abrupt midline difference was evident [Figure - 4]. Buccal mucosa appeared normal.

The dentition exhibited accelerated physiological development only in relation to lower right quadrant. The already erupted mandibular right second permanent molar was bigger in size and possessed a large supernumerary cusp on its buccal aspect [Figure - 5]. The occlusal plane was unequal with the right-sided teeth erupting faster than the left-sided teeth. Midline was shifted to the left. All other areas of the oral cavity were normal.

An orthopantomogram revealed advanced dentition development on the right side of lower jaw with erupted second permanent molar. The alveolus appeared thickened on the right side of mandible. There was accelerated root formation of all the right lower teeth. Enlarged pulp chambers of right lower canine and right lower second premolar were noticed [Figure - 6]. PA view of the skull showed normal study except an obvious enlargement of the mandible on the right side. A pediatric consultation was arranged and no systemic abnormality was detected. The skin lesions were diagnosed as connective tissue nevi on biopsy. Except for a mild elevation in the serum alkaline phosphatase levels, all other routine blood investigations were within normal limits.

Based on the findings of the investigations, a gingivectomy was performed for esthetic reasons and specimen sent for histopathology. The specimens showed a fibro-epithelial hyperplasia suggestive of gingival hypertrophy. Based on the findings, following differential diagnosis were kept in mind in our case:

  1. Idiopathic gingival fibromatosis
  2. Fibrous dysplasia, Albright's syndrome
  3. Neurofibromatosis
  4. Hyperparathyroidism


But the absence of cafι au lait spots, normal SMR and involvement of dentition and soft tissues ruled out these differential diagnoses and final diagnosis of crossed hemifacial hyperplasia was established.

On follow up, the gingival hyperplasia was found to recur. Further treatment was deferred until patient completed growth. The patient was adviced to follow up regularly.


   Discussion Top


Rowe [8] described the criteria for true hemiqhypertrophy. According to him hemifacial hypertrophy is an unusual condition which produces facial asymmetry by a marked unilateral localized over growth of all the tissues in the affected area, i.e., facial soft tissues, bone and teeth. The unilateral enlargement of the viscerocranium is bounded by frontal bone superiorly (not including eye), inferiorly by the border of mandible medially by midline of the face and laterally by ear, pinna being included within hypertrophic area. The disorder occurs more commonly in females (ratio 3:2). The asymmetry is almost always evident at birth and the enlarged side generally grows at a rate proportional to but slightly faster than normal side. The disproportionate growth rate is maintained until the time of skeletal maturation and when growth of both sides ceases, results in an asymmetry existing throughout life.

Associated features reported are the size of abnormalities of anatomical structures, advanced physiologic development, anatomical, and functional defects traceable to embryonic origin and a multitude of non-specific associated anomalies. [9],[10] The various systems affected by this disorder are integumentary, musculoskeletal, cardiovascular, neurological genitourinary, endocrine, and respiratory systems. Multi-system involvement has resulted in etiological heterogenicity. No single theory explains the etiology adequately. Factors that can be implicated are heredity, chromosomal abnormalities, atypical forms of twinning, altered intrauterine environment, endocrine dysfunctions, anatomical, and functional anomalies of vascular/lymphatic systems and disturbances of the central nervous system. [9],[10]

The variation and complexity of reported cases of facial hemihypertrophy has documentation of a range of orofacial findings. Involvement of orofacial structures is related to asymmetric morphogenesis of teeth, bone and soft tissues. Dentition abnormalities with respect to crown size, root size and shape, rate of development, and number of teeth are seen. [8],[11] Tooth size enlargement is random with the frequency of involvement more in cuspids followed by premolars and first molars and least occurring in incisors, second molars and third molars. [12],[13] Usually enlargement does not exceed 50% of normal size. Primary dentition is seldom affected. Root size and shape may be proportionally enlarged, shortened, or unusually premature development may occur. Precocious eruption of permanent teeth by up to 4-5 years is usually seen. [14]

Skeletal findings may be in the form of asymmetric growth of frontal bone, maxilla, palate, mandible, or condyles. Abnormal occlusal relationships such as midline deviations, unequal occlusal plane levels contralaterally, open bite, and widely spaced teeth on involved side have been reported. [1],[8],[10],[11],[14]

Soft tissues abnormalities include thickened and enlarged anatomical tissues on involved side with sharply demarcated midline. Tongue on involved side may be thickened and distorted with grossly hypertrophic fungiform papillae. Sensory disturbances and lipoma-like excrescences of buccal mucosa have also been reported. [1],[8],[9],[10],[11],[13],[14] The importance of a thorough diagnostic evaluation in pediatric dentistry cannot be overstated. However, the timing and sequence of treatment in such disorders is effective with a multi-disciplinary team approach rather than restricting the child patient to a single specialist.

 
   References Top

1.Ringrose RE, Jabbour JT, Keele DK. Hemihypertrophy. Pediatrics 1965;36:434-48.  Back to cited text no. 1  [PUBMED]  
2.Farkus LG, James JS. Anthropometry of the face in lateral facial dysplasia: The unilateral form. Cleft Palate J 1977;14:193-9.  Back to cited text no. 2    
3.Pollock RA, Newman MH, Burdi AR, Condit DP. Congenital hemifacial hyperplasia: An embryonic hypothesis and case report. Cleft Palate J 1985;22:173-84.  Back to cited text no. 3  [PUBMED]  
4.Meckel JF. Ueber die Seitliche Asymmetrie im Tierischen Korper. Anatomische physiologische Beobachtungen und Untersuchungen Halle. Renger, 1822 cited by Halperin; 1931. p. 147.  Back to cited text no. 4    
5.Gessel A. Hemihypertrophy and twinning. Am J Med Sci 1927;173:542.  Back to cited text no. 5    
6.Ward J, Lerner HH. A review of the subject of congenital hemihypertrophy and a complete case report. J Pediat 1947;31:403.  Back to cited text no. 6    
7.Tommasi AF, Jitomirski F. Crossed congenital hemifacial hyperplasia. Oral Surg Oral Med Oral Pathol 1989;67:190-2.  Back to cited text no. 7  [PUBMED]  
8.Rowe NH. Hemifacial hypertrophy: Review of literature and addition of four cases. Oral Surg Oral Med Oral Path 1962;15:572-87.  Back to cited text no. 8  [PUBMED]  
9.Fraumeni JF Jr, Geiser CF, Manning MD. Wilms' tumor and congenital hemihypertrophy: report of five new cases and review of literature. Pediatrics 1967;40:886-99.  Back to cited text no. 9  [PUBMED]  
10.Rudolph CE, Norvold RW. Congenital partial hemihypertrophy involving marked malocclusion. J Dent Res 1944;23:133.  Back to cited text no. 10    
11.Gorlin RJ, Meskin LH. Congenital hemihypertrophy. J Pediatr 1962;61:870-9.  Back to cited text no. 11    
12.Burke PH. True hemihypertrophy of the face. Br Dent J 1951;91:213-5.  Back to cited text no. 12    
13.Hanley FJ, Floyd CE, Parker D. Congenital partial hypertrophy of the face: Report of three cases. J Oral Surg 1968;26:136-41.  Back to cited text no. 13    
14.Loh HS. Congenital hemifacial hypertrophy. Br Dent J 1982;153:111-2.  Back to cited text no. 14    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]
 
 
    Tables

  [Table - 1]



 

Top
Print this article  Email this article
Previous article Next article

    

 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (146 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed7119    
    Printed154    
    Emailed1    
    PDF Downloaded379    
    Comments [Add]    

Recommend this journal


Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 
  2005 - Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow 
Online since 1st May '05