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Clinical and Imaging Findings of True Hemifacial Hyperplasia

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Clinical and Imaging Findings of True Hemifacial Hyperplasia Hindawi Publishing Corporation Case Reports in Dentistry Volume 2013, Article ID 152528, 7 pages http://dx.doi.org/10.1155/2013/152528 Case Report Clinical and Imaging Findings of True Hemifacial Hyperplasia Bansari A. Bhuta,1 Archana Yadav,1 Rajiv S. Desai,1 Shivani P. Bansal,1 Vipul V. Chemburkar,2 and Prashant V. Dev2 1 Department of Oral Pathology, Nair Hospital Dental College, Dr. A. L. Nair Road, Mumbai, Maharashtra 400 008, India 2 Department of Radiology, Topiwala National Medical College & B. Y. L. Nair Hospital, Mumbai 400008, India Correspondence should be addressed to Rajiv S. Desai; [email protected] Received 15 July 2013; Accepted 24 August 2013 Academic Editors: M. Darling, A. C. B. Delbem, G. Spagnuolo, and K. H. Zawawi Copyright © 2013 Bansari A. Bhuta et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Congenital hemifacial hyperplasia is a rare developmental disorder of unknown etiology, characterized by a marked unilateral facial asymmetry. It involves the hard (bones and teeth) and soft tissues of the face. We report an interesting case of true hemifacial hyperplasia in a 25-year-old male highlighting the clinical and computed tomography imaging findings. 1. Introduction We report an interesting case of true hemifacial hyper- plasia(THFH)ina25-year-oldmale,discussingclinical Congenital hemihyperplasia is a rare developmental disorder features, imaging findings, and differential diagnosis in detail characterized by unilateral overgrowth of one or more body to supplement the current literature. parts resulting in marked asymmetry. This phenomenon was first described by Meckel [1]in1822andfirstreportedby 2. Case Report Kottmeier and Wagner [2]in1839.Rowe[3]in1962classified hemihypertrophy into (1) complex hemihypertrophy, involv- A 25-year-old male reported to our department for opinion ing the entire half of the body, (2) simple hemihypertrophy regarding a massive, asymptomatic enlargement of the right affecting one or both limbs, and (3) hemifacial hypertrophy. half of the face since childhood (Figure 1). The asymmetry Depending on involvement of soft tissues, teeth, and bones, hadincreasedwithageandceasedtogrowafter18yearsof he further classified hemifacial hypertrophy into (a) true age. Family history was unavailable since he was an orphan. hemifacial hypertrophy and (b) partial hemifacial hypertro- Medical examination did not reveal any other health issues. phy. True hemifacial hypertrophy exhibits unilateral enlarge- The patient had deferred treatment until now due to the lack mentofalltissues,teeth,bones,andsofttissues,characterized of financial resources. Extraoral examination revealed a mas- by viscerocranial enlargement, bounded by frontal bone siveanddiffuseenlargementoftherightsideoftheface.The superiorly (sparing the eye), inferior border of the mandible enlargement extended from the midline to the preauricular inferiorly, midline medially, and ear including the pinna region, superiorly to the frontal bone and inferiorly to the laterally. In partial hemifacial hypertrophy not all structures inferior border of mandible. The nose and chin were deviated are enlarged to the same degree or limited to one structure. towards the left side of the face, with the facial midline We prefer to use the term congenital hemifacial hyperplasia describing a gentle arc from nasion to gnathion instead of the although it is usually referred to as hemifacial hypertrophy. usual vertical straight line. The right corner of the mouth was The term hyperplasia is more precise histologically, as all droopedandlipswerelargerontherightside.Theskinofthe tissues show an increase in the number of cells rather than involved right side of the face was coarser than the unaffected an increase in cell size [4]. The asymmetry usually remains left side. The right pinna was larger than that of the left side. constant with the end of adolescence, and as the skeletal There was monstrous enlargement of the soft tissue over the maturation occurs the condition stabilizes thereafter. zygoma, the infraorbital region and the symphyseal region. 2 Case Reports in Dentistry Figure 1: Unilateral enlargement of right side of the face. Note the Figure 2: Occlusal view of the maxillary and mandibular dental asymmetry of the forehead, cheeks, nose, lips, chin, and the closed casts showing macrodontia of the right side, midline shift to the left, eye. along with granular surface of the right palatal mucosa. Theenlargedsofttissuemassontherightsideoftheface caused closure of the right eye, compromising the patient’s vision. There appeared to be an excessive increase in size of the right side of the mandible, maxilla, zygoma, and frontal bone, as compared to the contralateral side. On palpation a nontender, soft tissue swelling over the right parietal bone was noticed. No temporomandibular joint disorder or dys- function was detected. No discrepancy in range of mandibu- lar motion was noted. Intraoral examination disclosed an obvious alveolar enlargement of the right maxillary and mandibular quadrants as compared to the left quadrants (Figure 2). The surface of the gingival tissue and palate on the right side was granular in appearance. Right half of the tongue showed an obvious enlargement to the midline with polypoid excrescences rep- resenting enlargement of the fungiform papillae (Figure 3). The right buccal mucosa was thickened and hung in pendu- lous folds (Figure 4). A distinct tooth size discrepancy was Figure 3: Intraoral photograph showing enlargement of right side observed between right and left side. Detailed coronal dimen- of the tongue with enlarged papillae. sions of the teeth were measured on the casts with vernier calipers, which revealed major variations in size between the teeth of the affected side compared to the uninvolved side. Right permanent maxillary canine, first premolar, second incisor, canine, first premolar, and second premolar demon- premolar, first molar, and second molar were larger cervicoin- strated 337%, 150%, 116%, and 57.14% increase in volume, cisally, mesiodistally, and labiolingually than those of the left respectively, than their contralateral counterparts. The max- side (Table 1). Similarly, right permanent mandibular lateral illary and mandibular midline was shifted to the left. A gen- incisor, canine, first premolar, and second premolar were eralised crossbite was present due to a prognathic mandible larger cervicoincisally, mesiodistally, and labiolingually than (Figure 5). The occlusal plane on the right side was canted those of the left sideTable ( 2). The above mentioned right downwards. maxillary and mandibular teeth were considerably enlarged The panoramic radiograph showed enlarged right body of than their contralateral counterparts. The right permanent the mandible with the widening of the right inferior alveolar maxillary canine, first premolar, second premolar, first molar, canal (Figure 6).PASkullshowedenlargementofrighthalfof andsecondmolardemonstrated224%,100%,63%,110%,and mandible,maxilla,andzygoma(Figure 7). Soft tissue enlarge- 75% increase in volume, respectively, than their contralateral ment was seen on the right side of the face and also encircling counterparts, while the right permanent mandibular lateral the symphysis. Case Reports in Dentistry 3 Table 1: Comparison of crown sizes of the maxillary teeth. Maxillary arch Dimension (mm) Canine 1st premolar 2nd premolar 1st molar 2nd molar LRL R L R LRL R Cervicoincisal 11 17 9 12 9 10 9 12 9 11 Mesiodistal 7 11 8 9 7 8 11 13 10 13 Labiolingual 6 8 6 8 7 9 9 12 10 11 Total surface volume (cu. mm) 462 1496 432 864 441 720 891 1872 900 1573 Enlarged by 224% 100% 63% 110% 75% L: left; R: right. Table2:Comparisonofcrownsizesofthemandibularteeth. Mandibular arch Dimension (mm) Lateral incisor Canine 1st premolar 2nd premolar LRLRLRLR Cervicoincisal 9 15 14 17 10 13 8 11 Mesiodistal 6 9 8 11 7 10 8 8 Labiolingual 4 7 6 9 6 7 7 8 Total surface volume (cu. mm) 216 945 672 1683 420 910 448 704 Enlarged by 337% 150% 116% 57.14% L: left; R: right. Figure 4: Intraoral photograph showing velvety buccal mucosa Figure 5: Intraoral photograph showing major variability between hanging in pendulous folds and granular gingival surface along with crown sizes of the teeth on the right and the left side with generalised macrodontic permanent right lateral incisor and canine. crossbite relationship. Computed tomographic (CT) scan of face revealed side (Figure 10). An intracranial lipoma in the quadrigeminal enlargement of the bony structures, including right half of cistern of the right side was also seen (Figure 11). Bony orbit maxilla, mandible, condyle, zygoma, bony walls of external on right side was deformed; however, globe, intra-, and extra- auditory canal, and pterygoid bone (Figures 8 and 9). There orbital structures were normal. Deformation and deviation was bony overgrowth of the glenoid fossa with irregularity of the nasal bone and chin were seen towards the left side of the articular surface. The right condyle was found to due to enlargement of overlying soft tissues. Prominent be irregular and flattened; however, the temporomandibular vessels and few nodular serpiginous areas were seen within joint space was maintained. The right external auditory canal right parotid gland which was enlarged with heterogenous appeared stenosed due to bony overgrowth. The right frontal appearance
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