PMC5554634_01
a) facial features of the patient; b) intra-oral findings showing anterior open bite; c) shallow buccal vestibule; d) high arched palate
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On facial examination mild frontal bossing, orbital hypertelorism, and incompetent lips were noted (Figure 1 a). Intraoral examination showed malaligned teeth with anterior open bite (Figure 1 b). There buccal vestibule was shallow with high arched palatal vault ( Figure 1 c, d).
a) asymmetrical scapular position (Sprengel's deformity); (b,c) palmar pitting
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General physical examination showed mild imbalanced in the scapular position due to Sprengel s deformity (Figure 2 a). Examination of skin on palmar surface showed punctiform brownish black depression on both hands suggestive of palmar pitting (Figure 2 b, c).
a) orthopantomogram showing multiple jaw cysts; b) posterio-anterior and lateral view of the skull showing calcification of falx cerebri; c) bridging of sella turcia
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Orthopantomogram of the patient revealed multiple radiolucent lesions involving both the jaw bones (Figure 3 a). Posterio-anterior and lateral view of the skull taken to study skeletal discrepancies showed calcification of falx cerebri (Figure 3 b) and bridging of sella turcia (Figure 3 c).
(a,b,c,d) transverse slides of CT, Formatted 3 dimensional images; e) showing multiple jaw cyst
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Multiple similar lesions were noted in ramus of left mandible, alveolar process of maxilla on right side causing thinning of bone and abutting the ipsilateral maxillary sinus and right posterior maxilla extending into the maxillary sinus and showing impacted molar teeth within it (Figure 4).
a) CT image showing multiple calcifications along the falx cerebrii; b) tentorium cerebelli; (c,d) linear measurements showing increased inter-canthal, orbit globe distance; e) narrowing of sella turcica
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Incidentally multiple calcifications along the falx cerebrii and tentorium cerebelli were seen (Figure 5 a, b). Linear measurements on scan showed an increased in inter-canthal distance suggestive of hypertelorism and narrowing of sella turcica (Figure 5 c, d, e).
photomicrograph showing cyst lining (H & E stain; 10 X magnifications)
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Histopathological study of the specimen showed presence of six to eight cell layers thick parakeratinized stratified squamous cell epithelium suggestive of keratocystic odontogenic tumor (KCOT) (Figure 6).
a) surgical removal of multiple KCOTs of jaw; enucleation of lesion; b) the bone cavity treated with carnoy's solution; c) lesions of right maxilla; d: enucleated lesion
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Enucleation of the jaw cysts along with chemical cauterization with carnoy's solution was performed under general anesthesia (Figure 7).