PMC4034637_01
(A) Frontal and lateral view face showing the frontal bossing, midface hypoplasia and a relatively large mandible, shallow orbits, hypertelorism, exophthalmos, short and incompetent upper lip, depressed nasal bridge, (B) lateral view of the face
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F1
Other facial features included short and incompetent upper lip, depressed nasal bridge and low-set ears but without any hearing loss (Figs 1A and B).
PMC4034637_02
Pseudocleft in the palate
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F2
On intraoral examination, V-shaped maxillary arch with high-arched palate and bilateral palatal swellings which was mimicking a pseudocleft (Fig. 2), retruded maxilla with a relatively large mandible were found.
Intraoral view showing crowding of upper and lower teeth, reverse overjet with posterior crossbite and anterior open bite
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F3
His oral hygiene was poor with crowding of upper and lower teeth, reverse over-jet with posterior crossbite and anterior open bite (Fig. 3), tongue tie (Fig. 4) and decayed teeth were present.
AP and lateral view of the skull-demonstrating maxillary retrusion, relative mandibular prognathism and 'hammered silver' (beaten metal/copper beaten) appearance
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F5
The skull radiographs revealed the 'scaphocephalic' skull shape, hypoplastic maxilla and zygoma with shallow orbits (Figs 5A and B).
(A to C) Three-dimensional CT scan of skull revealing surgically opened coronal sutures, frontal bossing, shallow orbits, hypoplastic maxilla and zygoma; (D and E) obliteration of sagittal and lambdoid sutures
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F6
Three-dimensional computed tomographic (CT) scans of the skull showed fused sagittal and lambdoid sutures and surgically opened coronal sutures (Figs 6A to E), moderate degree of hydrocephalus with diffuse indentation of inner table of skull (Figs 7A and B).
Moderate degree of hydrocephalus, diffuse indentation of inner table of skull seen in the CT scan
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F7
Three-dimensional computed tomographic (CT) scans of the skull showed fused sagittal and lambdoid sutures and surgically opened coronal sutures (Figs 6A to E), moderate degree of hydrocephalus with diffuse indentation of inner table of skull (Figs 7A and B).