PMC8387074_01
Photomicrographs of the biopsied soft tissue lesion: A - stratified squamous para-keratinized epithelium overlying a fibro-cellular connective tissue stroma predominantly comprising of blood vessels, neural tissue, smooth muscle bundles and forming hyaline cartilage in deeper stroma (H&E, 10X); B - Masson trichrome stain used to differentiate the smooth muscle cells (stained pink) from dense collagen fibres (stained blue) (10X).
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The stroma consisted of loosely arranged collagen fibers, loosely arranged bundles of differentiated smooth muscle fibers, nerve fascicles, thick-walled blood vessels and ectopic cartilaginous tissue (Figure 1A). The Masson Trichrome special stain was used to delineate the different connective tissue components (Figure 1B).
Clinical examination: A - showing soft tissue swelling over the alveolar mucosa (arrow head), abnormal frenal attachment, midline diastema, median alveolar cleft, mesio-labial rotation of the right central incisor, pseudo cleft of the lower lip (yellow dotted line), milia (arrows); B - Philtrum pits (arrowhead), milia (arrows); C - Low set ears; D - Thin scanty hair.
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Extra-orally, micrognathia, pseudo-clefting of the lower lip, two midline pits in the labial philtrum and low set ears were evident (Figure 2A-C). The skin of patient was dry with thin scanty hair, crops of milia were noted on the nose, along the nasolabial folds and chin (Figure 2A, B, D).
Tomographic examination of maxilla showing median alveolar cleft. A - 3D reconstruction; B - Axial view.
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The CBCT (Figure 3A, B) and the OPG confirmed the presence of a median alveolar cleft of the maxilla, while the abdominal ultrasound was unremarkable.