The Experts below are selected from a list of 315 Experts worldwide ranked by ideXlab platform
Hyun Tae Yook - One of the best experts on this subject based on the ideXlab platform.
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Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome
Maxillofacial Plastic and Reconstructive Surgery, 2018Co-Authors: Bong Kuen Cha, Dong Soon Choi, In San Jang, Hyun Tae Yook, Seung Youp Lee, Sang Shin Lee, Suk Keun LeeAbstract:Background A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Methods Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. Results The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Conclusion Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
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aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of apert syndrome
Maxillofacial plastic and reconstructive surgery, 2018Co-Authors: Dong Soon Choi, In San Jang, Hyun Tae YookAbstract:A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
Dong Soon Choi - One of the best experts on this subject based on the ideXlab platform.
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Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome
Maxillofacial Plastic and Reconstructive Surgery, 2018Co-Authors: Bong Kuen Cha, Dong Soon Choi, In San Jang, Hyun Tae Yook, Seung Youp Lee, Sang Shin Lee, Suk Keun LeeAbstract:Background A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Methods Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. Results The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Conclusion Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
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aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of apert syndrome
Maxillofacial plastic and reconstructive surgery, 2018Co-Authors: Dong Soon Choi, In San Jang, Hyun Tae YookAbstract:A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
Suk Keun Lee - One of the best experts on this subject based on the ideXlab platform.
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Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome
Maxillofacial Plastic and Reconstructive Surgery, 2018Co-Authors: Bong Kuen Cha, Dong Soon Choi, In San Jang, Hyun Tae Yook, Seung Youp Lee, Sang Shin Lee, Suk Keun LeeAbstract:Background A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Methods Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. Results The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Conclusion Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
Tomoki Kosho - One of the best experts on this subject based on the ideXlab platform.
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a new ehlers danlos syndrome with craniofacial characteristics multiple congenital contractures progressive joint and skin laxity and multisystem fragility related manifestations
American Journal of Medical Genetics Part A, 2010Co-Authors: Tomoki Kosho, Noriko Miyake, Atsushi Hatamochi, Jun Takahashi, Hiroyuki Kato, Teruyoshi Miyahara, Yasuhiko Igawa, Hiroshi Yasui, Tadao Ishida, Takashi KosudaAbstract:We previously described two unrelated patients showing characteristic facial and skeletal features, overlapping with the kyphoscoliosis type Ehlers–Danlos syndrome (EDS) but without lysyl hydroxylase deficiency [Kosho et al. (2005) Am J Med Genet Part A 138A:282–287]. After observations of them over time and encounter with four additional unrelated patients, we have concluded that they represent a new clinically recognizable type of EDS with distinct craniofacial characteristics, multiple congenital contractures, progressive joint and skin laxity, and multisystem fragility-related manifestations. The patients exhibited strikingly similar features according to their age: craniofacial, large fontanelle, hypertelorism, Short and downslanting palpebral fissures, blue sclerae, Short Nose with hypoplastic columella, low-set and rotated ears, high palate, long philtrum, thin vermilion of the upper lip, small mouth, and micro-retrognathia in infancy; slender and asymmetric face with protruding jaw from adolescence; skeletal, congenital contractures of fingers, wrists, and hips, and talipes equinovarus with anomalous insertions of flexor muscles; progressive joint laxity with recurrent dislocations; slender and/or cylindrical fingers and progressive talipes valgus and cavum or planus, with diaphyseal narrowing of phalanges, metacarpals, and metatarsals; pectus deformities; scoliosis or kyphoscoliosis with decreased physiological curvatures of thoracic spines and tall vertebrae; cutaneous, progressive hyperextensibility, bruisability, and fragility with atrophic scars; fine palmar creases in childhood to acrogeria-like prominent wrinkles in adulthood, recurrent subcutaneous infections with fistula formation; cardiovascular, cardiac valve abnormalities, recurrent large subcutaneous hematomas from childhood; gastrointestinal, constipation, diverticula perforation; respiratory, (hemo)pneumothorax; and ophthalmological, strabismus, glaucoma, refractive errors. © 2010 Wiley-Liss, Inc.
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a new ehlers danlos syndrome with craniofacial characteristics multiple congenital contractures progressive joint and skin laxity and multisystem fragility related manifestations
American Journal of Medical Genetics Part A, 2010Co-Authors: Tomoki Kosho, Noriko Miyake, Atsushi Hatamochi, Jun Takahashi, Hiroyuki Kato, Teruyoshi Miyahara, Yasuhiko Igawa, Hiroshi Yasui, Tadao Ishida, Kurahito OnoAbstract:We previously described two unrelated patients showing characteristic facial and skeletal features, overlapping with the kyphoscoliosis type Ehlers-Danlos syndrome (EDS) but without lysyl hydroxylase deficiency [Kosho et al. (2005) Am J Med Genet Part A 138A:282-287]. After observations of them over time and encounter with four additional unrelated patients, we have concluded that they represent a new clinically recognizable type of EDS with distinct craniofacial characteristics, multiple congenital contractures, progressive joint and skin laxity, and multisystem fragility-related manifestations. The patients exhibited strikingly similar features according to their age: craniofacial, large fontanelle, hypertelorism, Short and downslanting palpebral fissures, blue sclerae, Short Nose with hypoplastic columella, low-set and rotated ears, high palate, long philtrum, thin vermilion of the upper lip, small mouth, and micro-retrognathia in infancy; slender and asymmetric face with protruding jaw from adolescence; skeletal, congenital contractures of fingers, wrists, and hips, and talipes equinovarus with anomalous insertions of flexor muscles; progressive joint laxity with recurrent dislocations; slender and/or cylindrical fingers and progressive talipes valgus and cavum or planus, with diaphyseal narrowing of phalanges, metacarpals, and metatarsals; pectus deformities; scoliosis or kyphoscoliosis with decreased physiological curvatures of thoracic spines and tall vertebrae; cutaneous, progressive hyperextensibility, bruisability, and fragility with atrophic scars; fine palmar creases in childhood to acrogeria-like prominent wrinkles in adulthood, recurrent subcutaneous infections with fistula formation; cardiovascular, cardiac valve abnormalities, recurrent large subcutaneous hematomas from childhood; gastrointestinal, constipation, diverticula perforation; respiratory, (hemo)pneumothorax; and ophthalmological, strabismus, glaucoma, refractive errors.
In San Jang - One of the best experts on this subject based on the ideXlab platform.
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Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome
Maxillofacial Plastic and Reconstructive Surgery, 2018Co-Authors: Bong Kuen Cha, Dong Soon Choi, In San Jang, Hyun Tae Yook, Seung Youp Lee, Sang Shin Lee, Suk Keun LeeAbstract:Background A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Methods Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. Results The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Conclusion Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
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aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of apert syndrome
Maxillofacial plastic and reconstructive surgery, 2018Co-Authors: Dong Soon Choi, In San Jang, Hyun Tae YookAbstract:A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagNosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a Short Nose with bulbous tip. Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. The Apert syndrome patient had a Shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; Shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; Shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
