• Journal of Korean Foot and Ankle Society
• /
• v.14 no.1
• /
• pp.105-107
• /
• 2010

The scanty literature on distal tibiofibular synostosis includes descriptions of relatively few specific complaints. Here we report a case of a 24-year-old young soldier who sustained a left ankle lateral malleolar fracture about 6 months ago and was initially treated by the open reduction and internal fixation with plate & screw. But 6 months later, he suffered from an vague ankle pain, each time the symptoms occurred right after an active ambulation, ankle dorsiflexion, especially when he had exercised aggressively. The radiographs revealed that there was a mature distal tibiofibular synostosis. We treated the patient with surgical excision of synostosis. Post-operative condition was satisfied to all concern and the result was found to be excellent during one-year follow-up.

• Archives of Craniofacial Surgery
• /
• v.21 no.2
• /
• pp.106-108
• /
• 2020

Premature fusion of one or other of the minor sutures can subtly influence the shape of the human skull. Although infrequently reported or not clinically recognized, it can such contribute to a variety of craniofacial dysmorphisms. We herein report a case of late presenting, isolated bilateral synostosis of the squamosal suture dysmorphologies whose presentation mimics aspects of sagittal synostosis.

• Journal of Korean Neurosurgical Society
• /
• v.65 no.1
• /
• pp.107-113
• /
• 2022

Objective : To describe the surgical management and postoperative outcomes in infants with metopic synostosis. Methods : We conducted a 5 years retrospective chart review of patients who underwent surgical correction of metopic synostosis at two university hospitals in Egypt during the period between June 2014 and June 2019. The study is conducted to 18 children. The type of surgical procedures and postoperative outcomes were assessed in all patients. Results : Five cases (27.8%) underwent endoscopic-assisted suturectomy, 10 cases (55.6%) underwent craniofacial reconstruction, and three cases (16.6%) underwent open burring of the metopic ridge. Fifteen patients underwent one surgery and three patients (16.6%) who need second operation. Ten patients (55.6%) had class I Whitaker classification. Conclusion : Regardless of type of surgery, the outcomes of surgical correction of metopic synostosis are excellent with only a few patients require revision or develop major complications.

• Archives of Plastic Surgery
• /
• v.38 no.1
• /
• pp.77-80
• /
• 2011

Purpose: We report a patient with DiGeorge syndrome who was later diagnosed as mild metopic synostosis and received anterior 2/3 calvarial remodeling. Methods: A 16-month-old boy, who underwent palatoplasty for cleft palate at Chungnam National University Hospital when he was 12 months old of age, visited St. Mary's Hospital for known DiGeorge syndrome with craniosynostosis. He had growth retardation and was also diagnosed with hydronephrosis and thymic agenesis. His chromosomal study showed microdeletion of 22q11.2. On physical examination, there were parieto-occipital protrusion and bifrontotemporal narrowing. The facial bone computed tomography showed premature closure of metopic suture, orbital harlequin sign and decreased anterior cranial volume. The interorbital distance was decreased (17 mm) and the cephalic index was 93%. Results: After the correction of metopic synostosis by anterior 2/3 calvarial remodeling, the anterior cranial volume expanded with increased interorbital distance and decreased cephalic index. Fever and pancytopenia were noted at 1 month after the operation, and he was diagnosed as hemophagocytic lymphohistiocytosis by bone marrow study. He however, recovered after pediatric treatment. There was no other complication during the 12 month follow up period. Conclusion: This case presents with a rare combination of DiGeorge syndrome and metopic synostosis. When a child is diagnosed with DiGeorge syndrome soon after the birth, clinicians should keep in mind the possibility of an accompanying craniosynostosis. Other possible comorbidities should also be evaluated before the correction of craniosynostosis in patients as DiGeorge syndrome. In addition, postoperative management requires a thorough follow up by a multidisciplinary team of plastic surgeons, neurosurgeons, ophthalmologists and pediatricians.

• Archives of Craniofacial Surgery
• /
• v.9 no.2
• /
• pp.85-89
• /
• 2008

Purpose: Unicoronal synostosis is the craniofacial anomaly caused by premature fusion of unilateral coronal suture. Ipsilateral flattening of the frontal and parietal bones, temporal retrusion with elevation and recession of the supraorbital rim are main clinical features. Compensatory contralateral frontal bossing and deviation of the nasal root and/or chin can also occur. There is a controversy about techniques for surgical correction, however, bilateral approach technique is more effective for correction of deformity. Methods: A 4-year-old patient with unicoronal synostosis had undergone unilateral suturectomy at 28-month-old but fronto-facial deformity had remained and aggravated as she grew older. She had both fronto-facial and endocranial asymmetry. We performed coronal cranial approach and fully exposed affected cranium including supraorbital rim. Anterior 2/3 calvarial reconstruction with bilateral frontal bone osteotomy and fronto-orbital bandeau advancement was performed. Results: Fronto-facial symmetry including fronto-orbital contour, nasal devation was improved. Endocranial twisting was also improved from $158^{\circ}$ to $162^{\circ}$ in CSO(crista gallisella turcica-opisthion) degree. There was no postoperative complications and no need for revision, and facial asymmetry improved at the period of 2 years of follow-up. Conclusion: Bilateral approach with fronto-orbital bandeau remodeling in surgery of unicoronal synostosis looked superior to unilateral approach in achieving better symmetry and preventing recurrence of asymmetry. Remodeling surgery should be tried in patients even at an older age to correct fronto-facial asymmetry.

• Journal of Genetic Medicine
• /
• v.13 no.1
• /
• pp.31-35
• /
• 2016

Antley-Bixler syndrome (ABS) is a rare form of syndromic craniosynostosis with additional systemic synostosis, including radiohumeral or radioulnar synostosis. Another characteristic feature of ABS is mid-facial hypoplasia that leads to airway narrowing after birth. ABS is associated with mutations in the FGFR2 and POR genes. Patients with POR mutations present with either skeletal manifestations or congenital adrenal hyperplasia with ambiguous genitalia. We report here two cases of ABS caused by mutations in FGFR2 and POR. Although the patients had craniosynostosis and radiohumeral synostosis in common and cranioplasty was performed in both cases, the male with POR mutations showed an elevated level of $17{\alpha}$-hydroxyprogesterone during newborn screening and was diagnosed with congenital adrenal hyperplasia by adrenocorticotropic hormone stimulation. This patient has been treated with hydrocortisone and fludrocortisone. He had no ambiguous genitalia but had bilateral cryptorchidism. On the other hand, the female with the FGFR2 mutation showed severe clinical manifestations: upper airway narrowing leading to tracheostomy, kyphosis of the cervical spine, and coccyx deformity. ABS shows locus heterogeneity, and mutations in two different genes can cause similar craniofacial and skeletal phenotypes. Because the long-term outcomes and inheritance patterns of the disease differ markedly, depending on the causative mutation, early molecular genetic testing is helpful.

• Proceedings of The Korean Cleft Lip And Palate Association
• /
• 1997.06a
• /
• pp.51-51
• /
• 1997

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.17 no.2
• /
• pp.180-185
• /
• 1995

TMJ ankylosis is classified with true and false type. A true ankylosis is defined as any condition that produces fibrous or bony adhesions between the articular surfaces of the temporomandibular joint. The main causes of true ankylosis are trauma or infection. A false ankylosis results from pathologic conditions outside the joint that limit mobility of the mandible such as myogenic disorders, coronoid impingement or rare direct bony fusion between maxilla and mandible. The treatment of choice of TMJ ankylosis is surgical intervention. We experienced the male patient with complete mouth opening limitation since 45 years before. This patient has true TMJ ankylosis and rare bony synostosis between maxilla and mandible in the right posterior region. We performed surgical intervention and had a favorable result.

• Archives of Reconstructive Microsurgery
• /
• v.12 no.2
• /
• pp.93-98
• /
• 2003

Purpose : The purpose of this study is to investigate an occurrence of valgus deformity of ankle joint after vascularized fibular graft in children. Materials and Methods : Four children under 15 years who were surgically treated with vascularized fibular graft were studied. The age of the patients was from 4 years to 13 years, the follow-up period was from 24 months to 108 months. The causes of vascularized fibular graft were open fracture (1 case), congenital psuedarthrosis (2 cases), hypoplastic ulna (1 case). The tibiofibular synostosis was done in 3 cases and not in 1 case. We measured the tibiotalar angle and bimalleolar angle at immediately postoperative and final radiography, and checked ankle motion, pain, and instability of ankle joint. Results : The A-P mortise angle was not different between initial and final radiography in all cases. The intermalleolar angle increased in all cases at the final radiography. There were no pain, instability and limitation of ankle motion. Conclusion: We consider the tibiofibular synostosis can prevent from ankle valgus deformity after vascularized fibular graft in children.

• Journal of Korean Neurosurgical Society
• /
• v.59 no.3
• /
• pp.204-213
• /
• 2016

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.