Intraosseous haemangiomas (IOHs) are benign vascular bone tumours that account for 1% of all primary bone tumours. They are most frequently seen in the vertebrae and skull, and are rarely found in long bones. Herein, we present an uncommon case of a 25-year-old woman with a solitary IOH that occupied the left femoral neck. We describe the clinical, radiological and histological details of the case, as well as the three-year outcome of the surgical treatment, which successfully preserved the femoral head. We also conducted a review of the literature on this uncommon entity.
Adult ; Angiography ; Diagnosis, Differential ; Female ; Femur Neck ; blood supply ; Humans ; Magnetic Resonance Imaging ; Skull ; abnormalities ; surgery ; Spine ; abnormalities ; surgery ; Tomography, X-Ray Computed ; Vascular Malformations ; diagnosis ; surgery ; Vascular Surgical Procedures ; methods

Adult ; Dandy-Walker Syndrome ; diagnosis ; surgery ; Female ; Humans ; Neurofibromatosis 1 ; diagnosis ; surgery ; Skull ; abnormalities

A 46-year-old female patient presented to our hospital with history of pharyngeal discomfort for 6 months. Physical examination showed that she had facial asymmetry, loss of the left nasolabial fold, rightward de- viation of uvula, swelling of the left soft palate. Magnetic resonance imaging revealed a bigger neoplasm in left pa- rapharyngeal space which invaded into left lateral skull base. The left internal carotid artery, vein and the styloid process was jostled backward. The primary clinical diagnosis is pleomorphic adenoma of the parapharyngeal space.
Adenoma, Pleomorphic ; surgery ; Carotid Artery, Internal ; Endoscopes ; Endoscopy ; Face ; abnormalities ; Facial Asymmetry ; congenital ; Female ; Humans ; Hyperplasia ; Magnetic Resonance Imaging ; Middle Aged ; Nasal Cavity ; Paranasal Sinuses ; Pharynx ; Skull Base Neoplasms ; surgery

PURPOSE: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. METHODS: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. RESULTS: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. CONCLUSION: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.
Atrophy ; Brain ; Cerebrum ; Congenital Abnormalities ; Humans ; Hypertrophy ; Magnetic Resonance Imaging ; Middle Aged ; Nasal Bone ; Paranasal Sinuses ; Rare Diseases ; Skull ; Surgery, Plastic

PURPOSE: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. METHODS: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. RESULTS: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. CONCLUSION: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.
Atrophy ; Brain ; Cerebrum ; Congenital Abnormalities ; Humans ; Hypertrophy ; Magnetic Resonance Imaging ; Middle Aged ; Nasal Bone ; Paranasal Sinuses ; Rare Diseases ; Skull ; Surgery, Plastic

The Muenke syndrome (MS) is characterized by unicoronal or bicoronal craniosynostosis, midfacial hypoplasia, ocular hypertelorism, and a variety of minor abnormalities associated with a mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. The birth prevalence is approximately one in 10,000 live births, accounting for 8-10% of patients with coronal synostosis. Although MS is a relatively common diagnosis in patients with craniosynostosis syndromes, with autosomal dominant inheritance, there has been no report of MS, in an affected Korean family with typical cephalo-facial morphology that has been confirmed by molecular studies. Here, we report a familial case of MS in a female patient with a Pro250Arg mutation in exon 7 (IgII-IGIII linker domain) of the FGFR3 gene. This patient had mild midfacial hypoplasia, hypertelorism, downslanting palpebral fissures, a beak shaped nose, plagio-brachycephaly, and mild neurodevelopmental delay. The same mutation was confirmed in the patient's mother, two of the mother's sisters and the maternal grandfather. The severity of the cephalo-facial anomalies was variable among these family members.
Adult ; Asian Continental Ancestry Group/*genetics ; Child, Preschool ; Craniosynostoses/*genetics/surgery ; DNA Mutational Analysis ; Female ; Humans ; Hypertelorism/genetics ; Korea ; Male ; *Mutation ; Pedigree ; Phenotype ; Receptor, Fibroblast Growth Factor, Type 3/*genetics ; Skull/*abnormalities/surgery ; Syndrome ; Treatment Outcome

OBJECTIVETo investigate the technique of skull reconstruction for the congenital craniosynostosis.

METHODSMany technique of skull plasty were adopted to correct the congenital craniosynostosis. We advanced fronto-orbital, reversal change of the frontal bone to correct the brachycephalic, hydrocephaly acrocephaly, which were supported by parietal bone. Plum-plasty of the calvaria was performed for sagittal synostosis; Fronto-orbital plasty correct the trigonocephaly; for single coronal synostosis, which result to frontal plagiocephaly,we reversed both frontal sides, advanced orbital strip and tilted the invalid side . The post plagiocephaly because of single Lambdoid synostosis, we performed of plum-plasty, reversal change of total occipital. Fronota-facial advancement cured the brachycephalic complicating with Apert or Crouzon which caused by coronal synostosis and cranial basal suture synostosis.

RESULTS37 cases were recovery without complication. The figures were improved.

CONCLUSIONSBig calvarial flap plasty is an optimal technique to correct the congenital craniosynostosis. But it is not substituted by bone lengthening and minimally invasive technique.

Child, Preschool ; Cranial Sutures ; abnormalities ; Craniosynostoses ; surgery ; Female ; Humans ; Infant ; Male ; Reconstructive Surgical Procedures ; methods ; Skull ; surgery

OBJECTIVETo probe the possibility of trans-sutural distraction osteogenesis for correction of children midfacial hypoplasia.

METHODSThe trans-sutural distraction system of mid-facial skeleton consisted of the bone-borne traction hooks of titanium, the face-bow, and the elastic loops. Nine children with midfacial hypoplasia were treated at their 6 - 12 years of age. No osteotomy was made in them. Bone holes were drilled with a dental bur at each side of the lateral-inferior rim of the aperture, or at the anterior part of the hard palate. The traction device was hatched to the holes through the nostrils. Protraction began 3 days postoperatively, with the forces adjusted dependently upon the rate of progress. When the skeleton reached to the planed position, it was retained with a minor force for 8 weeks.

RESULTSThe mid-facial skeleton of the nine children showed a balanced advancement. Their facial profile and cross-bite were corrected satisfactory.

CONCLUSIONSPatients with severe mid-facial hypoplasia could be corrected ideally by the new technique, with minor trauma, easy manipulation. The design of protraction system was reasonable.

Child ; Humans ; Maxillofacial Abnormalities ; surgery ; Oral Surgical Procedures ; methods ; Orbit ; abnormalities ; surgery ; Osteogenesis, Distraction ; methods ; Skull ; abnormalities ; surgery ; Treatment Outcome

OBJECTIVETo explore the method to obtain good aesthetic and functional results in surgical management of craniomaxillofacial fibrous dysplasia and correct the grotesque deformity.

METHODSAccording to the type of the lesions, different excision and reconstruction methods were used.

RESULTS19 cases (4 monostotic cases and 15 polyostotic cases) were surgically treated. The period of follow-up range from 9 months to 5 years, all patients obtained satisfactory aesthetic and functional results. No relapse happened during follow up.

CONCLUSIONSBased on modern craniomaxillofacial techniques and computer aided design, extensive radical excision and craniomaxillofacial skeleton reconstruction could be safely accomplished, and the better results were obtained, both aestheticly and functionally.

Adolescent ; Adult ; Bone Transplantation ; Child ; Computer-Aided Design ; Craniofacial Abnormalities ; surgery ; Facial Bones ; Female ; Fibrous Dysplasia of Bone ; surgery ; Follow-Up Studies ; Humans ; Male ; Reconstructive Surgical Procedures ; Skull ; Surgical Flaps ; Young Adult

surgery and reconstruction, and esthetic surgery. Complications were such as injury to frontal branch of the facial nerve, motor nerve paralysis, hematoma under flap, trismus, ptosis, epiphora, infection and anterior temporal depression. Medpor(R) is made up of dense polyethylene connected in porous structures. It is easily shapable without collapsing the pores due to it's hardness and tissue growth takes place at the porosities. Based on these advantages, Medpor(R) has been used in augmentation and restoration in craniofacial defect. A temporal depression after the coronal approach for treatment of Le Fort III fracture was successfully reconstruction with Medpor(R) and we report this case with review of literature.]]>
Congenital Abnormalities ; Depression ; Facial Nerve ; Hardness ; Hematoma ; Lacrimal Apparatus Diseases ; Paralysis ; Polyethylene ; Porosity ; Skull ; Surgery, Plastic ; Trismus