Adenoid cystic carcinoma is a malignant tumor of the head and neck, this article reports a case of a large adenoid cystic carcinoma of the skull base, with the lesion involving the sphenoid sinus, sphenoid bone wings, pterygopalatine fossa, nfratemporal fossa, hard palate, and other structures. The treatment plan consisted of surgical excision, primary reconstrction of the surgical defect,and postoperative radiotherapy, resulting in a favorable prognosis for the patient.
Humans ; Carcinoma, Adenoid Cystic/surgery* ; Maxillary Sinus/surgery* ; Maxillary Sinus Neoplasms/surgery* ; Palate, Hard/surgery* ; Skull Base Neoplasms/surgery* ; Surgical Flaps

Objective:To compare the outcomes of simultaneous versus staged nasal bone reduction and septoplasty in the treatment of traumatic nasal bone fractures with associated septal fractures. Methods:Patients with traumatic nasal bone fractures, with or without septal fractures, were recruited from two hospitals and divided into three groups. Group A underwent simultaneous nasal bone reduction and septoplasty, Group B underwent staged nasal bone reduction and septoplasty, and Group C underwent nasal bone reduction only. Nasal appearance scores and nasal congestion Visual Analogue Scale （VAS） scores were measured preoperatively, at 2 weeks postoperatively, and at 3 months postoperatively. For Group B, scores were also recorded at 2 weeks and 3 months after the second-stage surgery. Differences were analyzed using statistical software. Results:Two weeks post-surgery, the nasal appearance scores significantly decreased in all three groups compared to preoperative scores （P< 0.01）, with no statistically significant differences between the groups （P= 0.43, 0.71, 0.58）. In Group A, the VAS score for nasal congestion decreased significantly following simultaneous surgery （P<0.01）. In Group B, there were no significant differences in nasal congestion VAS scores between pre-surgery and post-first-stage, nor between three months post-first-stage and two weeks post-first-stage （P= 0.61, 0.13）. However, the VAS scores significantly decreased after the second-stage surgery compared to pre-surgery, and three months post-second-stage surgery compared to three months post-first-stage surgery （P<0.01）. The VAS scores for nasal congestion at two weeks post-surgery in Group A were lower than those in Group B after the first-stage surgery （P<0.01）. The incidence rates of nasal adhesions post-surgery in Groups A, B, and C were 0%, 6.9%, and 4.3%, respectively. Conclusion:For traumatic nasal bone fractures with associated septal fractures, performing simultaneous nasal bone reduction and septoplasty under general anesthesia using endoscopy is more time-efficient compared to staged surgery, and it results in a lower occurrence rate of post-surgical nasal adhesions.
Humans ; Nasal Bone/surgery* ; Nasal Septum/injuries* ; Treatment Outcome ; Rhinoplasty/methods* ; Male ; Female ; Adult ; Skull Fractures/surgery* ; Fractures, Bone/surgery* ; Middle Aged ; Adolescent

Objective:To investigate the clinical diagnosis and treatment of IgG4-related disease（IgG4-RD） primarily involving the nasal cavity and skull base. Methods:A retrospective analysis was conducted on the clinical data of 8 patients with IgG4-RD primarily involving the nasal cavity and skull base who visited the Nasal and Skull Base Surgery Department at Xiangya Hospital from October 2017 to January 2024. The cohort comprised 4 males and 4 females, aged 8 to 69 years. Clinical data, laboratory examination results, imaging findings, histopathological results, and treatment plans were collected. The clinical manifestations, diagnosis, treatment and follow-up results of IgG4-RD primarily involving nasal cavity and skull base were summarized and previous literature were also reviewed. Results:The initial symptoms in the 8 patients included nasal congestion, headache, sensory function decline, and facial deformities. Three patients also had parotid and pulmonary involvement. Among the 8 patients, 4 underwent partial surgical resection combined with glucocorticoid therapy; 1 underwent partial surgical resection combined with glucocorticoid and immunosuppressant therapy; 1 received glucocorticoid therapy alone; and 2 received glucocorticoid combined with immunosuppressant therapy. Follow-up was conducted one month after treatment, lasting from 5 to 79 months. During the follow-up period, recurrence was observed in 1 patient treated with glucocorticoid combined with immunosuppressants and in 1 patient treated with glucocorticoid alone, while the other 6 patients achieved significant remission. Conclusion:The diagnosis of nasal cavity and skull base IgG4-RD requires the combination of histopathology, laboratory tests, and imaging results. Treatment primarily includes glucocorticoids or combined immunosuppressants. For patients with significant compression symptoms, sensory function impairment, or facial deformities, surgical resection is an important treatment option. Given the high risk of recurrence, early intervention, active treatment, and long-term follow-up are crucial.
Humans ; Male ; Skull Base/pathology* ; Female ; Middle Aged ; Retrospective Studies ; Aged ; Nasal Cavity/pathology* ; Adult ; Immunoglobulin G4-Related Disease/therapy* ; Immunoglobulin G ; Child ; Young Adult ; Adolescent

With the advancement of rigid endoscopic nasal surgery techniques, the indications for surgery have continued to expand, encompassing an increasing number of anatomical regions. As a surgical adjunct, imaging-guided system assists surgeons in identifying vital structures and mitigating surgical risks, and they are extensively employed in operations involving the nasal cavity, sinuses, and skull base. Nevertheless, existing imaging-guided system faces several unresolved challenges. With ongoing technological advancements and the integration of novel technologies, these systems are demonstrating renewed vigor.
Humans ; Skull Base/surgery* ; Endoscopy/methods* ; Surgery, Computer-Assisted/methods*

Extraskeletal Ewing's sarcoma（EES） in the head and neck is extremely rare, with non-specific clinical manifestations, high malignancy, easy recurrence and metastasis, and poor prognosis. This paper reports a case of EES of the parapharyngeal skull base presenting with secretory otitis media as the initial symptom.The treatment consisted of surgery, chemotherapy and radiotherapy. No further metastasis or recurrence was observed during the two years and six months follow-up. Now we reviewed the relevant literatures and summarized the experience of diagnosis and treatment in EES.
Humans ; Otitis Media with Effusion/etiology* ; Sarcoma, Ewing/therapy* ; Skull Base ; Skull Base Neoplasms/therapy*

OBJECTIVE: To explore the clinical application value of mineralized collagen (MC) bone scaffolds in repairing various types of skull defects, and to assess the suitability and repair effectiveness of porous MC (pMC) scaffolds, compact MC (cMC) scaffolds, and biphasic MC composite (bMC) scaffolds. METHODS: A retrospective analysis was conducted on the clinical data of 105 patients who underwent skull defect repair with pMC, cMC, or bMC between October 2014 and April 2022. The cohort included 63 males and 42 females, ranging in age from 3 months to 55 years, with a median age of 22.7 years. Causes of defects included craniectomy after traumatic surgery in 37 cases, craniotomy in 58 cases, tumor recurrence or intracranial hemorrhage surgery in 10 cases. Appropriate MC scaffolds were selected based on the patient's skull defect size and age: 58 patients with defects <3 cm² underwent skull repair with pMC (pMC group), 45 patients with defects ≥3 cm² and aged ≥5 years underwent skull repair with cMC (cMC group), and 2 patients with defects ≥3 cm² and aged <5 years underwent skull repair with bMC (bMC group). Postoperative clinical follow-up and imaging examinations were conducted to evaluate bone regeneration, the biocompatibility of the repair materials, and the occurrence of complications. RESULTS: All 105 patients were followed up 3-24 months, with an average of 13 months. No material-related complication occurred in any patient, including skin and subcutaneous tissue infection, excessive ossification, and rejection. CT scans at 6 months postoperatively showed bone growth in all patients, and CT scans at 12 months postoperatively showed complete or near-complete resolution of bone defects in all patients, with 58 cases repaired in the pMC group. The CT values of the defect site and the contralateral normal skull bone in the pMC group at 12 months postoperatively were (1 123.74±93.64) HU and (1 128.14±92.57) HU, respectively, with no significant difference ( t=0.261, P=0.795). CONCLUSION MC exhibits good biocompatibility and osteogenic induction ability in skull defect repair. pMC is suitable for repairing small defects, cMC is suitable for repairing large defects, and bMC is suitable for repairing pediatric skull defects.
Humans ; Tissue Scaffolds ; Male ; Female ; Collagen ; Retrospective Studies ; Adult ; Child ; Adolescent ; Middle Aged ; Child, Preschool ; Skull/surgery* ; Young Adult ; Infant ; Plastic Surgery Procedures/methods* ; Tissue Engineering/methods* ; Craniotomy/methods* ; Bone Regeneration ; Treatment Outcome ; Porosity ; Biocompatible Materials

Objective:To explore the surgical techniques and clinical outcomes of endoscopic transnasal approaches in the treatment of skull base chondrosarcomas. Methods:Data from patients diagnosed with skull base chondrosarcomas and treated via endoscopic transnasal surgery at the Department of Otorhinolaryngology and Head and Neck Surgery, Xuanwu Hospital, Capital Medical University, from 2013 to 2022 were collected. This retrospective study analyzed the patients' clinical presentations, histopathological grading, involved sites and extents, and complications following the endoscopic transnasal surgery. Disease-free survival rates were calculated using the Kaplan-Meier method. Results:Complete data from 31 patients showed that the primary tumor site was in the petroclival region in 27 cases（87%）, and the anterior skull base in 4 cases（13%）. Pathological grades were Grade Ⅰ（12 cases）, Grade Ⅱ（16 cases）, and Grade Ⅲ（3 cases）. Total resection was achieved in 25 cases, with residual disease post-surgery in 6 cases. The average follow-up duration was 35.7 months（ranging from 6 to 120 months）. Among those who achieved complete resection, recurrence occurred in 5 cases（5/25）, with a five-year disease-free survival rate of 80%. Postoperative complications included transient abducens nerve palsy in 6 patients and cerebrospinal fluid rhinorrhea in 4 patients. There were no cases of death or permanent cranial nerve palsy. Total resection rate（P=0.001） and involvement of the cerebellopontine angle and jugular foramen（P=0.037） were identified as independent risk factors for residual disease and recurrence of chondrosarcoma. Conclusion:The endoscopic transnasal approach is a safe and feasible treatment option for skull base chondrosarcomas.
Humans ; Chondrosarcoma/surgery* ; Skull Base Neoplasms/surgery* ; Retrospective Studies ; Male ; Female ; Endoscopy/methods* ; Middle Aged ; Skull Base/surgery* ; Treatment Outcome ; Adult ; Neoplasm Recurrence, Local ; Disease-Free Survival ; Nose/surgery*

Humans ; Intervertebral Disc Displacement/surgery* ; Skull ; Lumbar Vertebrae/surgery* ; Blindness ; Treatment Outcome ; Intervertebral Disc Degeneration/surgery*

The cranial neural crest plays a fundamental role in orofacial development and morphogenesis. Accordingly, mutations with impact on the cranial neural crest and its development lead to orofacial malformations such as cleft lip and palate. As a pluripotent and dynamic cell population, the cranial neural crest undergoes vast transcriptional and epigenomic alterations throughout the formation of facial structures pointing to an essential role of factors regulating chromatin state or transcription levels. Using CRISPR/Cas9-guided genome editing and conditional mutagenesis in the mouse, we here show that inactivation of Kat5 or Ep400 as the two essential enzymatic subunits of the Tip60/Ep400 chromatin remodeling complex severely affects carbohydrate and amino acid metabolism in cranial neural crest cells. The resulting decrease in protein synthesis, proliferation and survival leads to a drastic reduction of cranial neural crest cells early in fetal development and a loss of most facial structures in the absence of either protein. Following heterozygous loss of Kat5 in neural crest cells palatogenesis was impaired. These findings point to a decisive role of the Tip60/Ep400 chromatin remodeling complex in facial morphogenesis and lead us to conclude that the orofacial clefting observed in patients with heterozygous KAT5 missense mutations is at least in part due to disturbances in the cranial neural crest.
Animals ; Mice ; Chromatin Assembly and Disassembly ; Cleft Lip/genetics* ; Cleft Palate/genetics* ; DNA Helicases/metabolism* ; DNA-Binding Proteins ; Neural Crest/metabolism* ; Skull ; Transcription Factors/metabolism*

Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.
Adult ; Female ; Humans ; Male ; Middle Aged ; Chondrosarcoma/surgery* ; Fibrosarcoma ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skull Base/surgery* ; Skull Base Neoplasms/surgery* ; Temporal Bone/pathology* ; Treatment Outcome