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BACKGROUND Little is known about the detailed growth of the sphenoidal and temporal bones, even though they contribute significantly to the cranial base and cranial fossa skeletons. They also serve to connect the cranial vault with facial structure. This study details their morphologic development in isolated bicoronal synostosis and associated syndromes. METHODS Eighty-one CT scans were included (nonsyndromic bicoronal synostosis, n = 28; Apert syndrome associated with bicoronal synostosis, n = 19; Crouzon syndrome associated with bicoronal synostosis, n = 8; and controls, n = 26), and measured using Materialize software. RESULTS Sphenoidal and temporal bone volumes in nonsyndromic bicoronal synostosis are reduced 23% (P = 0.005) and 24%(P = 0.003) at 6 months of age, compared to controls. Apert and Crouzon syndrome patients developed similar reduced volumes. The greater wing of the sphenoid and pterygoid processes in nonsyndromic bicoronal synostosis are initially inferiorly rotated at 2 months of age, by 9.60° (P = 0.002) and 4.33° (P = 0.023), respectively. In Apert syndrome, these rotations were reduced by 4.82° (P = 0.003) and 12.60° (P

OBJECTIVE This study evaluated the efficacy of 3-dimensional fluid-attenuated inversion recovery (3D FLAIR) for detecting intradural ecchordosis physaliphora (EP). METHODS We retrospectively determined the presence or absence of intradural EP on 3D FLAIR for 3888 consecutive patients, classifying the EP as "classical" or "possible" and analyzing the prevalence, size, and presence or absence of an intraosseous stalk. Where available, magnetic resonance cisternography images were compared with the 3D FLAIR images. RESULTS Intradural EP was identified in 50 patients (1.3%): 36 (0.9%) classical and 14 (0.4%) possible. The classical EPs were significantly larger than the possible EPs (P < 0.01). Nine EPs (18.0%) showed an osseous stalk. Magnetic resonance cisternography was performed for 19 EPs (16 classical, 3 possible), detecting all 16 classical EPs but none of the possible EPs. CONCLUSIONS Classical EPs were detected by 3D FLAIR as with magnetic resonance cisternography. The 3D FLAIR findings suggested a new type of possible EP variant previously unreported.

BACKGROUND Based on an established classification system of Apert syndrome subtypes, detailed regional morphology and volume analysis may be useful to provide additional clarification to individual Apert cranial structure characteristics, and treatment planning. METHODS Computed tomography scans of 32 unoperated Apert syndrome and 50 controls were included and subgrouped as: type I, bilateral coronal synostosis; type II, pansynostosis; type III, perpendicular combination synostosis. Three-dimensional analysis of craniometric points was used to define structural components using Materialise Mimics and 3-Matics software. RESULTS Occipitofrontal circumference of all subtypes of Apert syndrome patients is normal. Intracranial volumes of types I and II were normal, but type III was 20% greater than controls. Middle cranial fossa volume was increased in all 3 types, with the greatest increase in type II (86%). Type II developed a 69% increase in anterior cranial fossa volume, whereas type III had 39% greater posterior cranial fossa volume. Increased cranial fossa depth contributed most to above increased volume. The anteroposterior lengths of middle and posterior cranial fossae were reduced in type I (15% and 17%, respectively). However, only the anterior cranial fossa was significantly shortened in type III. CONCLUSIONS Occipitofrontal circumference and overall intracranial volume is not always consistent in individual subunits of Apert syndrome. Detailed and segmental anterior, middle, and posterior cranial fossae volumes and morphology should be analyzed to see what impact this may have related to surgical planning.

The use of posterior calvarial distraction (PCD) for the management of craniosynostosis is well recognized. The advantages of using this technique include increased cranial volume, decreased intracranial pressure, relief of posterior fossa crowding, improved cerebrospinal fluid (CSF) circulation at the cranio-cervical junction with cessation, and possible resolution of syrinx.The authors retrospectively review their first 50 patients who have undergone PCD under the senior author's care in our unit.The demographics, diagnoses, intraoperative approach with techniques in distractor placement and outcomes of each patient were obtained through an electronic craniofacial database and written patient records. Analysis of complication rates (bleeding, distraction problems, CSF leaks, and infection) was included.A total of 31 boys and 19 girls underwent the procedure between October 2006 and September 2015 with a median age was 17.7 months (range 4 months to 19 years). Of those 50 children, 34 of the cohort were proven to be syndromic by genetic testing.The median length of inpatient stay was 9.4 days (range 3-43 days). Average distraction distance was 24 mm.Complications including CSF leaks, bleeding, distractor problems, and severe complications (recorded in 3 patients) are discussed. Our overall complication rate was 50%.Favorable outcomes included resolution of Chiari, syrinx, and raised intracranial pressure in the majority of patients where distraction was successful.The authors recommend that PCD should be considered the primary treatment for increasing calvarial volume. The authors discuss our experiences and technical innovations over the past decade.

To determine the efficacy and morbidity of repairing spontaneous cerebrospinal fluid (CSF) leaks with the middle cranial fossa (MCF) approach without the use of a lumbar drain (LD), as perioperative use of LD remains controversial.Retrospective review from 2003 to 2015.University of Iowa Hospitals and Clinics and Indiana University Health Center.Those with a confirmed lateral skull base spontaneous CSF leaks and/or encephaloceles.MCF approach for repair of spontaneous CSF leak and/or encephalocele without the use of lumbar drain. Assessment of patient age, sex, body mass index (BMI), and medical comorbidities.Spontaneous CSF leak patient characteristics (age, sex, BMI, obstructive sleep apnea) were collected. Length of stay (LOS), hospital costs, postoperative complications, CSF leak rate, and need for LD were calculated.Sixty-five operative MCF repairs were performed for spontaneous CSF leaks on 60 patients (five had bilateral CSF leaks). CSF diversion with LD was used in 15 of 60 patients, mostly before 2010. After 2010, only three of 44 patients (6.7%) had postoperative otorrhea requiring LD. The use of LD resulted in significantly longer LOS (3.6 ± 1.6 versus 8.7 ± 2.9 d) and hospital costs ($29,621). There were no postoperative complications in 77% (50 of 65) of cases. Three cases required return to the operating room for complications including frontal subdural hematoma (1), subdural CSF collection (1), and tension pneumocephalus (1). No patients experienced long-term neurologic sequelae or long-term CSF leak recurrence with an average length of follow-up of 19.5 months (range 3-137 mo). The average patient BMI was 37.5 ± 8.6 kg/m. The average age was 57.5 ± 11.4 years and 68% were female. Obstructive sleep apnea was present in 43.3% (26 of 60) of patients.The morbidity of the MCF craniotomy for repair of spontaneous CSF leaks is low and the long-term efficacy of repair is high. Universal use of perioperative lumbar drain is not indicated and significantly increases length of stay and hospital costs. Obesity and obstructive sleep apnea are highly associated with spontaneous CSF leaks.

The posterior fossa is an important brain structure containing the cerebellum, cerebral ventricle, and cistern. Early evaluation of the cerebellar structure and function may be valuable for early detection of fetal deformities. At present, no normal value for the fetal posterior fossa has been established yet. This study is aimed to investigate the development of the posterior fossa in normal Chinese fetuses by using magnetic resonance imaging (MRI). Pregnant women who need MRI scan were enrolled in our Hospital between January 2012 and December 2014. The fetal supero-inferior diameter (SID), anterio-posterior diameter (APD), cerebellar vermis area, cerebellar width (CW), cerebellar volume (CV), superior cerebellar cistern width, and cerebellomedullary cistern width were measured using MRI. Pearson's correlation analysis was used to detect the relationship between those parameters and gestational age. A regression analysis was performed for all parameters. A total of 92 participants were retrospectively enrolled finally. The results indicated SID, APD, cerebellar vermis area, CW, and CV were positively associated with gestational age, while no significant correlation was found between the superior cerebellar cistern width and cerebellomedullary cistern width and gestational age. Each equation was established. Our study demonstrated that MRI has the advantages over ultrasound imaging for prenatal evaluation of the fetal posterior fossa with multiple views. Normal value of the posterior fossa of Chinese fetuses was established in this study.

Background It has been well documented that along with tonsillar herniation, Chiari Malformation Type I (CMI) is associated with smaller posterior cranial fossa (PCF) and altered CSF flow and tissue motion in the cranio-cervical junction (CCJ).

To present the results of the traumatic intratemporal facial nerve injury that have undergone facial nerve decompression by using middle cranial fossa (MCF) approach.A retrospective studyTertiary referral centerIn this study, 13 patients who developed facial paralysis due to temporal bone trauma and undergone decompression by using MCF approach in Department of Otorhinolaryngology of İzmir Katip Celebi University Research and Training Hospital between January 1993 and December 2012 were presented retrospectively. Patients were assessed in terms of side, etiology, fracture type, House-Brackmann (HB) grade, electroneuronography (ENOG), electromyography (EMG), hearing loss, operation time, and the region of the injury.The fracture was at the right side in 7 (53.8%) and at the left side in 6 patients (46.1%). The type of temporal bone fracture was longitudinal in 6 (46.1%), transverse in 2 (15.3%), and mixed in 5 patients (38.4%). Total axonal degeneration in EMG and ENOG were seen in all patients, who were HB grade 6 at preoperative assessment. Mean operation time was 30 days. The lesion in all patients was at the region of geniculate ganglion. There was conductive hearing loss in 7 patients (53.8%), sensorineural in 4 (30.7%), and mixed in 1 patient (7.6%); hearing was normal in 1 patient (7.6%). Seven patients (53.8%) improved to HB grade 2.In the light of the information obtained from HRCT, ENOG, and EMG, we believe that better results can be achieved with facial nerve decompression that is performed before 1 month, and geniculate ganglion region may be better controlled by MCF approach.

Background Chordomas are primarily malignant tumors encountered at either end of the neural axis; the craniovertebral junction and the sacrococcygeal junction. In this article, we discuss the surgical management of craniovertebral junction chordomas. Objective In this paper, we discuss the surgical management of craniovertebral junction chordomas. Results The following approaches are illustrated: transoral-transpalatopharyngeal approach, high anterior cervical retropharyngeal approach, endoscopic transoral approach, and endoscopic transnasal approach. No single operative approach can be used for all craniovertebral chordomas. Therefore, the location of the tumor dictates which approach or approaches should be used.

Objective The indications and operative technique for a number of posterolateral approaches to the craniovertebral junction (CVJ) are reviewed. Methods The literature addressing posterolateral approaches to the CVJ is reviewed, and illustrative cases are presented. Results The far lateral approach and its variants, including the transcondylar, supracondylar, and paracondylar approaches, are an effective means of addressing intradural anterior and anterolateral CVJ lesions. These approaches provide exposure of the lower third of the clivus, the foramen magnum, and the upper cervical spine; do not cross contaminated regions; and enable a watertight dural closure to be performed. They are associated with minimal morbidity and usually do not significantly decrease the stability of the CVJ. Conclusion All surgeons treating lesions of the CVJ should be familiar with the posterolateral approach and its modifications.

A 27-year-old woman developed a rapidly progressive left orbital tumor that extended in the cranial fossa. MRI revealed a heterogenous enhancing lesion confined to the left frontal bone and superior orbit. An incisional biopsy was performed, and histopathologic examination of the specimen showed fin

Osteosarcoma can develop secondary to malignant transformation within a preexisting benign bone lesion, such as Paget disease, osteonecrosis, fibrous dysplasia, and chronic infection, or it can arise in previously irradiated areas. This article presents the case of a 36-year-old woman who was affected by a radioinduced osteosarcoma after radiotherapy for an adenoid cystic carcinoma of the left parotid gland treated when she was 24 years old.

A 12-year-old boy presented to our emergency department after being shot in the face. A computed tomographic scan revealed a bullet through the posterolateral wall of the maxillary sinus into the right infratemporal area, just adjacent to the skull base. We elected transantral approach with the help of endoscopy and C-arm. The bullet was successfully removed. Little is known on the best strategy for removing the infratemporal foreign body. Our experience in this case provides a safe and effective way for such injury.

An unusual case of massive oropharyngeal swelling and macroglossia occurring after cervical spine surgery performed on a patient in the prone position is described. Possible etiological factors are reviewed, and measures to prevent these complications are suggested.

A meningoencephalocele is a herniation of meninges and brain out of the cranial fossa through a bony defect. Cerebrospinal fluid leakage may occur due to a defect in the wall of the meningoencephalocele. The defect may be traumatic, iatrogenic, or may appear spontaneously. In this report, the authors present an unrecognized transethmoidal meningoencephalocele that resulted in a cerebrospinal fluid leak during dacryocystorhinostomy.

Traumatic posterior fossa hematomas are uncommon. Subdural hematomas in this location are probably the rarest. The natural history in acute cases is one of rapid deterioration and death from brain-stem compression. Rapid surgical intervention is indicated in acute cases. We report a case of a traumatic posterior fossa subdural hematoma in a 3-year-old child treated conservatively with a good outcome.

A case of benign osteoblastoma of the lower clivus is reported. The clinical and radiological features and the surgical rationale for the transoral approach are briefly discussed.

The brain stem auditory evoked response (BAER) was monitored intraoperatively in 11 patients with tumors of the posterior fossa. A technique for the objective quantitative assessment of the BAER based on the variance of the phase angle between small group averages is described. The resulting component synchrony measure corroborated standard peak latency and amplitude changes of the BAER during operative manipulation. A discussion of the replication and use of the objective measure is presented.