Delayed presentation of morning glory disc anomaly and transsphenoidal encephalocele: A management dilemma.

Background Morning glory disc anomaly (MGDA) is a rare developmental abnormality of the optic disc that is associated with many other neurological and vascular conditions. Most cases are diagnosed in childhood. Case Report We report a 57-year-old woman who presented to the ophthalmology department for assessment of long-standing poor vision in the left eye and exotropia. Examination showed a left MGDA and bitemporal hemianopsia. These findings prompted magnetic resonance imaging, revealing a transsphenoidal basal meningoencephalocele with herniation of the optic chiasm and inferior hypothalamus into the dural sac. Due to the eloquence of the neurovascular structures it contained, a decision was made not to reduce the meningoencephalocele. Instead, a ventriculoperitoneal shunt was placed. The patient's ophthalmologic examination remained stable over the following year. Conclusion While rare, MGDA can be first diagnosed in late adulthood and a thorough evaluation should be completed to assess for midline cranial defects, vascular abnormalities, and other associated abnormalities. Patients presenting late in life with basal encephalocele, herniation of the optic chiasm, and bitemporal hemianopsia present a management dilemma. In this case, a ventriculoperitoneal shunt was placed with the intention of lowering intracranial pressure to prevent further herniation and reduce the risk of cerebrospinal fluid leak.

Optimizing ventriculoperitoneal shunt placement in the treatment of idiopathic intracranial hypertension: an analysis of neuroendoscopy, frameless stereotaxy, and intraoperative CT.

OBJECTIVE: Cerebrospinal fluid shunting can effectively lower intracranial pressure and improve the symptoms of idiopathic intracranial hypertension (IIH). Placement of ventriculoperitoneal (VP) shunts in this patient population can often be difficult due to the small size of the ventricular system. Intraoperative adjuvant techniques can be used to improve the accuracy and safety of VP shunts for these patients. The purpose of this study was to analyze the efficacy of some of these techniques, including the use of intraoperative CT (iCT) and frameless stereotaxy, in optimizing postoperative ventricular catheter placement. METHODS: The authors conducted a retrospective review of 49 patients undergoing initial ventriculoperitoneal shunt placement for the treatment of IIH. The use of the NeuroPEN Neuroendoscope, intraoperative neuronavigation, and iCT was examined. To analyze ventricular catheter placement on postoperative CT imaging, the authors developed a new grading system: Grade 1, catheter tip terminates optimally in the ipsilateral frontal horn or third ventricle; Grade 2, catheter tip terminates in the contralateral frontal horn; Grade 3, catheter terminates in a nontarget CSF space; and Grade 4, catheter tip terminates in brain parenchyma. All shunts had spontaneous CSF flow upon completion of the procedure. RESULTS: The average body mass index among all patients was 37.6 ± 10.9 kg/m2. The NeuroPEN Neuroendoscope was used in 44 of 49 patients. Intraoperative CT scans were obtained in 24 patients, and neuronavigation was used in 32 patients. Grade 1 or 2 final postoperative shunt placement was achieved in 90% of patients (44 of 49). In terms of achieving optimal postoperative ventricular catheter placement, the use of iCT was as effective as neuronavigation. Two patients had their ventricular catheter placement modified based on an iCT study. The use of neuronavigation significantly increased time in the operating room (223.4 ± 46.5 vs. 190.8 ± 31.7 minutes, p = 0.01). There were no shunt infections in this study. CONCLUSIONS: The use of iCT appears to be equivalent to the use of neuronavigation in optimizing ventricular shunt placement in IIH. Additionally, it may shorten operating room time and limit overall costs.

Recurrent ectopic craniopharyngioma in the sylvian fissure thirty years after resection through a pterional approach: a case report and review of the literature.

Local recurrence of craniopharyngiomas after apparently complete resection occurs frequently. Ectopic recurrence remote from the original site has been reported in 18 adult patients. The interval between the original diagnosis and the time of recurrence varies widely in these reports (1-26 years). We report a case of an ectopic recurrence in the sylvian fissure of an adamantinomatous type craniopharyngioma 34 years after the initial presentation and 30 years after the last surgical resection. In addition to this being the latest reported ectopic recurrence, the location of this new lesion in the sylvian fissure is fairly rare, having been reported in only three other cases. We also reviewed the English literature for reports of ectopic recurrent craniopharyngiomas in order to conduct an analysis of surveillance and treatment strategies.

Endoscopic endonasal craniopharyngioma resection.

The authors present the case of a 21-year-old female with a progressive bitemporal hemianopsia. Cranial MR imaging revealed a large cystic suprasellar, retrochiasmatic lesion consistent with craniopharyngioma. The lesion was fully resected through an endoscopic endonasal transsphenoidal and transplanum approach. Closure of the resultant dural defect was performed with a bilayer fascia lata button and autologous mucoperichondrial nasoseptal flap. Each portion of this procedure was recorded and is presented in an edited high-definition format. The video can be found here: http://youtu.be/i3-qieLlbVk .

Surgical management of temporal meningoencephaloceles, cerebrospinal fluid leaks, and intracranial hypertension: treatment paradigm and outcomes.

OBJECT: Thinning of the tegmen tympani and mastoideum components of the temporal bone may predispose to the development of meningoencephaloceles and spontaneous CSF leaks. Surgical repair of these bony defects and associated meningoencephaloceles aids in the prevention of progression and meningitis. Intracranial hypertension may be a contributing factor to this disorder and must be fully evaluated and treated when present. The purpose of this study was to establish a treatment paradigm for tegmen defects and elucidate causative factors. METHODS: The authors conducted a retrospective review of 23 patients undergoing a combined mastoidectomy and middle cranial fossa craniotomy for the treatment of a tegmen defect. RESULTS: The average body mass index (BMI) among all patients was 33.2 ± 7.2 kg/m(2). Sixty-five percent of the patients (15 of 23) were obese (BMI > 30 kg/m(2)). Preoperative intracranial pressures (ICPs) averaged 21.8 ± 6.0 cm H(2)O, with 10 patients (43%) demonstrating an ICP > 20 cm H(2)O. Twenty-two patients (96%) had associated encephaloceles. Five patients underwent postoperative ventriculoperitoneal shunting. Twenty-two CSF leaks (96%) were successfully repaired at the first attempt (average follow-up 10.4 months). CONCLUSIONS: Among all etiologies for CSF leaks, those occurring spontaneously have the highest rate of recurrence. The surgical treatment of temporal bone defects, as well as the recognition and treatment of accompanying intracranial hypertension, provides the greatest success rate in preventing recurrence. After tegmen dehiscence repair, ventriculoperitoneal shunting should be considered for patients with any combination of the following high-risk factors for recurrence: spontaneous CSF leak not caused by another predisposing condition (that is, trauma, chronic infections, or prior surgery), high-volume leaks, CSF opening pressure > 20 cm H(2)O, BMI > 30 kg/m(2), preoperative imaging demonstrating additional cranial base cortical defects (that is, contralateral tegmen or anterior cranial base) and/or an empty sella turcica, and any history of an event that leads to inflammation of the arachnoid granulations and impairment of CSF absorption (that is, meningitis, intracranial hemorrhage, significant closed head injury, and so forth).

The Lingual Process of the Sphenoid Bone and the Petrolingual Ligament: Surgical Anatomy, Landmarks, and Clinical Relevance.

BACKGROUND: The lingual process of the sphenoid bone (LP) and the petrolingual ligament (PLL) surround laterally the internal carotid artery within the middle cranial fossa (MCF). OBJECTIVE: To study the LP and the PLL and anatomical variations considering their relationships with different structures and landmarks within the MCF, especially oriented toward the endoscopic endonasal approaches. METHODS: Seventy-two sides of dry skulls and 20 sides of embalmed specimens were studied. The measurements of the LP and the PLL were obtained, considering important landmarks in the MCF. RESULTS: The LP had a mean length and height of 5 mm and 3 mm, respectively. Its distance from the foramen lacerum was 6 mm, from the foramen ovale 10 mm, foramen rotundum 15 mm, and petrous apex 9 mm. In 44 sides (61.11%), the LP partially closed the lateral aspect of the carotid sulcus; in 17 sides (23.61%), it was found as a near-ring; and in 11 sides (15.2%), it was considered rudimentary. Considering the PLL, its length and height were, respectively, 9 mm, and 4 mm. CONCLUSION: The LP and PLL separate the carotid artery at the inferior aspect of Meckel's cave and constitute important landmarks for endoscopic endonasal approaches to Meckel's cave and MCF, and their identification and removal is essential for internal carotid artery mobilization in this area.

Pituitary hyperplasia causing complete bitemporal hemianopia with resolution following surgical decompression: case report.

In this report, the authors demonstrated that idiopathic pituitary hyperplasia (PH) can cause complete bitemporal hemianopia and amenorrhea, even in the setting of mild anatomical compression of the optic chiasm and normal pituitary function. Furthermore, complete resolution of symptoms can be achieved with surgical decompression. PH can occur in the setting of pregnancy or end-organ insufficiency, as well as with medications such as oral contraceptives and antipsychotics, or it can be idiopathic. It is often found incidentally, and surgical intervention is usually unnecessary, as the disorder rarely progresses and can be managed by treating the underlying etiology. Here, the authors present the case of a 24-year-old woman with no significant prior medical history, who presented with bitemporal hemianopia and amenorrhea. Imaging revealed an enlarged pituitary gland that was contacting, but not compressing, the optic chiasm, and pituitary hormone tests were all within normal limits. The patient underwent surgical decompression of the sella turcica and exploration of the gland through an endoscopic endonasal transsphenoidal approach. Pathology results demonstrated PH. A postoperative visual field examination revealed complete resolution of the bitemporal hemianopia, and menstruation resumed 3 days later. The patient remains asymptomatic with no hormonal deficits.

Lengthening the nasoseptal flap pedicle with extended dissection into the pterygopalatine fossa.

OBJECTIVES/HYPOTHESIS: Releasing the nasoseptal flap (NSF) pedicle from the sphenopalatine artery (SPA) foramen may considerably improve flap reach and surface area. Our objectives were quantify increases in pedicle length and NSF reach through extended pedicle dissection into the pterygopalatine fossa (PPF) through cadaveric dissections and present clinical applications. STUDY DESIGN: Anatomical study and retrospective clinical cohort study. METHODS: Twelve cadaveric dissections were performed. Following standard NSF harvest, the distance from the anterior edge of the flap to the anterior nasal spine while pulling the flap anteriorly was measured. As dissection into the SPA foramen and PPF continued, similar interval measurements were completed in four stages after release from the SPA foramen, release of the internal maxillary artery (IMAX), and transection of the descending palatine artery (DPA). The extended pedicle dissection technique was performed in seven consecutive patients for a variety of different pathologies. RESULTS: The mean length of the NSF from the anterior nasal spine and maximum flap reach were 1.91 ± 0.40 cm/9.3 ± 0.39 cm following standard harvest, 2.52 ± 0.61 cm/9.75±1.06 cm following SPA foramen release, 4.93 ± 0.89 cm/12.16 ± 0.54 cm following full IMAX dissection, and 6.18 ± 0.68 cm/13.41 ± 0.75 cm following DPA transection. No flap dehiscence or necrosis was observed in all seven surgical patients. CONCLUSIONS: Extended pedicle dissection of the NSF to the SPA/IMAX markedly improves the potential length and reach of the flap. This technique may provide a feasible option for reconstruction of large anterior skull base and craniocervical junction defects. Seven successful cases are presented here, but further studies with larger series are warranted to validate findings in a clinical setting. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:18-24, 2020.

A comparison of hinge craniotomy and decompressive craniectomy for the treatment of malignant intracranial hypertension: early clinical and radiographic analysis.

OBJECT: Hinge craniotomy (HC) has recently been described as an alternative to decompressive craniectomy (DC). Although HC may obviate the need for cranial reconstruction, an analysis comparing HC to DC has not yet been published. METHODS: A retrospective review was conducted of 50 patients who underwent cranial decompression (20 with HC, 30 with DC). Baseline demographics, neurological examination results, and underlying pathology were reviewed. Clinical outcome was assessed by length of ventilatory support, length of intensive care unit stay, and survival at discharge. Control of intracranial hypertension was assessed by average daily intracranial pressure (ICP) for the duration of ICP monitoring and an ICP therapeutic intensity index. Radiographic outcomes were assessed by comparing preoperative and postoperative CT scans for: 1) Rotterdam score; 2) postoperative volume of cerebral expansion; 3) presence of uncal herniation; 4) intracerebral hemorrhage; and 5) extraaxial hematoma. Postoperative CT scans were analyzed for the size of the craniotomy/craniectomy and magnitude of extracranial herniation. RESULTS: No significant differences were identified in baseline demographics, neurological examination results, or Rotterdam score between the HC and DC groups. Both HC and DC resulted in adequate control of ICP, as reflected in the average ICP for each group of patients (HC = 12.0 +/- 5.6 mm Hg, DC = 12.7 +/- 4.4 mm Hg; p > 0.05) at the same average therapeutic intensity index (HC = 1.2 +/- 0.3, DC = 1.2 +/- 0.4; p > 0.05). The need for reoperation (3 [15%] of 20 patients in the HC group, 3 [10%] of 30 patients in the DC group; p > 0.05), hospital survival (15 [75%] of 20 in the HC group, 21 [70%] of 30 in the DC group; p > 0.05), and mean duration of both mechanical ventilation (9.0 +/- 7.2 days in the HC group, 11.7 +/- 12.0 days in the DC group; p > 0.05) and intensive care unit stay (11.6 +/- 7.7 days in the HC group, 15.6 +/- 15.3 days in the DC group; p > 0.05) were similar. The difference in operative time for the two procedures was not statistically significant (130.4 +/- 71.9 minutes in the HC group, 124.9 +/- 63.3 minutes in the DC group; p > 0.05). The size of the cranial defect was comparable between the 2 groups. Postoperative imaging characteristics, including Rotterdam score, also did not differ significantly. Although a smaller volume of cerebral expansion was associated with HC (77.5 +/- 54.1 ml) than DC (105.1 +/- 65.1 ml), this difference was not statistically significant. CONCLUSIONS: Hinge craniotomy appears to be at least as good as DC in providing postoperative ICP control and results in equivalent early clinical outcomes.

Complications of cranioplasty following decompressive craniectomy: analysis of 62 cases.

OBJECT: Decompressive craniectomy is a potentially life-saving procedure used in the treatment of medically refractory intracranial hypertension, most commonly in the setting of trauma or cerebral infarction. Once performed, surviving patients are obligated to undergo a second procedure for cranial reconstruction. The complications following cranial reconstruction are not well described in the literature and may very well be underreported. A review of the complications would suggest measures to improve the care of these patients. METHODS: A retrospective chart review was undertaken of all patients who had undergone cranioplasty during a 7-year period. Demographic data, indications for craniectomy, as well as preoperative, intraoperative, and postoperative parameters following cranioplasty, were recorded. Perioperative and postoperative complications were also recorded. Patients were classified as having no complications, any complications, and complications requiring reoperation. The groups were compared to identify risk factors predictive of poor outcomes. RESULTS: The authors identified 62 patients who had undergone cranioplasty. The immediate postoperative complication rate was 34%. Of these, 46 patients did not require reoperation and 16 did. Of those requiring reoperation, 7 were due to infection, 2 from wound breakdown, 2 from intracranial hemorrhage, 3 from bone resorption, and 1 from a sunken cranioplasty, and 1 patient's cranioplasty procedure was prematurely ended due to intraoperative hypotension and bradycardia. The only factor statistically associated with need for reoperation was the presence of a bifrontal cranial defect (bifrontal: 8 [67%] of 12, requiring reoperation; unilateral: 8 [16%] of 49 requiring reoperation; p < 0.01) CONCLUSIONS: Cranioplasty following decompressive craniectomy is associated with a high complication rate. Patients undergoing a bifrontal craniectomy are at significantly increased risk for postcranioplasty complications, including the need for reoperation.

Temporal Bone and Cerebellopontine Angle Epidermoid Resulting in Facial Nerve Paralysis: Resection and Facial Nerve Coaptation.

Objectives Demonstrate the utilization of a transcochlear approach for resection of an epidermoid involving the temporal bone and cerebellopontine angle (CPA) with end-to-end facial nerve coaptation. Designs Single case-based operative video. Setting Tertiary center with dedicated skull base team. Participants The patient is a 50-year-old left handed male with a history of a remote left Bell's palsy, left sudden sensorineural hearing loss, and a rapidly progressive facial nerve paralysis. His balance was impaired, and his videonystagmography showed a significant left sided peripheral vestibular weakness. Computed tomography (CT) scan showed an erosive lesion of his left temporal bone involving the cochlea and semicircular canals, and magnetic resonance imaging (MRI) showed a T2 hyperintense lesion with restricted diffusion and no enhancement on postcontrast T1 sequences. Main Outcome Measures Gross total resection of the epidermoid, recovery of facial nerve function, balance improvement. Results The patient underwent resection via a transcochlear approach. The tumor involved the epitympanum and eroded the semicircular canals, vestibule, and basal turn of the cochlea. Gross total tumor resection was attained. The facial nerve was isolated in the mastoid and tympanic segments, traced proximally to the geniculate ganglion, and then into the internal auditory canal (IAC). The nerve was discontinuous in the distal IAC and a reactive neuroma was resected. The facial nerve was mobilized and an end-to-end coaptation was performed in the CPA using a collagen tubule. The 3-month postoperative MRI showed no residual or recurrent disease. His postoperative balance was improved. Partial facial nerve recovery is not expected prior to 9 to 12 months. The link to the video can be found at: https://youtu.be/C6N8qPwBt2Y .

Microvascular Decompression and Nervus Intermedius Sectioning for the Treatment of Geniculate Neuralgia.

Objectives Demonstrate the surgical treatment of geniculate neuralgia via microvascular decompression and nervus intermedius sectioning. Designs Single case-based operative video. Setting Tertiary center with dedicated skull base team. Participants The patient is a 62-year-old female with a history of deep right-sided otalgia consistent with geniculate neuralgia. She failed appropriate medical treatment. Her magnetic resonance imaging (MRI) showed an ectatic vertebrobasilar system as well as an anterior inferior cerebellar artery (AICA) loop causing compression of the VII/VIII nerve complex in the cerebellopontine angle. Main Outcome Measures Resolution of right-sided otalgia. Results The patient underwent retrosigmoid craniotomy with microvascular decompression of the VII/VIII nerve complex and nervus intermedius sectioning. Intraoperatively, the patient was noted to have an ectatic vertebral artery and AICA that were compressing the root entry zone of the VII/VIII nerve complex. Microvascular decompression was performed of both the vertebral artery and AICA with Teflon. The nervus intermedius was sharply sectioned. The patient's postoperative course was uneventful with no complications. She continues to have resolution of her right sided otalgia at 6 months postoperatively. The link to the video can be found at: https://youtu.be/uRb_QfrINSk .

The Magnetic Resonance Imaging Appearance of Endoscopic Endonasal Skull Base Defect Reconstruction Using Free Mucosal Graft.

OBJECTIVE: At our institution, skull base reconstruction using a free mucosal graft from the nasal cavity floor has been the standardized technique after pituitary adenoma resection via transsellar approach. In this study, the expected appearance of the reconstruction on postoperative magnetic resonance imaging (MRI) scans is described and its integrity and impact on the sinonasal cavity are assessed. METHODS: Fifty patients were selected, and their electronic medical records were reviewed for postoperative course, Sino-Nasal Outcome Test-22 (SNOT-22) scores, and nasal endoscopy reports. A total of 116 postoperative MRI scans were available to evaluate 1) the appearance and thickness of the graft, 2) the enhancement of the graft, and 3) the T2 signal in sphenoid sinus as a potential indication for inflammatory disease. RESULTS: There was no significant change in the thickness of the graft over time. Except for the 7 scans that were obtained without intravenous contrast, all scans showed enhancement of the graft. About half of the patients showed persistent T2 hyperintense signal at 12 and 24 months. However, this finding was not clinically significant, because postoperative SNOT-22 scores showed minimal sinonasal impact. CONCLUSIONS: Postoperative MRI surveillance scans showed a stable appearance of the graft that mimics the native mucosa, with enhancement through time, reflecting its robust vascularization and integration to the skull base. Although persistent T2 hyperintense signal was detected in the sphenoid sinus, clinical evidence based on nasal endoscopy reports and SNOT-22 scores indicated minimal sinonasal morbidity.

Implementation of Free Mucosal Graft Technique for Sellar Reconstruction After Pituitary Surgery: Outcomes of 158 Consecutive Patients.

BACKGROUND: Cerebrospinal fluid (CSF) leak is a common complication after surgeries involving sellar reconstruction. Various techniques, including the nasoseptal flap, have been developed to limit postoperative CSF leak. However, the nasoseptal flap causes complications owing to donor site morbidity. A free mucosal graft may be just as effective in reducing CSF leaks as well as reducing postoperative nasal discomfort. This study aimed to assess operative outcomes of free mucosal graft after pituitary resection. METHODS: A retrospective chart review was performed for patients who underwent endoscopic endonasal resection of pituitary adenomas. The following data were collected: demographic data, intraoperative CSF leak, postoperative CSF leak, other complications, and mucosal graft healing at 1 month. Also, the Sinonasal Outcome Test-22 was administered preoperatively and 1 month and 3 months postoperatively. RESULTS: Charts of 158 patients were reviewed, including patients who underwent no mucosal reconstruction, free mucosal graft reconstruction, and nasoseptal flap reconstruction. There was a 7.4% postoperative CSF leak rate in patients who underwent no reconstruction (n = 27), whereas postoperative CSF leak rate was 0.82% in patients undergoing free mucosal graft reconstruction (n = 122) (P < 0.05). Sinonasal Outcome Test-22 scores for patients with free mucosal graft reconstruction showed no significant worsening postoperatively. CONCLUSIONS: The free mucosal graft is a simple and effective means of sellar reconstruction in patients undergoing endonasal endoscopic pituitary resection, and its efficacy is similar to nasoseptal flaps. The free mucosal graft technique does not worsen sinonasal morbidity postoperatively.

Extended Endoscopic Endonasal Resection of a Suprasellar and Third Ventricular Retrochiasmatic Craniopharyngioma with a Narrow Pituitary Gland-Optic Chiasm Interval: Techniques to Optimize Resection.

The extended endoscopic endonasal approach can be utilized to surgically treat pathology within the suprasellar space. This relies on a sufficient corridor and interval between the superior aspect of the pituitary gland and the optic chiasm. Tumors located in the retrochiasmatic space and within the third ventricle, however, may not have a widened interval through which to work. With mass effect on the superior and posterior aspect of the optic chiasm, the corridor between the chiasm and the pituitary gland might even be further narrowed. This may negate the possibility of utilizing the endoscopic endonasal approach for the management of pathology in this location. We present a case of a retrochiasmatic craniopharyngioma with a narrow resection corridor that was treated with the extended endoscopic approach and we review techniques to potentially overcome this limitation. The link to the video can be found at: https://youtu.be/ogRZj-aBqeQ .

Complications of Extended Endoscopic Endonasal Surgery in Elderly Patients.

Extended endoscopic endonasal procedures are not unique among surgical interventions in carrying increased risk in the elderly population. There are, however, components of the procedure, namely high-flow cerebrospinal fluid leaks, that do result in the potential for increased perioperative morbidity for these patients. We present the case of a 77-year-old male with a large invasive pituitary macroadenoma resected through a transplanum-transtuberculum-transsellar endonasal approach. A gross total resection was obtained with resolution of the patient's preoperative ophthalmologic deficits. One month postoperatively, the patient developed progressive lethargy and cranial imaging demonstrated a left convexity subacute subdural hematoma. This was evacuated through a twist drill craniostomy. Despite measures to limit the operative time of the initial endonasal procedure as well as the absence of a postoperative cerebrospinal fluid fistula, the patient still developed this complication. Along with more typical potential causes of postoperative decline following extended endonasal procedures, problems from high-flow intraoperative cerebrospinal fluid leaks alone can result in morbidity in the elderly population. This should be acknowledged preoperatively and a high suspicion should exist for the presence of intracranial hemorrhage in these patients with any postoperative deficits. Additional intraoperative measures can be utilized to minimize such risks. The link to the video can be found at: https://youtu.be/EkLmt2T8_UE .

Intratumoral Hemorrhage within Petrous Meningioma.

BACKGROUND: Intracranial hemorrhage stemming from a benign intracranial lesion is much less commonly seen than from malignant tumors such as gliomas or metastases. Cerebellopontine angle (CPA) lesions rarely present with hemorrhage. CASE DESCRIPTION: We describe the case of a 49-year-old male with a recurrent right CPA meningioma arising from the petrous bone that was previously treated with a subtotal resection and postsurgical radiosurgery, presenting with acute left-sided hemiparesis secondary to intratumoral hemorrhage. Although surgical evacuation and decompression were recommended, the patient declined operative intervention and was managed medically. CONCLUSIONS: Meningiomas can cause subarachnoid, intraparenchymal, and rarely intratumoral hemorrhage. Symptomatic hemorrhage can worsen the prognosis, with increased morbidity and mortality. Several etiologies have been proposed for this phenomenon including rupture of aberrant vasculature, intratumoral necrosis, and tearing of stretched bridging veins. Only 2 prior cases of CPA meningioma have been reported in the literature. Recognition of CPA meningioma hemorrhage as a clinical entity can help in future diagnoses and management.

A familial syndrome of hypothalamic hamartomas, polydactyly, and SMO mutations: a clinical report of 2 cases.

Hypothalamic hamartomas are benign tumors known to cause gelastic or dacrystic seizures, precocious puberty, developmental delay, and medically refractory epilepsy. These tumors are most often sporadic but rarely can be associated with Pallister-Hall syndrome, an autosomal dominant familial syndrome caused by truncation of glioblastoma transcription factor 3, a downstream effector in the sonic hedgehog pathway. In this clinical report, the authors describe two brothers with a different familial syndrome. To the best of the authors' knowledge, this is the first report in the literature describing a familial syndrome caused by germline mutations in the Smoothened (SMO) gene and the first familial syndrome associated with hypothalamic hamartomas other than Pallister-Hall syndrome. The authors discuss the endoscopic endonasal biopsy and subtotal resection of a large hypothalamic hamartoma in one of the patients as well as the histopathological findings encountered. Integral to this discussion is the understanding of the hedgehog pathway; therefore, the underpinnings of this pathway and its clinical associations to date are also reviewed.

Optimizing Sellar Reconstruction After Pituitary Surgery with Free Mucosal Graft: Results from the First 50 Consecutive Patients.

BACKGROUND: Postoperative cerebrospinal fluid leak after endoscopic pituitary surgery ranges from 1.9% to 10% in different series. Vascularized flaps have reduced the incidence of leak; however, this carries nasal morbidity. This study presents a technique for sellar reconstruction with free mucosal graft from the nasal cavity floor including inferior meatus mucosa. This technique aims to standardize sellar reconstruction without the use of the nasoseptal flap and to keep the advantage of mucosal coverage of the defect in all cases. METHODS: Fifty consecutive patients who had endoscopic surgery for pituitary tumors and reconstruction with nasal cavity floor free mucosal graft were retrospectively reviewed. There were a total of 50 patients with postoperative follow-up from 3 to 16 months. Collagen dural graft was used inlay and free mucosal graft overlay to cover the sellar defect. No fat grafts or lumbar drains were used. A Sinonasal Outcome Test-22 (SNOT-22) was performed before, 1 and 3 months after surgery. RESULTS: There were 40% detected intraoperative leaks and no postoperative leaks. Nasal endoscopy performed at 1 month follow-up showed complete healing of the graft to the skull base and near total or complete mucosalization of the donor site. No significant difference was found in the SNOT-22 comparing the total preoperative and 1-month scores. CONCLUSIONS: The nasal cavity floor free mucosal graft is an easy and safe technique, with minimal nasal morbidity. There were no postoperative cerebrospinal fluid leaks, despite aggressive tumor resection. No lumbar drains or fat graft were used. The harvest of mucosal graft does not worsen the quality of life measured with the SNOT-22 test.