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“Like mother, like son:” hereditary anterior sacral meningocele

Case report and review of the literature

Paul A. Gardner and A. Leland Albright

✓Anterior sacral meningocele (ASM) is a relatively rare, congenital disorder. Usually it presents sporadically, but there are case reports of hereditary ASMs and evidence of a dominant mode of inheritance. In this article the authors describe a case illustrating the hereditary nature of ASM and present available literature on the disease.

The authors present the case of a 19-month-old boy in whom an ASM was diagnosed during a workup for constipation. The child's 31-year-old mother had been treated for the same condition 20 years earlier, when she had presented with back pain. These cases are discussed in the context of previous reports of similar cases.

There are several case reports in the literature in which an ASM occurred as a familial, isolated disorder (in the absence of other caudal abnormalities or syndromes). The condition is reported more commonly in women, but it is unclear whether this is a true difference in prevalence or a diagnosis or reporting bias. A review of the literature indicates an autosomal-dominant inheritance with variable penetrance and presentation.

Anterior sacral meningoceles can be hereditary. Given the potential complications of the disease if left untreated and the simplicity of screening—obtaining an abdominal radiograph and the patient's clinical history—we recommend screening of immediate family members of affected individuals. Surgical treatment is recommended if an ASM is discovered.

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Letter to the Editor. Prognostic molecular panel for skull base chordoma

Hai-Chao He and Zhihui Dai

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Management and outcomes of isolated interhemispheric subdural hematomas associated with falx syndrome

Daniel A. Tonetti, William J. Ares, David O. Okonkwo, and Paul A. Gardner

OBJECTIVE

Large interhemispheric subdural hematomas (iSDHs) causing falx syndrome are rare; therefore, a paucity of data exists regarding the outcomes of contemporary management of iSDH. There is a general consensus among neurosurgeons that large iSDHs with neurological deficits represent a particular treatment challenge with generally poor outcomes. Thus, radiological and clinical outcomes of surgical and nonsurgical management for iSDH bear further study, which is the aim of this report.

METHODS

A prospectively collected, single-institution trauma database was searched for patients with isolated traumatic iSDH causing falx syndrome in the period from January 2008 to January 2018. Information on demographic and radiological characteristics, serial neurological examinations, clinical and radiological outcomes, and posttreatment complications was collected and tallied. The authors subsequently dichotomized patients by management strategy to evaluate clinical outcome and 30-day survival.

RESULTS

Twenty-five patients (0.4% of those with intracranial injuries, 0.05% of those with trauma) with iSDH and falx syndrome represented the study cohort. The average age was 73.4 years, and most patients (23 [92%] of 25) were taking anticoagulants or antiplatelet medications. Six patients were managed nonoperatively, and 19 patients underwent craniotomy for iSDH evacuation; of the latter patients, 17 (89.5%) had improvement in or resolution of motor deficits postoperatively. There were no instances of venous infarction, reaccumulation, or infection after evacuation. In total, 9 (36%) of the 25 patients died within 30 days, including 6 (32%) of the 19 who had undergone craniotomy and 3 (50%) of the 6 who had been managed nonoperatively. Patients who died within 30 days were significantly more likely to experience in-hospital neurological deterioration prior to surgery (83% vs 15%, p = 0.0095) and to be comatose prior to surgery (100% vs 23%, p = 0.0031). The median modified Rankin Scale score of surgical patients who survived hospitalization (13 patients) was 1 at a mean follow-up of 22.1 months.

CONCLUSIONS

iSDHs associated with falx syndrome can be evacuated safely and effectively, and prompt surgical evacuation prior to neurological deterioration can improve outcomes. In this study, craniotomy for iSDH evacuation proved to be a low-risk strategy that was associated with generally good outcomes, though appropriately selected patients may fare well without evacuation.

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Minimally invasive endoscopic-assisted posterior thoracic sympathectomy

Paul A. Gardner, Pawel G. Ochalski, and John J. Moossy

Palmar hyperhidrosis is a disorder of the autonomic nervous system characterized by excessive perspiration of the palms, but may involve other body parts as well. Traditional posterior approaches have been performed less often in favor of less invasive thoracoscopic sympathectomies, which have a high success rate with low associated morbidity. However, some patients are not candidates for a transthoracic surgery and may need an alternative treatment strategy.

In situations in which a posterior approach may be necessary, the authors have developed a minimal access endoscopic-assisted dorsal sympathectomy procedure, applying minimally invasive spine muscle splitting techniques. The authors believe that the development of this technique may help to minimize surgical morbidity associated with the traditional posterior approach by reducing pain, tissue damage, and length of postoperative recovery. This paper is a report on the successful treatment of palmar hyperhidrosis using a minimally invasive posterior technique and describes the surgical approach and outcomes in 2 patients who have been treated in this manner.

Two patients underwent minimally invasive endoscopic-assisted posterior thoracic sympathectomy for hyperhidrosis. Both patients experienced relief of their symptoms after surgery with follow-up durations of 32 and 9 months and length of stays of 0.9 and 2.8 days, respectively. One patient suffered a unilateral Horner syndrome and underwent an eyelid lift. The other patient was readmitted to the hospital 2 days after discharge with atelectasis. She was obese and suffered from chronic obstructive pulmonary disease at baseline, which were reasons she opted for a posterior approach. Neither patient suffered a pneumo- or hemothorax.

Minimally invasive endoscopic-assisted posterior thoracic sympathectomy can be safely performed for relief of hyperhidrosis. The procedure has risks for the usual complications of sympathectomy. This technique may provide an alternative to thoracoscopic approaches, especially in those patients with pulmonary disease or obesity.

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Endoscopic endonasal approach for clipping of a PICA aneurysm

Ezequiel Goldschmidt, Philippe Lavigne, Carl Snyderman, and Paul A. Gardner

This video depicts the case of a 59-year-old woman that presented to the emergency department with the worst headache of her life. CT showed subarachnoid hemorrhage and digital subtraction angiogram demonstrated a right-side posterior inferior cerebellar artery (PICA) aneurysm. Given the medial and ventral position of the aneurysm, deep to the lower cranial nerves, which obviated distal control from an open approach, and the absence of an endovascular option able to reliably preserve the PICA, an endonasal approach was offered. A far medial approach was performed, and the aneurysm was successfully clipped. The patient developed a postoperative CSF leak with persistent posthemorrhagic hydrocephalus treated with reexploration and an eventual ventriculoperitoneal shunt. The patient was discharged without neurological deficits.

The video can be found here: https://youtu.be/_9hsM2CaMow.

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Hemorrhage rates after external ventricular drain placement

Clinical article

Paul A. Gardner, Johnathan Engh, Dave Atteberry, and John J. Moossy

Object

External ventricular drain (EVD) placement is one of the most common neurosurgical procedures performed. Rates and significance of hemorrhage associated with this procedure have not been well quantified.

Methods

All adults who underwent EVD placement at the University of Pittsburgh Medical Center between July 2002 and June 2003 were evaluated for catheter-associated hemorrhage. Patients without postprocedural imaging were excluded.

Results

Seventy-seven (41%) of 188 EVDs were associated with imaging evidence of hemorrhage after either placement or removal. Most of these were insignificant, punctate intraparenchymal, or trace subarachnoid hemorrhages (51.9%). Thirty-seven (19.7%) were associated with larger hemorrhages, which were divided into 3 groups according to volume of hemorrhage: 16 patients (8.5%) had < 15 ml of hemorrhage, 20 (10.6%) had hemorrhages of > 15 ml or associated intraventricular hemorrhage, and in 1 case there was a subdural hematoma that required surgical evacuation. No hemorrhages larger than punctate or trace were seen after EVD removal. Hemorrhage was associated with 44.3% of EVDs placed in an intensive care unit compared with 34.8% in EVDs placed in the operating room (p > 0.10).

Conclusions

External ventricular drain placement has a significant risk of associated hemorrhage. However, the hemorrhages are rarely large and almost never require surgical intervention. There is a favorable trend, but no significant risk reduction when EVDs are placed in the operating room rather than the intensive care unit.

Open access

Endoscopic endonasal approach for resection of odontoid process, decompression of the cervicomedullary junction spinal cord, and resection of pannus

David T. Fernandes Cabral, Ricardo J. Fernández-de Thomas, Ali Alattar, David A. Paul, Eric W. Wang, and Paul A. Gardner

Odontoid pannus is an abnormal collection of degenerative or inflammatory tissue on the C1-dens joint that can result in severe spinal cord compression myelopathy. Treatment options vary depending on severity and etiology. In cases of severe cord compression, surgical management could be either through a purely posterior approach or in combination with an anterior decompression via endoscopic endonasal approach (EEA). This case presents a 77-year-old female who underwent posterior cervical fixation for odontoid pannus causing dramatic cervical myelopathy who failed to improve over a 6-month period and required anterior transodontoid pannus resection and decompression via EEA.

The video can be found here: https://stream.cadmore.media/r10.3171/2024.1.FOCVID23176

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Iatrogenic cerebral venous sinus occlusion with flowable topical hemostatic matrix

Clinical article

Richard H. Singleton, Brian T. Jankowitz, Daniel A. Wecht, and Paul A. Gardner

Object

The use of commercially available topical hemostatic adjuncts has increased the safety profile of surgery as a whole. Cranial surgery has also benefited from the development of numerous agents designed to permit more rapid achievement of hemostasis. Flowable topical hemostatic agents applied via syringe injection are now commonly employed in many neurosurgical procedures, including cranial surgery. Intravascular use of these strongly thrombogenic agents is contraindicated, but in certain settings, inadvertent intravascular administration can occur, resulting in vascular occlusion, thrombosis, and potential dissemination. To date, there have no reports detailing the presence and incidence of this complication.

Methods

The authors conducted a retrospective review of all cranial surgeries performed at Presbyterian University Hospital by members of the University of Pittsburgh Medical Center's Department of Neurological Surgery between 2007 and 2009. Cases complicated by vascular occlusion due to inadvertent intravascular administration of flowable topical hemostatic matrix (FTHM) were identified and analyzed.

Results

Iatrogenic vascular occlusion induced by FTHM was identified in 5 (0.1%) of 3969 cranial surgery cases. None of these events occurred in 3318 supratentorial cases, whereas 5 cases of cerebral venous sinus occlusion occurred in 651 infratentorial cases (0.8%). The risk of accidental vessel occlusion was significantly associated with infratentorial surgery, and all events occurred in the transverse and/or sigmoid sinus. No episodes of inadvertent vascular occlusion occurred during endoscopic surgery. No cases of arterial occlusion were identified. Of the 5 patients with FTHM-related cerebral venous sinus occlusion, none developed long-term neurological sequelae referable to the event.

Conclusions

Inadvertent intravascular administration of FTHM is a rare complication associated with cranial surgery that occurs most commonly during infratentorial procedures around the transverse and/or sigmoid sinuses. Modifications in the choice of when to use an FTHM and the method of application may help prevent accidental venous sinus administration.

Open access

Approach selection for resection of petroclival meningioma

Christina Jackson, Kent S. Tadokoro, Eric W. Wang, Georgios A. Zenonos, Carl H. Snyderman, and Paul A. Gardner

Petroclival meningiomas are surgically challenging tumors because of their deep location and involvement of critical neurovascular structures. A variety of approaches have been described, and selection of approach should be tailored to the location of the tumor relative to neurovascular structures and surgical experience. The authors present two patients with petroclival meningiomas with varying relationships to cranial nerves and skull base anatomy who underwent endoscopic endonasal and open petrosectomy approaches, to demonstrate the complementarity of the endonasal transpetrous and open transpetrosal corridors. Proficiency in both open and endonasal approaches is critical to appropriate approach selection and maximal safe resection.

The video can be found here: https://stream.cadmore.media/r10.3171/2022.1.FOCVID21252

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Early practices in endonasal skull base surgery during the COVID-19 pandemic: a global survey

Pierre-Olivier Champagne, Michael M. McDowell, Eric W. Wang, Carl H. Snyderman, Georgios A. Zenonos, and Paul A. Gardner

OBJECTIVE

During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, endoscopic endonasal surgery (EES) is feared to be a high-risk procedure for the transmission of coronavirus disease 2019 (COVID-19). Nonetheless, data are lacking regarding the management of EES during the pandemic. The object of this study was to understand current worldwide practices pertaining to EES for skull base/pituitary tumors during the SARS-CoV-2 pandemic and provide a basis for the formulation of guidelines.

METHODS

The authors conducted a web-based survey of skull base surgeons worldwide. Different practices by geographic region and COVID-19 prevalence were analyzed.

RESULTS

One hundred thirty-five unique responses were collected. Regarding the use of personal protective equipment (PPE), North America reported using more powered air-purifying respirators (PAPRs), and Asia and Europe reported using more standard precautions. North America and Europe resorted more to reverse transcriptase–polymerase chain reaction (RT-PCR) for screening asymptomatic patients. High-prevalence countries showed a higher use of PAPRs. The medium-prevalence group reported lower RT-PCR testing for symptomatic cases, and the high-prevalence group used it significantly more in asymptomatic cases.

Nineteen respondents reported transmission of COVID-19 to healthcare personnel during EES, with a higher rate of transmission among countries classified as having a medium prevalence of COVID-19. These specific respondents (medium prevalence) also reported a lower use of airborne PPE. In the cases of healthcare transmission, the patient was reportedly asymptomatic 32% of the time.

CONCLUSIONS

This survey gives an overview of EES practices during the SARS-CoV-2 pandemic. Intensified preoperative screening, even in asymptomatic patients, RT-PCR for all symptomatic cases, and an increased use of airborne PPE is associated with decreased reports of COVID-19 transmission during EES.