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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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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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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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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.

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David T. Fernandes Cabral, Georgios A. Zenonos, Juan C. Fernandez-Miranda, Eric W. Wang and Paul A. Gardner

OBJECTIVE

Iatrogenic tumor seeding after open surgery for chordoma has been well described in the literature. The incidence and particularities related to endoscopic endonasal surgery (EES) have not been defined.

METHODS

The authors retrospectively reviewed their experience with EES for clival chordoma, focusing on cases with iatrogenic seeding. The clinical, radiographic, pathological, and molecular characterization data were reviewed.

RESULTS

Among 173 EESs performed for clival chordomas at the authors’ institution between April 2003 and May 2016, 2 cases complicated by iatrogenic seeding (incidence 1.15%) were identified. The first case was a 10-year-old boy, who presented 21 months after an EES for a multiply recurrent clival chordoma with a recurrence along the left inferior turbinate, distinct from a right petrous apex recurrence. Both appeared as a T2-hypertintense, T1-isointense, and heterogeneously enhancing lesion on MRI. Resection of the inferior turbinate recurrence and debulking of the petrous recurrence were both performed via a purely endoscopic endonasal approach. Unfortunately, the child died 2 years later due progression of disease at the primary site, but with no sign of progression at the seeded site. The second patient was a 79-year-old man with an MRI-incompatible pacemaker who presented 19 months after EES for his clival chordoma with a mass involving the floor of the left nasal cavity that was causing an oro-antral fistula. On CT imaging, this appeared as a homogeneously contrast-enhancing mass eroding the hard palate inferiorly, the nasal septum superiorly, and the nasal process of the maxilla, with extension into the subcutaneous tissue. This was also treated endoscopically (combined transnasal-transoral approach) with resection of the mass, and repair of the fistula by using a palatal and left lateral wall rotational flap. Adjuvant hypofractionated stereotactic CyberKnife radiotherapy was administered using 35 Gy in 5 fractions. No recurrence was appreciated endoscopically or on imaging at the patient’s last follow-up, 12 months after this last procedure. In both cases, pathological investigation of the original tumors revealed a fairly aggressive biology with 1p36 deletions, and high Ki-67 levels (10%–15%, and > 20%, respectively). The procedures were performed by a team of right-handed surgeons (otolaryngology and neurosurgery), using a 4-handed technique (in which the endoscope and suction are typically passed through the right nostril, and other instruments are passed through the left nostril without visualization).

CONCLUSIONS

Although uncommon, iatrogenic seeding occurs during EES for clival chordomas, probably because of decreased visualization during tumor removal combined with mucosal trauma and exposure of subepithelial elements (either inadvertently or because of mucosal flaps). In addition, tumors with more aggressive biology (1p36 deletions, elevated Ki-67, or both) are probably at a higher risk and require increased vigilance on surveillance imaging and endoscopy. Further prospective studies are warranted to evaluate the authors’ proposed strategies for decreasing the incidence of iatrogenic seeding after EES for chordomas.

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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.

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Jose M. Pascual

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Juan C. Fernandez-Miranda, Carlos D. Pinheiro-Neto, Paul A. Gardner and Carl H. Snyderman

The authors present the technical and anatomical nuances needed to perform an endoscopic endonasal removal of a tuberculum sellae meningioma. The patient is a 47-year-old female with headaches and an incidental finding of a small tuberculum sellae meningioma with no vascular encasement, no optic canal invasion, but mild inferior to superior compression of the cisternal segment of the left optic nerve. Neuroophthalmology assessment revealed no visual defects. Treatment options included clinical observation with imaging follow-up studies, radiosurgery, and resection. The patient elected to undergo surgical removal and an endonasal endoscopic approach was the preferred surgical option.

Preoperative radiological studies showed the presence of an osseous ring between the left middle and anterior clinoids, the so-called carotico-clinoidal ring. The surgical implications of this finding and its management are illustrated. The surgical anatomy of the suprasellar region is reviewed, including concepts such as the chiasmatic sulcus and limbus sphenoidale, medial and lateral optico-carotid recesses, and the paraclinoidal and supraclinoidal segments of the internal carotid artery. Emphasis is made in the importance of exposing the distal dural ring of the internal carotid artery and the precanalicular segment of the optic nerve for adequate intradural dissection. The endonasal route allows for early coagulation of the tumor meningeal supply and extensive resection of dural attachments, and importantly, provides an inferior to superior access to the infrachiasmatic region that facilitates complete tumor removal without any manipulation of the optic nerve. The lateral limit of dural removal is formed by the distal dural ring, which is gently coagulated after the tumor is resected. A 45° scope is used to inspect for any residual tumor, in particular at the entrance of the optic nerve into the optic canal and at the most anterior margin of the exposure (limbus sphenoidale). The steps for reconstruction are detailed and include intradural placement of dural substitute and extradural placement of the nasoseptal flap. The nuances for proper harvesting, positioning, and reinforcement of the flap are described. No lumbar drain was used.

The patient had an uneventful recovery with no CSF leak or any other complications. Imaging follow-up at 6 months showed complete removal of the tumor. The patient had no sinonasal or neurological symptoms, and olfaction was fully preserved.

The video can be found here: http://youtu.be/kkuV-yyEHMg.

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Danilo Silva, Moshe Attia and Theodore H. Schwartz