Jay D. Turner, Charles A. Sansur, Eric A. Potts, and Adam S. Kanter
Oluwatoyin Akinnusotu, Albert M. Isaacs, Michelle Stone, and Christopher M. Bonfield
OBJECTIVE
Pediatric achondroplasia is often associated with conditions requiring neurosurgical intervention, including CSF diversion and multilevel spinal decompression. However, there is a lack of clinical guidelines and reliable estimates of the benefits and risks of these interventions. This study aimed to summarize the literature on the neurosurgical management of pediatric achondroplasia patients in order to aid in determining optimal treatment and standardization of care.
METHODS
A systematic review of peer-reviewed studies with an objective diagnosis of achondroplasia, patient demographic information, and available data on neurosurgical interventions performed before 18 years of age for cervicomedullary compression, spinal stenosis, and hydrocephalus was performed. Study quality and risks of bias were assessed using standardized scores. Independent patient data on surgical indications, outcomes, reoperations, and complication risks were aggregated using means and percentages.
RESULTS
Of 204 records, 25 studies with 287 pediatric achondroplasia patients (mean age 25 ± 36 months) treated for cervicomedullary compression (n = 153), spinal stenosis (n = 100), and obstructive hydrocephalus (n = 34) were evaluated. Symptomatic cervicomedullary compression occurred early in life (mean age 31 ± 25 months), with apnea (48%), T2-weighted MRI cord signal (28%), myelopathy (27%), and delayed motor skills (15%) requiring foramen magnum decompression observed in 99% of patients, as well as cervical laminectomy in 65% of patients. Although 91% of treated patients had resolution of symptoms, 2% mortality, 9% reoperation, and 21% complication rates were reported. Spinal stenosis was treated in relatively older children (mean age 13 ± 3 years) with laminectomy (23%), as well as with instrumented fusion (73%) for neurogenic claudication (59%), back pain (15%), and sciatica (8%). Although 95% of patients had symptom resolution after surgery, 17% reported complications and 18% required reoperation. Of the hydrocephalus patients (mean age 56 ± 103 months), half were treated with endoscopic third ventriculostomy (ETV) and half had a shunt placed for progressive ventriculomegaly (66%), headaches (32%), and delayed cognitive development (4%). The shunted patients had a 3% mortality rate and an average of 1.5 shunt revisions per patient. None of the patients who underwent ETV as the primary procedure required a revision.
CONCLUSIONS
Neurosurgical intervention for pediatric achondroplasia conditions, including cervicomedullary compression, spinal stenosis, and hydrocephalus, is associated with high recovery rates and good outcomes. However, complications and reoperations are common. Further studies with follow-up into adulthood are needed to evaluate the long-term outcomes.
Arjun Ganga, Owen P. Leary, Aayush Setty, Kevin Xi, Albert E. Telfeian, Adetokunbo A. Oyelese, Tianyi Niu, Joaquin Q. Camara-Quintana, Ziya L. Gokaslan, Patricia Zadnik Sullivan, and Jared S. Fridley
OBJECTIVE
Lumbar facet cysts (LFCs) can cause neurological dysfunction and intractable pain. Surgery is the current standard of care for patients in whom conservative therapy fails, those with neurological deficits, and those with evidence of spinal instability. No study to date has comprehensively examined surgical outcomes comparing the multiple surgical treatment options for LFCs. Therefore, the authors aimed to perform a combined analysis of cases both in the literature and of patients at a single institution to compare the outcomes of various surgical treatment options for LFC.
METHODS
The authors performed a literature review in accordance with PRISMA guidelines and meta-analysis of the PubMed, Embase, and Cochrane Library databases and reviewed all studies from database inception published until February 3, 2023. Studies that did not contain 3 or more cases, clearly specify follow-up durations longer than 6 months, or present new cases were excluded. Bias was evaluated using Cochrane Collaboration’s Risk of Bias in Nonrandomised Studies–of Interventions (ROBINS-I). The authors also reviewed their own local institutional case series from 2015 to 2020. Primary outcomes were same-level cyst recurrence, same-level revision surgery, and perioperative complications. ANOVA, common and random-effects modeling, and Wald testing were used to compare treatment groups.
RESULTS
A total of 1251 patients were identified from both the published literature (29 articles, n = 1143) and the authors’ institution (n = 108). Patients were sorted into 5 treatment groups: open cyst resection (OCR; n = 720), tubular cyst resection (TCR; n = 166), cyst resection with arthrodesis (CRA; n = 165), endoscopic cyst resection (ECR; n = 113), and percutaneous cyst rupture (PCR; n = 87), with OCR being the analysis reference group. The PCR group had significantly lower complication rates (p = 0.004), higher recurrence rates (p < 0.001), and higher revision surgery rates (p = 0.001) compared with the OCR group. Patients receiving TCR (3.01%, p = 0.021) and CRA (0.0%, p < 0.001) had significantly lower recurrence rates compared with those undergoing OCR (6.36%). The CRA group (6.67%) also had significantly lower rates of revision surgery compared with the OCR group (11.3%, p = 0.037).
CONCLUSIONS
While PCR is less invasive, it may have high rates of same-level recurrence and revision surgery. Recurrence and revision rates for modalities such as ECR were not significantly different from those of OCR. While concomitant arthrodesis is more invasive, it might lead to lower recurrence rates and lower rates of subsequent revision surgery. Given the limitations of our case series and literature review, prospective, randomized studies are needed.
Renqing Zhu, Qing Wang, Yuanfeng Zhou, Wei Shi, Yi Zhang, Min Wang, Hao Li, and Rui Zhao
OBJECTIVE
Hematological consequences of novel antiseizure medications (ASMs) or combined therapies are rarely reported, especially in pediatric patients undergoing surgery for epilepsy. This study aimed to assess the prevalence and risk factors of coagulation dysfunction in this population and evaluate their relationship with intra- and postoperative bleeding.
METHODS
Three hundred ninety children who underwent surgery for epilepsy and 104 children without epilepsy who underwent nonepilepsy surgery at the authors’ center were included in the study. The authors retrospectively collected and analyzed the following clinical data: sex, age, weight, course of epilepsy, antiseizure therapy, first laboratory data after admission, and transfusion-related data.
RESULTS
ASMs were responsible for the higher incidence of coagulation dysfunction in pediatric epilepsy surgery patients. Low body weight (OR 0.95, 95% CI 0.92–0.98) and valproic acid (VPA) therapy (OR 5.13, 95% CI 3.25–8.22) were the most relevant factors leading to coagulation dysfunction. The most common hematological side effects of VPA were thrombocytopenia and hypofibrinogenemia, whereas low body weight was only associated with hypofibrinogenemia. Both VPA and low body weight increased the need for intra- or postoperative transfusion (p < 0.001).
CONCLUSIONS
Pediatric epilepsy surgery patients often take multiple ASMs, resulting in an increased incidence of coagulopathy. VPA levels and low body weight were found to be the main influential factors associated with an increased risk of coagulation dysfunction. Platelet and fibrinogen levels were the main indices that were affected. Both VPA and low body weight were relevant to additional surgery-related transfusion, necessitating the need for increased awareness of preoperative coagulopathy before pediatric epilepsy surgery.
Clinical trial registration no.: NCT05675254 (ClinicalTrials.gov)
Omer Doron, Devon B. O’Donnell, Colin Dallimore, Hamza Khilji, Jacob D. Greisman, Michelle Villagran, Rafael A. Ortiz, Erez Nossek, Jason A. Ellis, and David J. Langer
OBJECTIVE
Direct cerebral revascularization is considered as one of the most technically challenging operations in neurosurgery. Technical errors are often not identified during the case, but only after the recirculation stage, making management crucial at that time of the procedure. In this study, the authors sought to describe troubleshooting of the technical errors encountered in initially failed bypass cases.
METHODS
A retrospective analysis describing a single-surgeon, single-institution experience between 2014 and 2021 was performed, based on operative reports and videos, including a 30-day follow-up period. Initially failed bypass was defined if the bypass was not patent or had a significant leak after recirculation, irrespective of the final result.
RESULTS
One hundred thirty-eight bypass cases were reviewed for complex aneurysms (n = 49), moyamoya disease (n = 59), and atherosclerosis (n = 30). Fifty-one initially failed anastomoses were identified; 43 of these were the result of a technical error. Etiologies of these failed anastomoses included a clot (n = 14), vessel kinking (n = 4), spasm (n = 5), suture-related cause (n = 5), inappropriate donor or recipient (n = 3), or lack of demand (n = 8). A major leak was attributed to an uncoagulated side branch (n = 4), vessel injury due to suture/clip placement (n = 1), or inadequate suture line coverage (n = 7). Thirty-seven (86%) of 43 cases were troubleshot successfully, as salvage maneuvers included papaverine vessel massage, donor repositioning, re-anastomosis for occlusion in select cases, local hemostatic agents, and suturing or coagulating side branches in a leak. Thirty-day follow-up revealed similar rates of patency between successfully troubleshot patients (35/37) and the rest of the cases (80/87, p = 0.6).
CONCLUSIONS
Three major patterns of a noncompatible bypass were found: a major leak, an acute occlusion, or a delayed occlusion. Based on the authors’ experience, salvage strategies proved successful, showing an eventual high patency rate. The authors suggest a gradual, structured algorithm to address this stage in surgery that may contribute specifically to cerebrovascular neurosurgeons at the beginning of their careers.
Rufei Zhang, Zongmeng Wang, Hui Zheng, Xiaodan Chen, Hu Chen, Yang Song, Zijian Liu, Yunjing Xue, and Lin Lin
OBJECTIVE
The general trend in meningioma treatment is shifting from surgery to active surveillance. However, the natural history of meningioma still needs to be clarified, and a simple, practical method is needed to identify fast-growing tumors. The authors aimed to determine whether diffusion-weighted imaging (DWI) could be a valuable imaging modality for predicting meningioma growth.
METHODS
Consecutive asymptomatic patients with a meningioma diagnosed on MRI and followed up at the authors’ institution between July 2011 and July 2019 were eligible for inclusion in this retrospective study. Univariable and multivariable Cox regression analyses were used to explore whether the relative apparent diffusion coefficient (rADC) was an independent predictor of meningioma growth. Correlations between tumor growth rate (TGR), tumor volume doubling time (VDT), Ki-67, and rADC were assessed using the Pearson correlation coefficient. The predictive ability of rADC was evaluated using receiver operating characteristic (ROC) curves and validated with internal validation data.
RESULTS
Sixty-four patients (47 females, 17 males) with a mean age of 62.2 ± 1.4 years were included in this study. Univariable and multivariable analyses revealed that rADC was an independent predictor of meningioma growth (p < 0.05). ROC curve analysis showed that baseline rADC had good predictive power for growing meningiomas (AUC = 0.88, 95% CI 0.78–0.96), as well as slow- or fast-growing meningiomas (AUC = 0.83, 95% CI 0.59–0.98). Moreover, rADC still had a good ability to discriminate between growing and nongrowing meningiomas in the validation set (AUC = 0.85, 95% CI 0.64–1.00). In the 20 patients with tumor growth, baseline rADC was moderately negatively correlated with TGR (r = −0.50, p = 0.02) and strongly positively correlated with VDT (r = 0.63, p = 0.003). Moreover, Ki-67 was significantly associated with rADC in 8 patients who had undergone surgery (r = −0.75, p = 0.03).
CONCLUSIONS
In asymptomatic meningiomas, the lower the rADC at baseline, the faster the TGR and the shorter the VDT. DWI could be a valuable tool in predicting meningioma growth in asymptomatic patients.
Fernando Guedes, Vinícius M. Henriques, Francisco L. Torrão, Neder P. Haikal, Gabriel E. Sanches, Daniel A. N. Barbosa, Felipe G. Marsicano, Livia A. N. Rosa, Ana C. Siquara, and Martijn J. A. Malessy
OBJECTIVE
Biopsies of peripheral nerve tumors (PNTs) are often used to plan an efficient treatment strategy. However, performing a biopsy is controversial when the mass is likely to be a benign PNT (BPNT). The aim of this study was to evaluate the side effects of biopsies in patients with potential PNTs.
METHODS
A retrospective and cross-sectional study was conducted on 24 patients who underwent biopsy of a mass of unknown origin potentially originating from a peripheral nerve (MUOPON), performed in nonspecialty services, and who were later referred to the authors’ service for resection of their lesion between January 2005 and December 2022. The patients were evaluated for pain score, presence of a motor or sensory deficit, biopsy diagnosis, and definitive histopathological postsurgical diagnosis.
RESULTS
The location of the tumor was supraclavicular in 7 (29.2%) patients, in the axillary region in 3 (12.5%), in the upper limb in 7 (29.2%), and in the lower limb in 7 (29.2%). Twenty-one (87.5%) patients were evaluated by MRI before biopsy, and 3 (12.5%) underwent ultrasound. One patient did not have an examination before the procedure. Based on the biopsy findings, 12 (50%) analyses had an inconclusive histopathological result. The preexisting pain worsened, as measured 1 week after biopsy, in all patients and had remained unchanged at the first evaluation by the authors (median 3 months, range 2–4 months). In 1 case, the open biopsy had to be interrupted because the patient experienced excruciating pain. Four (16.7%) patients developed motor deficits. Subsequent surgery was hampered by scar formation and intratumoral hemorrhage in 5 (20.8%) patients. The initial diagnosis obtained by biopsy differed from the final histopathological diagnosis in all patients, of whom 21 (87.5%) had BPNTs, 2 (8.3%) malignant peripheral nerve sheath tumors, and 1 (4.2%) an ancient schwannoma.
CONCLUSIONS
Biopsies of PNTs are controversial and may result in misdiagnosis, neuropathic pain, or neurological deficit due to axonal damage, and they may also hinder microsurgical resection when if performed when not indicated. Indications for biopsy of an MUOPON must be carefully considered, especially if BPNT is a possible diagnosis.
Landon D. Ehlers, Patrick J. Opperman, Jack E. Mordeson, Jonathan R. Thompson, and Daniel L. Surdell
BACKGROUND
Pedicle screw impingement on vessel walls has the potential for complications due to pulsatile effects and wall erosion. Artifacts from spinal instrumentation create difficulty in accurately evaluating this interface. The authors present the first case of intravascular ultrasound (IVUS) used to characterize a pedicle screw breach into the aortic lumen.
OBSERVATIONS
A 21-year-old female with surgically corrected scoliosis underwent computed tomography angiography (CTA) 3 years postoperatively, which revealed a pedicle screw within the thoracic aorta lumen. Metal artifact distorted the CTA images, which prompted the decision to use intraoperative IVUS. The IVUS confirmed the noninvasive imaging findings and guided final decisions regarding aortic endograft size and location during spine hardware revision.
LESSONS
For asymptomatic patients presenting with pedicle screws malpositioned in or near the aorta, treatment decisions revolve around the extent of vessel wall penetration. Intraluminal depth can be obscured by artifact on computed tomography or magnetic resonance imaging or inadequately evaluated by a transesophageal echocardiogram. In our intraoperative experience, IVUS confirmed the depth of vessel lumen violation by a single pedicle screw and no wall penetration by two additional screws of concern. This was useful in deciding on thoracic endovascular aortic repair graft size and landing zone and facilitated safe spinal instrumentation removal and revision.
Hironori Yamada, Ryokichi Yagi, Akihiro Kambara, Yoshihide Katayama, Yuichiro Tsuji, Ryo Hiramatsu, Naokado Ikeda, Masahiro Kameda, Naosuke Nonoguchi, Motomasa Furuse, Shinji Kawabata, Toshihiro Takami, and Masahiko Wanibuchi
BACKGROUND
The authors report a case of occult neurohypophyseal germinoma detected in a patient with long-term diabetes insipidus. Central diabetes insipidus is the initial symptom in 95% of cases of neurohypophyseal germinoma. In occult neurohypophyseal germinomas, no abnormalities are seen on magnetic resonance imaging (MRI) at the onset of symptoms. It can take several months or even years for these changes to be detected on MRI.
OBSERVATIONS
A 20-year-old male was diagnosed with central diabetes insipidus at the age of 17 years, and gonadal and adrenal corticosteroid insufficiency was noted at the age of 19 years. Head MRI showed an enlarged and enhanced pituitary stalk. He was referred to our department for a suspected neoplastic lesion. Endoscopic transsphenoidal biopsy indicated a pure germinoma. He was treated with chemotherapy and radiotherapy and then was discharged.
LESSONS
In this case, new imaging findings appeared 19 months after the onset of diabetes insipidus, and the pathological diagnosis was made after almost 24 months. Because the patient had a history of growth hormone deficiency and had a positive test result for diabetes insipidus, occult neurohypophyseal germinoma was suspected, and periodic contrast-enhanced MRI monitoring was deemed essential.
Demitre Serletis, Juan Bulacio, Justin Bingaman, Elham Abushanab, Stephen P. Harasimchuk, Richard Rammo, Silvia Neme-Mercante, and William Bingaman
BACKGROUND
Radiofrequency thermocoagulation (RFTC) during intracranial stereoelectroencephalography (sEEG) was first described as a safe technique for creating lesions of epileptic foci in 2004. Since that time, the method has been applied as a diagnostic and/or palliative intervention. Although widely practiced in European epilepsy surgical programs, the technique has not been popularized in the United States given the lack of Food and Drug Administration (FDA)–approved technologies permitting safe usage of in situ sEEG electrodes for this purpose.
OBSERVATIONS
The authors present a case report of a young female patient with refractory left neocortical temporal lobe epilepsy undergoing sEEG electrode implantation, who underwent sEEG-guided RFTC via a stereotactic temperature-sensing pallidotomy probe. Although used as a diagnostic step in her workup, the patient has remained seizure-free for nearly 18 months.
LESSONS
The use of in situ sEEG electrodes for RFTC remains limited in the United States. In this context, this case highlights a safe alternative and temporizing approach to performing diagnostic sEEG-guided RFTC, using a temperature-sensing pallidotomy probe to create small, precise stereotactic lesions. The authors caution careful consideration of this technique as a temporary work-around solution while also highlighting the rising need for new FDA-approved technologies for safe RFTC through in situ temperature-sensing sEEG electrodes.
