Operculoinsular cortectomy

Operculoinsular cortectomy

Operculoinsular cortectomy for refractory epilepsy is a relatively safe therapeutic option but temporary neurological deficits after surgery are frequent. A study of Bouthillier et al. highlighted the role of frontal/parietal opercula resections in postoperative complications. Corona radiata ischemic lesions are not clearly related to motor deficits. There were no obvious permanent neurological consequences of losing a part of an epileptic insula, including on the dominant side for language. A low complication rate can be achieved if the following conditions are met: 1) microsurgical technique is applied to spare cortical branches of the middle cerebral artery; 2) the resection of an opercula is done only if the opercula is part of the epileptic focus; and 3) the neurosurgeon involved has proper training and experience 1).


The goal of a study of Bouthillier et al. of the Sainte-Justine University Hospital CenterMontrealQuebecCanada, was to document seizure control outcome after operculoinsular cortectomy in a group of patients investigated and treated by an epilepsy team with 20 years of experience with this specific technique.

Clinical, imaging, surgical, and seizure control outcome data of all patients who underwent surgery for refractory epilepsy requiring an operculoinsular cortectomy were retrospectively reviewed. Tumors and progressive encephalitis cases were excluded. Descriptive and uni- and multivariate analyses were done to determine seizure control outcome and predictors.

Forty-three patients with 44 operculoinsular cortectomies were studied. Kaplan-Meier estimates of complete seizure freedom (first seizure recurrence excluding auras) for years 0.5, 1, 2, and 5 were 70.2%, 70.2%, 65.0%, and 65.0%, respectively. With patients with more than 1 year of follow-up, seizure control outcome Engel class I was achieved in 76.9% (mean follow-up duration 5.8 years; range 1.25-20 years). With multivariate analysis, unfavorable seizure outcome predictors were frontal lobe-like seizure semiology, shorter duration of epilepsy, and the use of intracranial electrodes for invasive monitoring. Suspected causes of recurrent seizures were sparing of the language cortex part of the focus, subtotal resection of cortical dysplasia/polymicrogyria, bilateral epilepsy, and residual epileptic cortex with normal preoperative MRI studies (insula, frontal lobe, posterior parieto-temporal, orbitofrontal).

The surgical treatment of operculoinsular refractory epilepsy is as effective as epilepsy surgery in other brain areas. These patients should be referred to centers with appropriate experience. A frontal lobe-like seizure semiology should command more sampling with invasive monitoring. Recordings with intracranial electrodes are not always required if the noninvasive investigation is conclusive. The complete resection of the epileptic zone is crucial to achieving good seizure control outcome 2).


In 2017 Bouthillier et al. published twenty-five patients underwent an epilepsy surgery requiring an operculoinsular cortectomy: mean age at surgery was 35 y (9-51), mean duration of epilepsy was 19 y (5-36), 14 were female, and mean duration of follow-up was 4.7 y (1-16). Magnetic resonance imaging of the operculoinsular area was normal or revealed questionable nonspecific findings in 72% of cases. Investigation with intracranial EEG electrodes was done in 17 patients. Surgery was performed on the dominant side for language in 7 patients. An opercular resection was performed in all but 2 patients who only had an insulectomy. Engel class I seizure control was achieved in 80% of patients. Postoperative neurological deficits (paresis, dysphasia, alteration of taste, smell, hearing, pain, and thermal perceptions) were frequent (75%) but always transient except for 1 patient with persistent mild alteration of thermal and pain perception. 3).

References

1)

Bouthillier A, Weil AG, Martineau L, Létourneau-Guillon L, Nguyen DK. Operculoinsular cortectomy for refractory epilepsy. Part 2: Is it safe? J Neurosurg. 2019 Sep 20:1-11. doi: 10.3171/2019.6.JNS191126. [Epub ahead of print] PubMed PMID: 31597116.
2)

Bouthillier A, Weil AG, Martineau L, Létourneau-Guillon L, Nguyen DK. Operculoinsular cortectomy for refractory epilepsy. Part 1: Is it effective? J Neurosurg. 2019 Sep 20:1-10. doi: 10.3171/2019.4.JNS1912. [Epub ahead of print] PubMed PMID: 31629321.
3)

Bouthillier A, Nguyen DK. Epilepsy Surgeries Requiring an Operculoinsular Cortectomy: Operative Technique and Results. Neurosurgery. 2017 Oct 1;81(4):602-612. doi: 10.1093/neuros/nyx080. PubMed PMID: 28419327.

Unplanned hospital readmission after cranial neurosurgery

Unplanned hospital readmission after cranial neurosurgery

Many readmissions may be preventable and occur at predictable time intervals. The causes and timing of readmission vary significantly across neurosurgical subgroups. Future studies should focus on detecting specific complications in select cohorts at predefined time points, which may allow for interventions to lower costs and reduce patient morbidity 1).


Hospital readmission to a hospital (non-index) other than the one from which patients received their original care (index) has been associated with increases in both morbidity and mortality for cancer patients.

Of patient readmissions following brain tumor resection, 15.6% occur at a non-index facility. Low procedure volume is a confounder for non-index analysis and is associated with an increased likelihood of major complications and mortality, as compared to readmission to high-procedure-volume hospitals. Further studies should evaluate interventions targeting factors associated with unplanned readmission 2).


In a single-center Canadian experience. Almost one-fifth of neurosurgical patients were readmitted within 30 days of discharge. However, only about half of these patients were admitted for an unplanned reason, and only 10% of all readmissions were potentially avoidable. This study demonstrates unique challenges encountered in a publicly funded healthcare setting and supports the growing literature suggesting 30-day readmission rates may serve as an inappropriate quality of care metric in neurosurgical patients. Potentially avoidable readmissions can be predicted, and further research assessing predictors of avoidable readmissions is warranted 3).

A study of Elsamadicy et al. suggested that infection, altered mental status, and new sensory/motor deficits were the primary complications leading to unplanned 30-day readmission after cranial neurosurgery 4).


The preponderance of postdischarge mortality and complications requiring readmission highlights the importance of posthospitalization management 5).


Obstructive sleep apnea (OSA) is known to be associated with negative outcomes and is underdiagnosed. The STOP-Bang questionnaire is a screening tool for OSA that has been validated in both medical and surgical populations. Given that readmission, after surgical intervention is an undesirable event, Caplan et al. sought to investigate, among patients not previously diagnosed with OSA, the capacity of the STOP-Bang questionnaire to predict 30-day readmissions following craniotomy for a supratentorial tumor.

For patients undergoing craniotomy for treatment of a supratentorial neoplasm within a multiple-hospital academic medical center, data were captured in a prospective manner via the Neurosurgery Quality Improvement Initiative (NQII) EpiLog tool. Data were collected over a 1-year period for all supratentorial craniotomy cases. An additional criterion for study inclusion was that the patient was alive at 30 postoperative days. Statistical analysis consisted of simple logistic regression, which assessed the ability of the STOP-Bang questionnaire and additional variables to effectively predict outcomes such as 30-day readmission, 30-day emergency department (ED) visit, and 30-day reoperation. The C-statistic was used to represent the receiver operating characteristic (ROC) curve, which analyzes the discrimination of a variable or model.

Included in the sample were all admissions for supratentorial neoplasms treated with craniotomy (352 patients), 49.72% (n = 175) of which were female. The average STOP-Bang score was 1.91 ± 1.22 (range 0-7). A 1-unit higher STOP-Bang score accurately predicted 30-day readmissions (OR 1.31, p = 0.017) and 30-day ED visits (OR 1.36, p = 0.016) with fair accuracy as confirmed by the ROC curve (C-statistic 0.60-0.61). The STOP-Bang questionnaire did not correlate with 30-day reoperation (p = 0.805) or home discharge (p = 0.315).

The results of this study suggest that undiagnosed OSA, as assessed via the STOP-Bang questionnaire, is a significant predictor of patient health status and readmission risk in the brain tumor craniotomy population. Further investigations should be undertaken to apply this prediction tool in order to enhance postoperative patient care to reduce the need for unplanned readmissions 6).


Lopez Ramos et al., from the Department of Neurological Surgery, University of California San Diego, La Jolla, CA, USA, examined clinical risk factors and postoperative complications associated with 30-day unplanned hospital readmissions after cranial neurosurgery.

They queried the American College of Surgeons National Surgical Quality Improvement Program database from 2011-2016 for adult patients that underwent a cranial neurosurgical procedure. Multivariable logistic regression with backwards model selection was used to determine predictors associated with 30-day unplanned hospital readmission.

Of 40,802 cranial neurosurgical cases, 4,147 (10.2%) had an unplanned readmission. Postoperative complications were higher in the readmission cohort (18.5% vs 9.9%, p <0.001). On adjusted analysis, clinical factors predictive of unplanned readmission included hypertension, COPD, diabetes, coagulopathy, chronic steroid use, and preoperative anemia, hyponatremia, and hypoalbuminemia (all p ≤ 0.01). Higher ASA class (III-V), operative time >216 minutes, and unplanned reoperation were also associated with an increased likelihood of readmission (all p ≤0.001). Postoperative complications predictive of unplanned readmissions were wound infection (OR 4.90, p <0.001), pulmonary embolus (OR 3.94, p <0.001), myocardial infarction/cardiac arrest (OR 2.37, p <0.001), sepsis (OR 1.73, p <0.001), deep venous thrombosis (1.50, p=0.002), and urinary tract infection (OR 1.45, p=0.002). Female sex, transfer status, and postoperative pulmonary complications were protective of readmission (all p <0.05)

Unplanned hospital readmission after cranial neurosurgery is a common event. Identification of high-risk patients who undergo cranial procedures may allow hospitals to reduce unplanned readmissions and associated healthcare costs 7).


Cusimano et al., conducted a systematic review of several databases; a manual search of the Journal of NeurosurgeryNeurosurgeryActa NeurochirurgicaCanadian Journal of Neurological Sciences; and the cited references of the selected articles. Quality review was performed using the STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) criteria. Findings are reported according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines.

A total of 1344 articles published between 1947 and 2015 were identified; 25 were considered potentially eligible, of which 12 met inclusion criteria. The 30-day readmission rates varied from 6.9% to 23.89%. Complications arising during or after neurosurgical procedures were a prime reason for readmission. Race, comorbidities, and longer hospital stay put patients at risk for readmission.

Although readmission may be an important indicator for good care for the subset of acutely declining patients, neurosurgery should aim to reduce 30-day readmission rates with improved quality of care through systemic changes in the care of neurosurgical patients that promote preventive measures 8).

References

1)

Taylor BE, Youngerman BE, Goldstein H, Kabat DH, Appelboom G, Gold WE, Connolly ES Jr. Causes and Timing of Unplanned Early Readmission After Neurosurgery. Neurosurgery. 2016 Sep;79(3):356-69. doi: 10.1227/NEU.0000000000001110. PubMed PMID: 26562821.
2)

Jarvis CA, Bakhsheshian J, Ding L, Wen T, Tang AM, Yuan E, Giannotta SL, Mack WJ, Attenello FJ. Increased complication and mortality among non-index hospital readmissions after brain tumor resection is associated with low-volume readmitting hospitals. J Neurosurg. 2019 Oct 4:1-13. doi: 10.3171/2019.6.JNS183469. [Epub ahead of print] PubMed PMID: 31585421.
3)

Wilson MP, Jack AS, Nataraj A, Chow M. Thirty-day readmission rate as a surrogate marker for quality of care in neurosurgical patients: a single-center Canadian experience. J Neurosurg. 2018 Jul 1:1-7. doi: 10.3171/2018.2.JNS172962. [Epub ahead of print] PubMed PMID: 29979117.
4)

Elsamadicy AA, Sergesketter A, Adogwa O, Ongele M, Gottfried ON. Complications and 30-Day readmission rates after craniotomy/craniectomy: A single Institutional study of 243 consecutive patients. J Clin Neurosci. 2018 Jan;47:178-182. doi: 10.1016/j.jocn.2017.09.021. Epub 2017 Oct 12. PubMed PMID: 29031542.
5)

Dasenbrock HH, Yan SC, Smith TR, Valdes PA, Gormley WB, Claus EB, Dunn IF. Readmission After Craniotomy for Tumor: A National Surgical Quality Improvement Program Analysis. Neurosurgery. 2017 Apr 1;80(4):551-562. doi: 10.1093/neuros/nyw062. PubMed PMID: 28362921.
6)

Caplan IF, Glauser G, Goodrich S, Chen HI, Lucas TH, Lee JYK, McClintock SD, Malhotra NR. Undiagnosed obstructive sleep apnea as a predictor of 30-day readmission for brain tumor patients. J Neurosurg. 2019 Jul 19:1-6. doi: 10.3171/2019.4.JNS1968. [Epub ahead of print] PubMed PMID: 31323636.
7)

Lopez Ramos C, Brandel MG, Rennert RC, Wali AR, Steinberg JA, Santiago-Dieppa DR, Burton BN, Pannell JS, Olson SE, Khalessi AA. Clinical Risk Factors and Postoperative Complications Associated with Unplanned Hospital Readmissions After Cranial Neurosurgery. World Neurosurg. 2018 Jul 24. pii: S1878-8750(18)31614-0. doi: 10.1016/j.wneu.2018.07.136. [Epub ahead of print] PubMed PMID: 30053566.
8)

Cusimano MD, Pshonyak I, Lee MY, Ilie G. A systematic review of 30-day readmission after cranial neurosurgery. J Neurosurg. 2017 Aug;127(2):342-352. doi: 10.3171/2016.7.JNS152226. Epub 2016 Oct 21. PubMed PMID: 27767396.

Polymicrogyria

Polymicrogyria

Small gyri with shallow sulci.

Epidemiology

The prevalence of isolated polymicrogyria is unknown. Researchers believe that it may be relatively common overall, although the individual forms of the disorder (such as bilateral generalized polymicrogyria) are probably rare.

Classification

The mildest form is known as unilateral focal polymicrogyria. This form of the condition affects a relatively small area on one side of the brain. It may cause minor neurological problems, such as mild seizures that can be easily controlled with medication. Some people with unilateral focal polymicrogyria do not have any problems associated with the condition.

Bilateral forms of polymicrogyria tend to cause more severe neurological problems. Signs and symptoms of these conditions can include recurrent seizures (epilepsy), delayed development, crossed eyes, problems with speech and swallowing, and muscle weakness or paralysis. The most severe form of the disorder, bilateral generalized polymicrogyria, affects the entire brain. This condition causes severe intellectual disability, problems with movement, and seizures that are difficult or impossible to control with medication.

Etiology

Gene abnormalities e.g. WDR62 and PIK3R2

Intrauterine cerebral injury, after approximately 20 weeks gestation, e.g. infection such as CMV, or hypoxia-ischemia

Metabolic etiology e.g. peroxisomal disorders.

Isolated polymicrogyria can have different inheritance patterns. Several forms of the condition, including bilateral frontoparietal polymicrogyria (which is associated with mutations in the ADGRG1 gene), have an autosomal recessive pattern of inheritance. In autosomal recessive inheritance, both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Polymicrogyria can also have an autosomal dominant inheritance pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Other forms of polymicrogyria appear to have an X-linked pattern of inheritance. Genes associated with X-linked conditions are located on the X chromosome, which is one of the two sex chromosomes. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons.

Some people with polymicrogyria have relatives with the disorder, while other affected individuals have no family history of the condition. When an individual is the only affected person in his or her family, it can be difficult to determine the cause and possible inheritance pattern of the disorder.


Proteins anchored to the cell surface via glycosylphosphatidylinositol (GPI) play various key roles in the human body, particularly in development and neurogenesis. As such, many developmental disorders are caused by mutations in genes involved in the GPI biosynthesis and remodeling pathway.

Murakami et al. described ten unrelated families with biallelic mutations in PIGB, a gene that encodes phosphatidylinositol glycan class B, which transfers the third mannose to the GPI. Ten different PIGB variants were found in these individuals. Flow cytometric analysis of blood cells and fibroblasts from the affected individuals showed decreased cell surface presence of GPI-anchored proteins. Most of the affected individuals have global developmental and/or intellectual delay, all had seizures, two had polymicrogyria, and four had peripheral neuropathy. Eight children passed away before four years old. Two of them had a clinical diagnosis of DOORS syndrome (deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures), a condition that includes sensorineural deafness, shortened terminal phalanges with small finger and toenails, intellectual disability, and seizures; this condition overlaps with the severe phenotypes associated with inherited GPI deficiency. Most individuals tested showed elevated alkaline phosphatase, which is a characteristic of the inherited GPI deficiency but not DOORS syndrome. It is notable that two severely affected individuals showed 2-oxoglutaric aciduria, which can be seen in DOORS syndrome, suggesting that severe cases of inherited GPI deficiency and DOORS syndrome might share some molecular pathway disruptions 1).

Associations

Polymicrogyria most often occurs as an isolated feature, although it can occur with other brain abnormalities. It is also a feature of several genetic syndromes characterized by intellectual disability and multiple birth defects. These include 22q11.2 deletion syndrome, Adams-Oliver syndromeAicardi syndromeGalloway-Mowat syndromeJoubert syndromeZellweger syndrome.

Megalencephaly-capillary malformation syndrome (MCAP).

Schizencephaly.

Diagnosis

May be difficult to diagnose by CT/MRI, and may be confused with pachygyria.

Treatment

Polymicrogyria (PMG), although the most common brain malformation, represents a low percentage among patients operated on for epilepsy. In cases of hemispheric PMG, electrical status epilepticus during slow sleep (ESESS) may occur leading to an aggravation of the neurological condition and risk of drug resistance. In such cases, surgical treatment can be offered.

Case series

From a population of 230 children who underwent hemispherotomy for epilepsy in the Rothschild Foundation Hospital Fohlen et al. retrospectively reviewed the patients with unilateral PMG and drug-resistant electrical status epilepticus during slow sleep (ESESS) focusing on clinical charts, electrophysiological data, and post-surgical outcome.

Eighteen patients were operated on at a mean age of 7.2 years. The average age was 2 years at seizure onset and 4.4 years at diagnosis of ESESS. All the patients preoperatively had some degree of developmental delay associated with hemiparesis. During ESESS all of them evidenced a cognitive decline and eight experienced a worsening of the hemiparesis; ESESS was resistant to at least three antiepileptic drugs. The outcome of epilepsy, with a mean follow-up of 12.8 years showed that ESESS disappeared in all patients while 16 of 18 became seizure-free. Improvement of behavior and cognitive condition was observed in all.

Hemispherotomy can be helpful in patients with drug-resistant ESESS and hemispheric PMG while keeping in mind that more often an accurate medical treatment can be sufficient. The main benefit of surgery is to definitively stop the seizures and to withdraw the medical treatment while keeping in mind the risk of motor aggravation 2).

References

1)

Murakami Y, Nguyen TTM, Baratang N, Raju PK, Knaus A, Ellard S, Jones G, Lace B, Rousseau J, Ajeawung NF, Kamei A, Minase G, Akasaka M, Araya N, Koshimizu E, van den Ende J, Erger F, Altmüller J, Krumina Z, Strautmanis J, Inashkina I, Stavusis J, El-Gharbawy A, Sebastian J, Puri RD, Kulshrestha S, Verma IC, Maier EM, Haack TB, Israni A, Baptista J, Gunning A, Rosenfeld JA, Liu P, Joosten M, Rocha ME, Hashem MO, Aldhalaan HM, Alkuraya FS, Miyatake S, Matsumoto N, Krawitz PM, Rossignol E, Kinoshita T, Campeau PM. Mutations in PIGB Cause an Inherited GPI Biosynthesis Defect with an Axonal Neuropathy and Metabolic Abnormality in Severe Cases. Am J Hum Genet. 2019 Aug 1;105(2):384-394. doi: 10.1016/j.ajhg.2019.05.019. Epub 2019 Jun 27. PubMed PMID: 31256876.
2)

Fohlen M, Dorfmüller G, Ferrand-Sorbets S, Dorison N, Chipaux M, Taussig D. Parasagittal hemispherotomy in hemispheric polymicrogyria with electrical status epilepticus during slow sleep: Indications, results and follow-up. Seizure. 2019 Jul 23;71:190-200. doi: 10.1016/j.seizure.2019.07.017. [Epub ahead of print] PubMed PMID: 31386962.
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