Intracranial cavernous malformation surgery

Intracranial cavernous malformation surgery

Indications

Indications for surgery for intracranial cavernous malformation:

1. accessible lesions with

a) Focal neurologic signs

b) or symptomatic hemorrhage

c) or seizures:

● new onset seizures: there is a suggestion that removing CMs before “kindling” occurs may have a better chance of preventing future seizures.

● difficult to manage seizures

2. less accessible lesions that repeatedly bleed with progressive neurologic deterioration may be considered for excision, even in delicate regions such as the brainstem 1) 2) 3).

Surgical technique

Goal of surgery: complete removal of the malformation. Since CMs are not particularly bloody, piecemeal excision is an option; especially important in brainstem lesions.

Stereotactic localization or intraoperative ultrasound may be particularly helpful in localizing. When operating on CMs that have bled, one usually encounters a cavity containing the CM and blood degradation products 4).

Initial dissection is directed at separating the lesion from the adjacent brain. Although bleeding is usually not a problem, it occasionally may be brisk if the CM is entered before the dissection and devascularization is complete. Once the dissection is complete, the contents of the CM capsule may be removed piecemeal to minimize the parenchymal opening (especially important in the brainstem). For supratentorial CMs presenting with seizures, it is desirable to also remove the hemosiderin-stained brain immediately surrounding the CM. Keep in mind the relatively common association of CMs with venous angiomas, which if encountered should not be removed as they represent the venous drainage of the area.


Eichberg et al., reviewed a single institution’s transcortical-transtubular intracranial cavernous malformation resections using either BrainPath endoport system (NICO, Indianapolis, Indiana) or ViewSite Brain Access System (Vycor Medical, Boca Raton, Florida) tubular retractors performed from 2013 to 2018 (n = 20).

Gross total resection was achieved in all patients. When a developmental venous anomaly (DVA) was present, avoidance of DVA resection was achieved in all cases (n = 4). All patients had a supratentorial cavernoma with mean depth below cortical surface of 44.1 mm. Average postoperative clinical follow-up was 20.4 wk. Early neurologic deficit rate was 10% (n = 2); permanent neurologic deficit rate was 0%. One patient (5%) experienced early postoperative seizures (< 1 wk postop). No patients experienced late seizures (> 1 wk follow-up). Engel class 1 seizure control at final clinical follow-up was achieved in 87.5% of patients presenting with preoperative epilepsy.

Tubular retractors provide a low-profile, minimally invasive operative corridor for resection of subcortical cavernomas. There were no permanent neurologic complications in the series of 20 cases, and long-term seizure control was achieved in all patients. Thus, tubular retractors appear to be a safe and efficacious tool for resection of subcortical cavernomas 5).

Controversies

An online survey composed of 61 items was sent to 26 centers to establish a multicenter international retrospective cohort of adult patients who underwent a surgical resection as the first-line treatment of a supratentorial cavernous angioma located within or close to eloquent brain area.

272 patients from 19 centers (mean 13.6 ± 16.7 per center) from eight countries were included. The pre-operative management varied significantly between centers and countries regarding the pre-operative functional assessment, the pre-operative epileptological assessment, the first given antiepileptic drug, and the time to surgery. The intra-operative environment varied significantly between centers and countries regarding the use of imaging systems, the use of functional mapping with direct electrostimulations, the extent of resection of the hemosiderin rim, the realization of a post-operative functional assessment, and the time to post-operative functional assessment. The present survey found a post-operative improvement, as compared to pre-operative evaluations, of the functional status, the ability to work, and the seizure control.

They observed a variety of practice between centers and countries regarding the management of cavernous angioma located within eloquentregions. Multicentric prospective studies are required to solve relevant questions regarding the management of cavernous angioma-related seizures, the timing of surgery, and the optimal extent of hemosiderin rim resection 6).


Meta-analysis and subgroup analyses were conducted to compare extended lesionectomy with lesionectomy. Pooled analysis demonstrated that seizure outcome was not statistically significantly improved in patients who underwent extended lesionectomy compared with lesionectomy (OR 0.77; 95% CI [0.39-1.51]; P=0.44; I2=15%).

Extended lesionectomy cannot contribute to better seizure control for CCMs with epilepsy. Resection of lesion and surrounding hemosiderin is sufficient for CCMs presenting with epilepsy 7).

References

1)

Bicknell JM. Familial Cavernous Angioma of the Brain Stem Dominantly Inherited in Hispanics. Neurosurgery. 1989; 24:102–105
2)

Ondra SL, Doty JR, Mahla ME, et al. Surgical Excision of a Cavernous Hemangioma of the Rostral Brain Stem: Case Report. Neurosurgery. 1988; 23:490–493
3)

Zimmerman RS, Spetzler RF, Lee KS, Zabramski JM, et al. Cavernous Malformations of the Brain Stem. J Neurosurg. 1991; 75:32–39
4)

Wascher TM, Spetzler RF, Carter LP, Spetzler RF, Hamilton MG. In: Cavernous malformations of the brain stem. Neurovascular Surgery. New York: McGraw -Hill; 1995:541–555
5)

Eichberg DG, Di L, Shah AH, Ivan ME, Komotar RJ, Starke RM. Use of Tubular Retractors for Minimally Invasive Resection of Deep-Seated Cavernomas. Oper Neurosurg (Hagerstown). 2019 Jul 13. pii: opz184. doi: 10.1093/ons/opz184. [Epub ahead of print] PubMed PMID: 31301143.
6)

Zanello M, Meyer B, Still M, Goodden JR, Colle H, Schichor C, Bello L, Wager M, Smits A, Rydenhag B, Tate M, Metellus P, Hamer PW, Spena G, Capelle L, Mandonnet E, Robles SG, Sarubbo S, Martino González J, Fontaine D, Reyns N, Krieg SM, Huberfeld G, Wostrack M, Colle D, Robert E, Noens B, Muller P, Yusupov N, Rossi M, Conti Nibali M, Papagno C, Visser V, Baaijen H, Galbarritu L, Chioffi F, Bucheli C, Roux A, Dezamis E, Duffau H, Pallud J. Surgical resection of cavernous angioma located within eloquent brain areas: International survey of the practical management among 19 specialized centers. Seizure. 2019 Mar 28;69:31-40. doi: 10.1016/j.seizure.2019.03.022. [Epub ahead of print] PubMed PMID: 30959423.
7)

Shang-Guan HC, Wu ZY, Yao PS, Chen GR, Zheng SF, Kang DZ. Does extended lesionectomy need to cerebral cavernous malformations presenting with epilepsy? A meta-analysis. World Neurosurg. 2018 Sep 6. pii: S1878-8750(18)31994-6. doi: 10.1016/j.wneu.2018.08.208. [Epub ahead of print] PubMed PMID: 30196170.

Microcephaly

Microcephaly

Head circumference more than 2 standard deviations below the mean for sex and gestational age. Terms that are sometimes used synonymously: microcrania, microcephalus. Not a single entity, many conditions may be associated with microcephaly. It may also result from maternal cocaine abuse. It is important to differentiate microcephaly from a small skull resulting from craniosynostosis in which surgical treatment may provide opportunity for improved cerebral development.

Etiology

Effects of maternal cocaine use on the fetal nervous system include: microcephaly 1).

May be present in Dandy Walker malformationAgenesis of the corpus callosumHydranencephaly.

Overshunting (controversial): Microcephaly accounted for ≈ 6% of skull deformities after shunting (about half of these had sagittal synostosis). Some of these changes were reversible (except when complete synostosis was present) if intracranial hypertension recurred.

Diagnosis

Occipitofrontal circumference:

Deviations below the curves or head growth in the premature infant in the neonatal period of less than 0.5 cm/wk (excluding the first few weeks of life) may indicate microcephaly.

Associations

Holoprosencephaly with:

Cyclopia

Ethmocephaly

Cebocephaly

with median cleft lip

with median philtrum-premaxilla anlage

Shaheen et al., previously suggested that a single founder splicing variant in human CTU2 causes a multiple congenital anomalies syndrome consisting of dysmorphic facies, renal agenesis, ambiguous genitalia, microcephalypolydactyly, and lissencephaly (DREAM-PL).

They described five new patients with DREAM-PL phenotype and whose molecular analysis expands the allelic heterogeneity of the syndrome to five different alleles; four of which predict protein truncation. Functional characterization using patient-derived cells for each of these alleles, as well as the original founder allele; revealed a specific impairment of wobble uridine thiolation in all known thiol-containing tRNAs.

This data establish a recognizable CTU2-linked autosomal recessive syndrome in humans characterized by defective thiolation of the wobble uridine. The potential deleterious consequences for the translational efficiency and fidelity during development as a mechanism for pathogenicity represent an attractive target of future investigations 2).


Majewski osteodysplastic primordial dwarfism Type II (MOPD II) is a rare genetic disorder. Features of it include extremely small stature, severe microcephaly, and normal or near-normal intelligence. Previous studies have found that more than 50% of patients with MOPD II have intracranial vascular anomalies, but few successful surgical revascularization or aneurysm-clipping cases have been reported because of the diminutive arteries and narrow surgical corridors in these patients.

Teo et al., report on a large series of patients with MOPD II who underwent surgery for an intracranial vascular anomaly.

In conjunction with an approved prospective registry of patients with MOPD II, a prospectively collected institutional surgical database of children with MOPD II and intracranial vascular anomalies who underwent surgery was analyzed retrospectively to establish long-term outcomes.

Ten patients with MOPD II underwent surgery between 2005 and 2012; 5 patients had moyamoya disease (MMD), 2 had intracranial aneurysms, and 3 had both MMD and aneurysms. Patients presented with transient ischemic attack (TIA) (n = 2), ischemic stroke (n = 2), intraparenchymal hemorrhage from MMD (n = 1), and aneurysmal subarachnoid hemorrhage (n = 1), and 4 were diagnosed on screening. The mean age of the 8 patients with MMD, all of whom underwent extracranial-intracranial revascularization (14 indirect, 1 direct) was 9 years (range 1-17 years). The mean age of the 5 patients with aneurysms was 15.5 years (range 9-18 years). Two patients experienced postoperative complications (1 transient weakness after clipping, 1 femoral thrombosis that required surgical repair). During a mean follow-up of 5.9 years (range 3-10 years), 3 patients died (1 of subarachnoid hemorrhage, 1 of myocardial infarct, and 1 of respiratory failure), and 1 patient had continued TIAs. All of the surviving patients recovered to their neurological baseline.

Patients with MMD presented at a younger age than those in whom aneurysms were more prevalent. Microneurosurgery with either intracranial bypass or aneurysm clipping is extremely challenging but feasible at expert centers in patients with MOPD II, and good long-term outcomes are possible 3).

References

1)

Volpe JJ. Effect of Cocaine Use on the Fetus. N Engl J Med. 1992; 327:399–407
2)

Shaheen R, Mark P, Prevost CT, AlKindi A, Alhag A, Estwani F, Al-Sheddi T, Alobeid E, Alenazi MM, Ewida N, Ibrahim N, Hashem M, Abdulwahab F, Bryant EM, Spinelli E, Millichap J, Barnett SS, Kearney HM, Accogli A, Scala M, Capra V, Nigro V, Fu D, Alkuraya FS. Biallelic Variants in CTU2 Cause DREAM-PL Syndrome and Impair Thiolation of tRNA Wobble U34. Hum Mutat. 2019 Jul 13. doi: 10.1002/humu.23870. [Epub ahead of print] PubMed PMID: 31301155.
3)

Teo M, Johnson JN, Bell-Stephens TE, Marks MP, Do HM, Dodd RL, Bober MB, Steinberg GK. Surgical outcomes of Majewski osteodysplastic primordial dwarfism Type II with intracranial vascular anomalies. J Neurosurg Pediatr. 2016 Dec;25(6):717-723. PubMed PMID: 27611897.

Hemodilution

Hemodilution

Increase in the fluid content of blood, resulting in diminution of the concentration of formed elements.

Indications

see Triple H therapy.

Hemodilution is used to lower hematocrit.

Consider for Emergency carotid endarterectomy with acute neuro deficit.

High hemoglobin (HGB) level was associated with increased risk of postsurgical cerebral ischemiapulmonary embolism and lower-limb DVT and poor prognosis in poor grade aneurysmal subarachnoid hemorrhage patients. Preoperative hemodilution therapy might be beneficial in reducing operative complications, reducing hospital stay and improving short-term prognosis for neurological functional recovery in aSAH patients with high hemoglobin concentration, but further detailed research is needed 1).

Complications

1)

Anqi X, Ruiqi C, Yanming R, Chao Y. Elevated hemoglobin is associated with poor prognosis in Tibetans with poor-grade aneurysmal subarachnoid hemorrhage after clipping: A Retrospective Case-Control Study. Neurochirurgie. 2019 Jul 10. pii: S0028-3770(19)30186-9. doi: 10.1016/j.neuchi.2019.06.005. [Epub ahead of print] PubMed PMID: 31301387.
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