Book: Complex Surgical Cases of the Limbic System

Complex Surgical Cases of the Limbic System
By Sepehr Sani

Complex Surgical Cases of the Limbic System

List Price: $90.00
The limbic system (also known as the paleomammalian brain) is a collection of brain structures located in the middle of the brain. It is not a discrete system itself but rather a collection of structures-anatomically related but varying greatly in function. The limbic system is the centre for emotional responsiveness, motivation, memory formation and integration, olfaction, and the mechanisms to keep ourselves safe ( This book is a guide to surgical procedures for the limbic system. Beginning with an overview of brain embryology and anatomy, each of the following sections covers surgical approaches for disorders in different parts of the limbic system. Procedures are explained in a step by step approach, with emphasis on anatomical markers and avoidance of complications. The final chapters discuss brain mapping during surgery, giant and unusual tumours, and vascular lesions. Authored by a team of highly experienced, Illinois and Wisconsin-based neurosurgeons, the book is enhanced by anatomical dissections, operative photographs and illustrations, and includes a DVD ROM demonstrating surgical procedures. Key points * Guide to surgical procedures for the limbic system * Step by step approach with emphasis on anatomical markers and avoidance of complications * Highly experienced, Illinois and Wisconsin-based author team * Includes DVD ROM demonstrating surgical procedures

Product Details

  • Original language: English
  • Dimensions: 9.50″ h x .0″ w x 6.30″ l,
  • Binding: Hardcover
  • 180 pages

Editorial Reviews

About the Author
Sepehr Sani MD Assistant Professor, Department of Neurosurgery, Rush University Medical Centre, Chicago, IL, USA Mustafa K Baskaya MD Department of Neurological Surgery, University of Wisconsin-Madison School of Medicine, Madison, WI, USA Richard W Byrne MD Chairman, Department of Neurosurgery, Rush University Medical Centre, Chicago, IL, USA

Update: A1 segment hypoplasia

Anterior cerebral artery A1 segment hypoplasia is an uncommon fetal variant of the circle of Willis.

There is an association of unilaterally absent or hypoplastic A1 segments of the anterior cerebral artery with ipsilateral decrease in internal carotid artery (ICA) caliber, and this can be seen on MR angiograms 1).


Hypoplasia of the proximal (A1) segment of the anterior cerebral artery (ACA) was examined in 50 human brains and on 8 selected carotid arteriograms. Hypoplasia has been found in 22% of the cases. Two types of this phenomenon exist, namely the mild and extreme hypoplasia. Mild hypoplasia has been noticed in 14% of the specimens. The hypoplastic vessel has ranged from 1.3 to 1.9 mm in diameter (average 1.6 mm) and it was from 0.6 to 0.9 mm smaller than the opposite A1 portion. Extremely hypoplastic proximal segment has been present in 8% of the cases. It has varied from 0.3 to 1.1 mm in size (average 0.9 mm) and has been more than 1 mm smaller than the opposite proximal segment. Both mild and extreme hypoplasia have been associated in 81.8% of the cases with the corresponding variations or malformations of the anterior cerebral, posterior cerebral, posterior communicating and basilar arteries 2).
The incidence of right-sided A1 segment hypoplasia either accompanied with AcomA aneurysm or not was much greater than that of left-sided. Intracranial AcomA aneurysm development appeared to be associated with A1 segment hypoplasia 3).


The A1 segment of the anterior cerebral artery is a principal supplier of anterior collateral blood flow.
The aim of a study was to determine whether A1 segment hypoplasia may be responsible for acute ischemic stroke.
Chuang et al. consecutively examined 280 acute ischemic stroke patients (aged 66.9 +/- 14.2 years). Cerebral magnetic resonance angiography was performed within 72 h of ischemic stroke onset. The overall incidence of A1 variation in our experimental group was 15.0% (n = 42, agenesis/hypoplasia = 18/24), which was statistically higher than in the control group (n = 12). The majority (n = 30, 71.42%) had ipsilateral striatal lacunar infarctions. Based on these results, A1 agenesis/hypoplasia appears to be a risk factor contributing to ischemic stroke, especially to strokes in arteries penetrating the striatal area 4).
Yamaguchi et al. report two cases of bilateral anterior cerebral artery (ACA) territory infarction. On magnetic resonance (MR) angiograms, the A1 segment of the ACA was unilaterally hypoplastic in both cases, suggesting that unilateral hypoplasia of A1 is a significant predisposing factor for this rare type of cerebral infarction. When the contralateral A1 is dominant, embolic materials may enter into it more easily 5).

Hypoplasia of the A1 segment of the anterior cerebral artery is frequently observed in patients with anterior communicating artery aneurysms. The effect of this anatomical variant on ACoA aneurysm morphology is not well understood 6).

Case series


Yang et al. retrospectively reviewed 251 patients with ACoA aneurysm who underwent surgical clipping in Beijing Tiantan Hospital between September 2011 and September 2016. Their clinical and radiologic features, as well as clinical outcomes were reviewed. In addition, univariate and multivariate logistic regression analysis was performed to identify independent risk factors for the postoperative infarction and unfavorable clinical outcomes of surgical clipping ACoA aneurysm.
The incidence of A1 segment hypoplasia was 49.8% (125 of 251 patients). Univariate analysis revealed that multiple aneurysm (P=0.025), diameter of aneurysm (P=0.040) and A1 segment hypoplasia (P=0.010) were associated with anterior cerebral artery (ACA) territories infarction, and A1 segment hypoplasia (P=0.002) is significantly correlated with unfavorable clinical outcomes of surgical clipping ACoA aneurysm. Moreover, multivariate analysis showed that multiple aneurysm (P=0.038, OR=2.571), diameter of aneurysm (P=0.034, OR=1.097) and A1 segment hypoplasia (P=0.007, OR=3.619) were strongly independent risk factors for ACA territories infarction. In addition, Hunt and Hess scores (HH) (P=0.036, OR=2.326) and A1 segment hypoplasia (P=0.002, OR=2.873) are significant independent risk factors for unfavorable clinical outcomes of surgical clipping ACoA aneurysm.
A1 segment hypoplasia is a significant independent risk factor for unfavorable clinical outcomes of surgical clipping ACoA aneurysm and ACA infarction after surgery 7).

Case reports


A case of anterior cerebral artery A1 segment hypoplasia syndrome presenting with right lower limb monoplegia, abulia, and urinary incontinence8).


A 68-year-old man presented with a Hunt and Hess Grade II subarachnoid hemorrhage and symptoms of headache, nuchal rigidity, and facial paresis. : Angiographic evaluation with superselective exploration revealed a small ruptured aneurysm located on a duplicated hypoplastic A1 segment of the left anterior cerebral artery with associated middle cerebral artery stenosis and secondary early moyamoya changes. Surgical clipping of the aneurysm was performed successfully while sparing the hypoplastic A1 segment.
A1 aneurysms occurring on a duplicated anterior cerebral artery segment probably develop from a congenital weakness of the parent vessel and increased local shear stress. Superselective angiography was helpful in the preoperative planning and facilitated the decision to treat with surgical clipping instead of embolization 9).

Kane AG, Dillon WP, Barkovich AJ, Norman D, Dowd CF, Kane TT. Reduced caliber of the internal carotid artery: a normal finding with ipsilateral absence or hypoplasia of the A1 segment. AJNR Am J Neuroradiol. 1996 Aug;17(7):1295-301. PubMed PMID: 8871715.

Marinković S, Kovacević M, Milisavljević M. Hypoplasia of the proximal segment of the anterior cerebral artery. Anat Anz. 1989;168(2):145-54. PubMed PMID: 2712326.

Chen H, Li MH. A1 segment hypoplasia accompanied by AcomA aneurysms assessed with magnetic resonance angiography. Surg Radiol Anat. 2014 May;36(4):353-7. doi: 10.1007/s00276-013-1182-5. Epub 2013 Aug 4. PubMed PMID: 23912562.

Chuang YM, Liu CY, Pan PJ, Lin CP. Anterior cerebral artery A1 segment hypoplasia may contribute to A1 hypoplasia syndrome. Eur Neurol. 2007;57(4):208-11. Epub 2007 Jan 19. PubMed PMID: 17268201.

Yamaguchi K, Uchino A, Sawada A, Takase Y, Kuroda Y, Kudo S. Bilateral anterior cerebral artery territory infarction associated with unilateral hypoplasia of the A1 segment: report of two cases. Radiat Med. 2004 Nov-Dec;22(6):422-5. PubMed PMID: 15648459.

Rinaldo L, McCutcheon BA, Murphy M, Bydon M, Rabinstein AA, Lanzino G. 360 Relationship of A1 Segment Hypoplasia to Anterior Communicating Artery Aneurysm Morphology and Risk Factors for Rupture. Neurosurgery. 2016 Aug;63 Suppl 1:207. doi: 10.1227/01.neu.0000489849.55193.67. PubMed PMID: 27399558.

Yang F, Li H, Wu J, Li M, Chen X, Jiang P, Li Z, Cao Y, Wang S. Relationship of A1 segment hypoplasia to the radiological and clinical outcomes of surgical clipping anterior communicating artery aneurysm. World Neurosurg. 2017 Jul 29. pii: S1878-8750(17)31222-6. doi: 10.1016/j.wneu.2017.07.122. [Epub ahead of print] PubMed PMID: 28765030.

Lakhotia M, Pahadiya HR, Prajapati GR, Choudhary A, Gandhi R, Jangid H. A case of anterior cerebral artery A1 segment hypoplasia syndrome presenting with right lower limb monoplegia, abulia, and urinary incontinence. J Neurosci Rural Pract. 2016 Jan-Mar;7(1):189-91. doi: 10.4103/0976-3147.168438. PubMed PMID: 26933381; PubMed Central PMCID: PMC4750332.

Taylor R, Connolly ES Jr, Duong H. Radiographic evidence and surgical confirmation of a saccular aneurysm on a hypoplastic duplicated A1 segment of the anterior cerebral artery: case report. Neurosurgery. 2000 Feb;46(2):482-4. PubMed PMID: 10690739.

Update: Maffucci syndrome

Ollier disease and Maffucci syndrome are non-hereditary skeletal disorders characterized by multiple enchondromas (Ollier disease) combined with spindle cell hemangiomas (Maffucci syndrome).

Additionally, neuroendocrine tumors including pituitary adenomas have been described in patients with Maffucci syndrome.
The rate of malignant transformation in Maffucci syndrome is high, with enchondromas transforming into chondrosarcomas and the development of secondary neoplasms, including pancreatic and hepatic adenocarcinoma, mesenchymal ovarian tumors, and brain tumors such as glioma. However, hematopoietic malignancies arising in Maffucci syndrome are rare 1)


The underlying genetic etiology lies in somatic mosaicism of mutations in isocitrate dehydrogenase 1 (IDH1) or isocitrate dehydrogenase 2 (IDH2)2).

Case series


Cerebral MRI was routinely performed in Ollier-Maffucci patients followed-up in tertiary centers. Patients with previous history of skull base or intracranial tumors were excluded from the study. Clinical and radiological datas were retrospectively collected. The occurrence rate and nature of abnormal cerebral MRIs were determined.
Twelve patients were included. A glioma-looking lesion was found in one patient (8%), while skull base lesions were evidenced in 3 others (25%). A regular MRI follow-up was recommended for each patient, with a time interval varying between 1year and 3years depending on the likelihood of tumoral evolutivity, as infered from the MRI findings.
All in all, the high rate of intracranial and skull base lesions with a malignant potential warrants to include cerebral MRI in the routine follow-up of Ollier-Maffucci patients 3).

Case report


A report describes a patient with Maffucci syndrome who presented with skull base tumors and suprasellar region. The patient underwent resection of both intracranial tumors, revealing histopathological diagnoses of chondrosarcoma and pituitary adenoma. DNA sequencing of the tumors was performed to identify common IDH1/2 mutations. Clinical, radiological, and biochemical assessments were performed. Genotypic studies used standard Sanger sequencing in conjunction with a target-specific peptide nucleic acid to detect IDH1 mutations in tumor tissues. DNA sequencing demonstrated identical IDH1 mutations (c.394C > T) in both tumors.
This report provides the first genetic evidence for the inclusion of pituitary adenomas among tumors characterizing Maffucci syndrome. In patients who are newly diagnosed with Maffucci syndrome, it is appropriate to monitor for development of pituitary pathology and neuroendocrine dysfunction 4).

A 39-year-old woman who was diagnosed with Maffucci syndrome together with intrahepatic cholangiocarcinoma (IHCC). Heterozygous somatic mutations in the isocitrate dehydrogenase 1 and 2 (IDH1/IDH2) genes are associated with a number of different tumor types (e.g. IHCC) and also with Maffucci syndrome. For IHCC, mutations in IDH1/IDH2 are associated with higher survival rates. IHCC tissue as well as normal liver tissue and peripheral blood were analyzed for IDH1/IDH2-mutations in our patient. In the tumor sample, we identified a recurrent somatic IDH1-mutation affecting Arg132, while in normal liver tissue and peripheral blood, no variants were detected, as expected.
This case report presents the second patient in the literature exhibiting the features of Maffucci syndrome along with cholangiocarcinoma. This supports the hypothesis that IDH1/2-mutations, which can be present in different types of tumor tissue simultaneously, arise during embryonic development in a mosaic pattern; as a result, a more aggressive follow-up is proposed in patients with Maffucci syndrome to exclude neoplasms 5).


First case of Maffucci syndrome associated with a pituitary adenoma and a probable brainstem glioma
A 35-year-old woman with Marfucci syndrome (diagnosed when she was 22 years old) who presented with complaints of decreased visual acuity and visual field defect. Neuroimaging revealed a pituitary macroadenoma and a suspected brainstem tumor. The macroadenoma was partially removed. There were no postoperative complications and the patient experienced rapid improvement in visual acuity. On follow-up examination 2 years later, the lesion in the pons showed the same dimensions. No sarcomatous changes of enchondromas or hemangiomas occurred. To the authors’ knowledge, including the present case, only 7 cases of Maffucci syndrome associated with glioma and 7 cases associated with pituitary adenoma have been reported in the literature. This report emphasizes that patients with this disease are at a higher risk for primary intracranial tumors and reinforces the concept of the multiplicity of tumors that may arise in this syndrome. It also underscores the importance of early diagnosis, regular clinical surveillance, and follow-up studies of these patients 6).

Akiyama M, Yamaoka M, Mikami-Terao Y, Ohyama W, Yokoi K, Arakawa Y, Takita J, Suzuki H, Yamada H. Somatic mosaic mutations of IDH1 and NPM1 associated with cup-like acute myeloid leukemia in a patient with Maffucci syndrome. Int J Hematol. 2015 Dec;102(6):723-8. doi: 10.1007/s12185-015-1892-z. Epub 2015 Oct 27. PubMed PMID: 26508204.

Pansuriya TC, van Eijk R, d’Adamo P, van Ruler MA, Kuijjer ML, Oosting J, Cleton-Jansen AM, van Oosterwijk JG, Verbeke SL, Meijer D, van Wezel T, Nord KH, Sangiorgi L, Toker B, Liegl-Atzwanger B, San-Julian M, Sciot R, Limaye N, Kindblom LG, Daugaard S, Godfraind C, Boon LM, Vikkula M, Kurek KC, Szuhai K, French PJ, Bovée JV. Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome. Nat Genet. 2011 Nov 6;43(12):1256-61. doi: 10.1038/ng.1004. PubMed PMID: 22057234; PubMed Central PMCID: PMC3427908.

Mandonnet E, Anract P, Martin E, Roujeau T, Spena G, Cormier-Daire V, Duffau H, Baujat G; Collaborators. Brain and skull base MRI findings in patients with Ollier-Maffucci disease: A series of 12 patient-cases. Clin Neurol Neurosurg. 2017 Jul 18;160:147-151. doi: 10.1016/j.clineuro.2017.07.011. [Epub ahead of print] PubMed PMID: 28750360.

Hao S, Hong CS, Feng J, Yang C, Chittiboina P, Zhang J, Zhuang Z. Somatic IDH1 mutation in a pituitary adenoma of a patient with Maffucci syndrome. J Neurosurg. 2016 Jun;124(6):1562-7. doi: 10.3171/2015.4.JNS15191. Epub 2015 Oct 16. PubMed PMID: 26473790.

Prokopchuk O, Andres S, Becker K, Holzapfel K, Hartmann D, Friess H. Maffucci syndrome and neoplasms: a case report and review of the literature. BMC Res Notes. 2016 Feb 27;9(1):126. doi: 10.1186/s13104-016-1913-x. PubMed PMID: 26920730; PubMed Central PMCID: PMC4769492.

Ruivo J, Antunes JL. Maffucci syndrome associated with a pituitary adenoma and a probable brainstem tumor. J Neurosurg. 2009 Feb;110(2):363-8. doi: 10.3171/2008.8.JNS08150. Review. PubMed PMID: 18976063.
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