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if their systemic condition poses reasonable anaesthetic risk.10 In the present case, we had planned direct clipping through a small inferior temporal corticotomy. If microsurgical clipping is impossible the parent artery may be trapped and the aneurysm excised. Recent reports highlight successful aggressive treatment of aneurysms with endovascular techniques.11 Coil embolization can be explored for mycotic aneurysms arising closer to the circle of Willis. When the aneurysm does involve an eloquent parent artery, such as the AchA, endovascular therapy should be limited to direct coil occlusion of the aneurysm, with parent artery preservation. Surgical treatment has a better rate of parent artery preservation or revascularization than does endovascular treatment in distal aneurysms of an eloquent parent artery. Therefore, a microsurgical approach was likely to be a better treatment modality than endovascular management in the present case. In conclusion, we present the rst reported case of a 60year-old woman with subarachnoid and intraventricular haemorrhage due to an aneurysm, which arose from a branch of the cisternal segment of the AchA. Early detection and intervention should be undertaken. References
1. Kwok-chu Wong G, Boet R, et al. Ruptured distal anterior choroidal artery aneurysm presenting with right intracerebral haematoma: doi:10.1016/j.jocn.2005.11.033

2.

3.

4.

5.

6.

7. 8.

9. 10. 11.

clipping aided by subpial uncal resection. J Clin Neurosci 2003;10:68991. Lee JK, Lee JH, Kim SH, et al. Distal anterior choroidal artery aneurysm in a patient with moyamoya disease. Case report. Neurosurgery 2001;48:2225. Nishihara J, Kumon Y, Matsuo Y, et al. A case of distal anterior choroidal artery aneurysm. Case report and review of the literature. Neurosurgery 1993;32:8347. Yanaka K, Tsuboi K, Fujita K, et al. Distal anterior choroidal artery aneurysm associated with arteriovenous malformation. Intraoperative localization and treatment. Surg Neurol 2000;53: 54651. Yoneoka Y, Ezuka I, Takai N, et al. Ruptured distal anterior choroidal artery aneurysm presenting with casting intraventricular haemorrhage. Acta Neurochir (Wien) 1998;140:1859. Nishioka H, Torner JC, Graf CJ, et al. Cooperative study of intracranial aneurysms and subarachnoid hemorrhage: a long-term prognostic study. III. Subarachnoid hemorrhage of undetermined etiology. Arch Neurol 1984;41:114751. Suzuki S, Kayama T, Sakurai Y, et al. Subarachnoid hemorrhage of unknown cause. Neurosurgery 1987;21:3103. Donauer E, Reif J, al-Khalaf B, et al. Intraventricular hemorrhage caused by aneurysms and angiomas. Acta Neurochir (Wien) 1993;122:2331. Rhoton Jr AL, Fujii K, Fradd B. Microsurgical anatomy of the anterior choroidal artery. Surg Neurol 1979;12:17187. Ojemann RG, Heros RC, Crowell RM. Surgical Management of Cerebrovascular Disease. Baltimore: Williams & Wilkins; 1988. Cloft HJ, Kallmes DF, Jensen ME, et al. Endovascular treatment of ruptured, peripheral cerebral aneurysms: parent artery occlusion with short Guglielmi detachable coils. Am J Neuroradiol 1999;20: 30810.

Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited

Ram K. Menon, Trimurti D. Nadkarni *, Ketan I. Desai, Atul Goel
Department of Neurosurgery, King Edward Memorial Hospital, Seth G.S. Medical College, Parel, Mumbai 400 012, India Received 4 August 2005; accepted 23 September 2005

Abstract A 32-year-old woman was diagnosed with chronic bilateral frontoparietal and interhemispheric subdural haematomas (SDH). Abdominal ultrasonography revealed chronic renal failure due to bilateral large cystic kidneys. Brain MRI demonstrated Dandy-Walker malformation associated with hypoplastic vermis, in addition to the chronic subdural haematomas. This association of polycystic kidneys with Dandy-Walker malformation has been referred to as Goldston or cerebrorenal syndrome. The case is rare and is the rst to be reported in an adult patient. The relevant literature is reviewed. 2006 Elsevier Ltd. All rights reserved.
Keywords: Dandy-Walker malformation; Polycystic kidneys; Goldston syndrome; Cerebrorenal syndrome

Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: tdnadkarni@hotmail.com (T.D. Nadkarni).

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1. Introduction The Dandy-Walker malformation (DWM) of hydrocephalus associated with a posterior fossa cyst and dysgenesis of the cerebellum, is a relatively uncommon central nervous system anomaly. Dandy-Walker syndrome is frequently associated with both neurological and systemic anomalies. Postnatal death in Dandy-Walker syndrome is frequently attributed to lethal malformations of the cardiac, skeletal, genitourinary and gastrointestinal organs. Goldston syndrome is an association of cystic dysplastic kidneys with DWM.1,2 We report a case of Goldston syndrome and discuss the relevant literature. 2. Case report A 32-year-old woman presented with headache, progressive inability to walk and occasional vomiting over the previous 5 days. There was a history of a trivial fall 3 months prior to the admission. On examination she had truncal ataxia and no other focal neurological decit. MRI revealed bilateral subdural collections in the frontoparietal areas extending to the temporal region and into the inter-

hemispheric ssure (Fig. 1A, B). These collections were hyperintense on both T1- and T2-weighted sequences. A large posterior fossa cyst communicating with the fourth ventricle was identied, which was associated with a hypoplastic vermis, suggestive of DWM (variant) (Fig. 1C). Ultrasonography of the abdomen revealed bilateral polycystic kidneys (Fig. 2). The right kidney was 10.9 6.4 cm and the left 11.6 6.3 cm. The liver and pancreas were normal. The coagulation prole was within normal limits. Renal function tests revealed azotemia due to chronic renal failure. Bilateral frontoparietal burr holes were performed and the subdural uid was drained. Approximately 150 mL of xanthochromic uid with small blood clots was drained from each side. The recovery was uneventful. Karyotyping was done postoperatively which revealed normal genotype (46XX). 3. Discussion Congenital renal anomalies are rarely associated with central nervous system (CNS) malformations.3 Meckels syndrome is considered to be the prototype and it is associated with CNS malformations such as cranial

Fig. 1. (A) Coronal T1-weighted MRI shows bilateral chronic subdural haematomas. (B) T1-weighted sagittal MRI shows the sudural collection to be associated with Dandy-Walker malformation. (C) T1-weighted axial MRI shows the hypoplastic vermis.

Fig. 2. Abdominal ultrasonography demonstrates bilateral polycystic kidneys (A) right (B) left.

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rachischisis and occipital meningoencephalocele, ocular anomalies, cleft palate, polydactyly, cardiac anomalies, cystic kidneys, pseudohermaphroditism and other malformations.2,4 One-fth of Meckels syndrome patients have hepatic anomalies, primarily congenital hepatic brosis.3 Generalised cystic dysplastic renal lesions, DWM and congenital hepatic brosis characterise Mirandas or cerebrohepatorenal syndrome, a rare familial disorder.1,4 Goldston syndrome is an association of cystic dysplastic kidneys with DWM.2 This combination of congenital anomalies was present in our patient. It appears that the Goldston and Mirandas syndrome are milder variants, with fewer anomalies, of Meckels syndrome. Gloeb et al. described the rst reported case of Goldston syndrome in intrauterine life during the seventeenth week of gestation.5 The condition was diagnosed by ultrasound in a 635 gm fetus. Moerman et al. described two siblings with a combination of central nervous system malformation, renal dysplasia, and hepatic ductal plate malformation.6 The rst child was diagnosed as having Goldston syndrome based on the presence of DWM. However, both the patients had cranium bidum with defects in the squamous part of occipital bone.6 They observed that Goldston syndrome is not a distinct entity but merely a variant of Meckels syndrome, and that a cerebellar DWM can be a component of Meckels syndrome. Walpole et al. reported a family in which three nonviable brothers had DWM (variant) with associated enlarged cystic renal dysplastic kidneys and hepatic brosis.7 In the absence of polydactyly and encephalocoele Walpole et al. suggested the possibility of a separate and distinct syndrome. This presentation is similar to Mirandas syndrome. Thus, the phenotypic spectrum of Meckels syndrome includes those described as Goldston and Mirandas syndrome. Osathanondh and Potter classied cystic kidneys as Type I to IV based on the pathological anatomy.8 These pathoanatomic types do not represent genetic entities. Type III kidneys include autosomal dominant polycystic kidney disease, which is present in childhood. The Type III changes are part of syndromes or nonhereditary malfordoi:10.1016/j.jocn.2005.09.012

mations, and often present only as mild manifestations.8 Our patient had Type O kidney changes, demonstrated as enlarged cystic, dysplastic kidneys bilaterally. Dandy-Walker malformation can be diagnosed in utero by prenatal sonography. An evaluation for associated supratentorial and extra-cranial defects should be performed. Coexisting structural and chromosomal anomalies may occur and result in poor survival. In a retrospective study of 15 cases of Dandy-Walker syndrome by Russ only 33% had abnormal karyotypes.9 Dandy-Walker malformation is not limited to a mere mechanical disturbance of CSF circulation but represents a more generalised disorder of neural development. In conclusion, the presence of DWM warrants a search for associated extra-cranial malformations, particularly in the cardiac, skeletal, genitourinary and gastrointestinal systems. References
1. Gulcan YH, Duman N, Kumral A, et al. Goldston syndrome: report of a case. Genet Couns 2001;12:2637. 2. Goldston AS, Burke EC, DAgostino A, et al. Neonatal polycystic kidney with brain defect. Am J Dis Child 1963;106:4848. 3. Bernstein J, Brough AJ, McAdams AJ. The renal lesion in syndromes of multiple congenital malformations. Cerebrohepatorenal syndrome; Jeune asphyxiating thoracic dystrophy; Tuberous sclerosis; Meckel syndrome. Birth Defects Orig Artic Ser 1974;10:3543. 4. Kudo M, Tamura K, Fuse Y. Cystic dysplastic kidneys associated with Dandy-Walker malformation and congenital hepatic brosis. Report of two cases. Am J Clin Pathol 1985;84:45963. 5. Gloeb DJ, Valdes-Dapena M, Salman F, et al. The Goldston syndrome: report of a case. Pediatr Pathol 1989;9:743. 6. Moerman P, Pauwels P, Vandenberghe K, et al. Goldston syndrome reconsidered. Genet Couns 1993;4:97102. 7. Walpole IR, Goldblatt J, Hockey A, et al. Dandy-Walker malformation (variant), cystic dysplastic kidneys, and hepatic brosis: a distinct entity or Meckel syndrome? Am J Med Genet 1991;39:2948. 8. Zerres K, Volpel MC, Weiss H. Cystic kidneys. Genetics, pathologic anatomy, clinical picture, and prenatal diagnosis. Hum Genet 1984;68:10435. 9. Russ PD, Pretorius DH, Johnson MJ. Dandy-Walker syndrome: a review of fteen cases evaluated by prenatal sonography. Am J Obstet Gynecol 1989;161:4016.

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

Borut Prestor
*
ka 7, 1525 Ljubljana, Slovenia Department of Neurosurgery, University Hospital Center, Zalos Received 19 January 2005; accepted 26 July 2005

Tel.: +386 15 223263; fax: +386 15 222218. E-mail address: borut.prestor@kclj.si.