Ciliary claws: their existence in various epithelial cysts of the central nervous system

Summary We describe claw-like projections and their issociated structures as encountered at the tips of cilia in a cerebellar epithelial cyst, an intraspinal bronchogenic cyst and two colloid cysts of the third ventricle. Ciliary claws appear either as a single structure or as a cluster of two to five projections measuring 22–28 nm in length and 8–10 nm in diameter, extending from the plasma membrane of the ciliary tip. The transmembranous fibrils of the ciliary claws are bound to a multilayered electron-dense disc which is attached by the distal ends of axonemal microtubules. These observations suggest that ciliary claws are common in several types of epithelial cysts of the central nervous system; the presence of these structures supports the premise that the corresponding epithelium is of endodermal origin.     

Spontaneous hemorrhage into spinal neurenteric cyst

Case report A case of spontaneous hemorrhage into an intradural extramedullary neurenteric cyst at the C4–C6 level in a 6-year-old boy is reported. The child presented with sudden neck pain, torticollis, and myelopathy. The cyst was of high signal intensity on both T1- and T2-weighted magnetic resonance images, suggesting hemorrhage into the cyst. The combination of CT and MR provided useful preoperative information regarding the nature and the location of the cyst. A posterior approach via osteoplastic laminotomy was used for this anteriorly localized cyst. The use of intraoperative ultrasound was helpful in identifying and puncturing the cyst to minimize the spinal cord manipulation during the excision of the cyst wall.Discussion We report the first documented case of hemorrhage into a spinal neurenteric cyst that has been confirmed surgically and histopathologically.     

Lumbar synovial cyst in a adolescence: case report

Background  Spinal extradural cysts are a rare cause of spinal cord or nerve root compression which tends to occur in the elderly but rarely reported in the under 20s. History  A 14-year-old girl with a 9-month history of left radicular pain was found to have an intraspinal cystic lesion causing radicular compression. Magnetic resonance imaging showed a 1.1-cm extradural cystic lesion with a low signal on T1-weighted images and high signal on T2-weighted images lying in the spinal canal at the L4 vertebral body level. The patient underwent an L4 hemilaminectomy and excision of a synovial cyst, and the radicular pain completely regressed. Discussion  We discuss the pathogenesis, radiological techniques and management of synovial cyst in a paediatric patient Conclusion  Intraspinal ganglion cysts are extremely rare in children and only two other cases have been reported previously. They are benign lesions, frequently presenting radiculopathy, and should be considered in the differential diagnosis in patients with low back pain and radiculopathy.     

Ante- and postnatal evaluation of the dandy-walker syndrome

In three cases an antenatal diagnosis of isolated Dandy-Walker cyst was made at 22–28 weeks of gestation by ultrasound examination. Biparietal growth and the relative severity of the anomaly remained constant, so that all cases could be safely delivered at term. Postnatally, the diagnosis was confirmed during the first days of life by ultrasound and computed tomography (CT). Early neonatal shunting procedures were performed. Dual shunts were inserted in all three cases, and mental and physical development were observed to be normal during a follow-up of from 1.5 to 4.5 years. Early antenatal diagnosis of Dandy-Walker cyst may improve the prognosis and reduce the mortality, which has been reported to be 30%–48%. Dual shunting of both the cyst in the posterior fossa and the lateral ventricles has proved to be a safe and reliable method during the neonatal period.     

Lumbar synovial cyst in an adolescent: case report

Background  Spinal extradural cysts are a rare cause of spinal cord or nerve root compression which tends to occur in the elderly but rarely reported in the under 20s. History  A 14-year-old girl with a 9-month history of left radicular pain was found to have an intraspinal cystic lesion causing radicular compression. MRI showed a 1.1-cm extradural cystic lesion with a low signal on T1 and high signal on T2-weighted images lying in the spinal canal at the L4 vertebral body level. The patient underwent an L4 hemi-laminectomy and excision of a synovial cyst, and the radicular pain completely regressed. Discussion  We discuss the pathogenesis, radiological techniques, and management of synovial cyst in a pediatric patient. Conclusion  Intraspinal ganglion cysts are extremely rare in children and only two other cases have been reported previously. They are benign lesions, frequently presenting radiculopathy, and should be considered in the differential diagnosis, in patients with low back pain and radiculopathy.     

Giant intradural extramedullary arachnoid cyst of the thoracic spine

Spinal intradural arachnoid cysts (ACs) are found frequently in the thoracic region, and often extend over four or five vertebral levels. We present a 28-year-old patient who had a giant thoracic congenital intradural extramedullary AC (T1–T12) with a 10-month history of pain, paresthesia, paraparesis and gait ataxia. A T3 to T6 laminectomy was performed. After durotomy, the posterior wall of the AC was visualized compressing the spinal cord. We resected the cyst wall as widely as possible and connected the cyst to the subarachnoid space using a catheter. There were no postoperative complications. At 1-year follow-up, the patient presented with no motor deficits or pain, and had experienced progressive resolution of the gait ataxia. The treatment of giant intradural extramedullary ACs, especially for those that cannot be totally excised, should include generous fenestration and the insertion of a cyst–subarachnoid shunt.     

Colloid cysts of the third ventricle: ultrastructural features are compatible with endodermal derivation

Summary The histogenesis of colloid cyst of the third ventricle remains unsettled. Ultrastructural and immunohistochemical analyses have suggested the following possible origins: (a) neuroepithelium, including paraphysis, ependyma, choroid plexus and tela chorioidea; and (b) endoderm, including respiratory and enteric epithelium. This report describes the ultrastructural features of the lining epithelium in four cases of colloid cyst. Six distinct cell types were recognized: (1) ciliated cells with occasional abnormal cilia; (2) non-ciliated cells with microvilli coated with granulofibrillary material; (3) goblet cells showing discharge of secretory granules; (4) basal cells with prominent tonofilaments and desmosomes; (5) basal-located cells with elongated electron-lucent cytoplasm and scattered membrane-bound dense-core granules (150–350 nm); and (6) small undifferentiated cells with scanty organelles. Junctional complexes were present in the former four cell types but absent in the latter two. The types of epithelial cells and their topographic distribution within the epithelium are both very similar to those of normal respiratory epithelium and to the lining epithelium of intraspinal bronchogenic cyst. The observations made in the present study are compatible with the hypothesis that colloid cysts of the third ventricle originate from the endoderm, most likely the respiratory epithelium.Presented in part at the Annual Meeting of the American Association of Neuropathologists, Baltimore, MD, June 20–23, 1991     

Characterization of normal brain-reactive antibodies in glioma cyst fluids

Anti-normal brain antibodies were studied in glioma cyst fluids. Cyst fluids were obtained by stereotaxic puncture from 34 patients with cystic gliomas. Immunoglobulins were analyzed by immunohistochemistry, immuno-Western blotting, concentration measures, and isoelectrofocalization. 80% of cyst fluids stained astrocytes and/or microvessels in non-tumoral white matter. In white matter extracts, cyst fluids recognized five immunoreactive bands having apparent molecular masses of 50–75 kDa. Although cyst proteins could be of systemic origin, isoelectrofocalization analysis suggests an additional local immune response. These antibody activities could be involved in certain peritumoral events such as brain edema.     

Rathke''s cleft cyst: a clinical and radiographic review

We report the case of a 62-year-old woman, who had a 4-year history of progressive visual acuity deficit. On neurological examination, visual acuity was 5/20 in the right and 4/20 in the left eye. A hormonal study revealed hypophyseal hypofunction. CT and MRI scans showed an intra-suprasellar cystic lesion, hyperintense in T1 and hyperintense in T2-weighted sequences. At surgery, the cyst wall was opened and the cavity placed in communication with the subarachnoid space. Histological examination showed a Rathke's cleft cyst. After four years of follow-up the patient's visual deficit remained unchanged, whereas the results of the hormonal assays were normal. An MRI scan confirmed the absence of the cyst. We review 216 reported cases of Rathke's cyst and discuss its pathogenesis, clinical features and treatment.

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Sommario Riportiamo il caso di una donna di 62 anni che presentava una storia clinica della durata di 4 anni caratterizzata da deficit del visus. All'esame neurologico, si evidenziava che l'acuità visiva era di 5/20 in OD e di 4/20 in OS. Lo studio della funzionalità ipofisaria evidenziava una ridotta attività. La TC e la RNM rivelavano una lesione cistica intra-sopra-sellare. All'intervento, la cisti veniva messa in comunicazione con lo spazio subaracnoideo. La diagnosi istologica era di cisti di Rathke. Ad un follow-up di 4 anni, il deficit visivo è invariato mentre la funzionalità ipofisaria è normale. Abbiamo rivisto i 216 casi di questa patologia riportati ed abbiamo trattato la patogenesi, i caratteri clinici ed il trattamento delle cisti di Rathke.

     

Discal Cyst of the Lumbar Spine

Discal cysts are rare lesions that can cause radiating leg pain. Because they are very rare, their natural history and the details of the therapeutic guidelines for the treatment of these cysts are still unknown. A 30-year-old male patient presented to our institute with radiating pain in his left leg and mild back pain. Magnetic resonance imaging (MRI) revealed an intraspinal extradural cystic mass with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images at the L5-S1 level. The partial hemilaminectomy and cyst resection were performed. We report a patient with low back pain and radiating leg pain caused by a lumbar discal cyst and discuss the treatment of this cyst.     

Lumbar intraspinal extradural ganglion cyst

A case is presented of an intraspinal extradural ganglion cyst at the L4–5 level. The clinical picture suggested a herniated nucleus pulposus at this level. A myelogram revealed a round lesion almost completely obstructing the flow of Pantopaque at the L4–5 level. A ganglion cyst with a haemorrhage into it and the surrounding tissue was removed, and surgery was followed by complete recovery.     

A case of hypothalamic hamartoma associated with arachnoid cyst

A 14-year-old girl presented with seizures. Radiological examinations revealed an arachnoid cyst in left middle fossa and a cystic mass in the interpeduncular cistern. The cyst was opened and the wall of the cyst and a mass were biopsied. The histological findings were characteristic of an arachnoid cyst and hamartoma, respectively. A hypothalamic hamartoma associated with an arachnoid cyst is comparatively rare; however, such a case may help clarify the genesis of this malformation. Received: 5 November 1998 Accepted: 19 January 1999     

Nasal dermal sinus and dermoid cyst with intrafalcial extension

Congenital nasal dermal sinus is an unusual lesion which comprises about 11%–12% of all cranial dermal sinuses. The sinus tract seldom extends intracranially and an associated intrafalcial inclusion cyst is extremely rare. The authors report a case of nasal dermal sinus and dermoid cyst with intrafalcial extension in a boy aged 1 year 4 month. Previous reports of this rare lesion are reviewed and the possible pathogenesis discussed.     

Spinal Extradural Meningeal Cyst in Klippel-Trenaunay Syndrome

A case of a symptomatic spinal extradural meningeal cyst (SEMC) in Klippel-Trenaunay syndrome (KTS) is introduced. A 38-year-old woman presented with right L2 radiculopathy. She underwent operations for varicose veins in both her lower extremities. She had port-wine nevi on her trunk and extremities. The edematous change in both legs had waxed and waned. Magnetic resonance imaging showed an 11.8×13 mm extradural meningeal cyst growing through the intervertebral foramen in L2-3. Multiple meningeal cysts were located in the dorsal aspect of the spinal cord from T3 to T10. A 5.8×6.2 mm cyst was also found in left pleural cavity. The extradural meningeal cyst was completely excised and the preoperative symptom was improved. KTS is a congenital disorder due to a mesodermal abnormality, which may predispose the dura to weakness. The SEMC may occur through the dural defect or weakened point.     

Neuroepithelial cyst of the cerebellopontine angle: A case report with a review of the literature

A 44-year-old woman had occasional headaches and dysfunction of the facial and trigeminal nerves on the left side as well as ataxia. These clinical manifestations disappeared after removal from the left cerebellopontine angle of a 3 × 4.5 × 5-cm prealbumin-positive cyst of the choroid plexus epithelium, a type of neuroepithelial cyst. A review of 57 reported cases of neuroepithelial cysts in the posterior cranial fossa disclosed 32 symptomatic cysts (56%) and 25 symptomless cysts (44%). The recorded age of 35 patients ranged from 5.5 months to 65 years with the average age of 30 years for both genders; 14% of patients were less than 1-year-old. The ratio of males and females was 1:1. The cysts were located at the following sites: fourth ventricle (51%); intraneural substance as in cerebellar vermis, pons and mesencephalon (7%), and posterior cranial fossa proper (42%) especially in the cerebellopontine angle (18%). The pathogenesis of these cysts is proposed to be related to developmental anomaly of the neuroepithelium which also includes the ectopic neuroepithelial elements. Surgical extirpation of the cyst is the treatment of choice. Total resection should be attempted if it is feasible depending on where the cyst is situated. Fenestrating the cyst wall is also a useful surgical procedure in order to allow communication between the cyst contents and the pathway of the cerebrospinal fluid for abolishing the increased intracranial pressure.     

The use of an adjustable valve to treat over-drainage of a cyst-peritoneal shunt in a child with a large sylvian fissure arachnoid cyst

Introduction The cyst-peritoneal shunt is a recognised surgical alternative in the management of sylvian fissure arachnoid cysts. Shunt overdrainage is well described in literature on ventriculo-peritoneal shunts, but not often appreciated as a complication of cysto-peritoneal shunts.Case report A 5-year-old boy presented with a symptomatic left sylvian fissure arachnoid cyst. This was initially treated by craniotomy and membrane fenestration in the carotid cistern. Recurrence led to insertion of a valveless cyst-peritoneal shunt 5 months later. Initial progress was followed by persistent headaches 18 months after shunt insertion. CT scan revealed a significant reduction in the cyst size, enlargement of the ipsilateral lateral ventricle, collapse of the contra-lateral ventricle and midline shift towards the side of the shunt. These findings were interpreted as over-drainage of the cyst-peritoneal shunt.Result A Codman Medos adjustable valve was inserted, with the intention of gradually increasing the pressure until the midline shift was restored and the contra-lateral ventricle was reconstituted. This was achieved with the valve set at 90 mm H₂O, verified by CT scan. Radiological improvement was associated with dramatic symptomatic improvement.Conclusion Over-drainage of cyst-peritoneal shunts is often not appreciated, especially when the main manifestation is headaches. As it is difficult to predict the required valve pressure setting, it may be advisable to consider the use of an adjustable valve.The material of this paper was presented as a poster at the 31st Annual Meeting of the International Society for Pediatric Neurosurgery, Monaco, 14–18 September 2003     

Spinal Extradural Arachnoid Cyst

Spinal extradural arachnoid cyst (SEAC) is a rare disease and uncommon cause of compressive myelopathy. The etiology remains still unclear. We experienced 2 cases of SEACs and reviewed the cases and previous literatures. A 59-year-old man complained of both leg radiating pain and paresthesia for 4 years. His MRI showed an extradural cyst from T12 to L3 and we performed cyst fenestration and repaired the dural defect with tailored laminectomy. Another 51-year-old female patient visited our clinical with left buttock pain and paresthesia for 3 years. A large extradural cyst was found at T1-L2 level on MRI and a communication between the cyst and subarachnoid space was illustrated by CT-myelography. We performed cyst fenestration with primary repair of dural defect. Both patients'' symptoms gradually subsided and follow up images taken 1-2 months postoperatively showed nearly disappeared cysts. There has been no documented recurrence in these two cases so far. Tailored laminotomy with cyst fenestration can be a safe and effective alternative choice in treating SEACs compared to traditional complete resection of cyst wall with multi-level laminectomy.     

A Boy With Arachnoid Cyst,a Fall,and Temporary and Reversible Visual Impairment

BackgroundTemporal arachnoid cysts have been shown to interfere with the function of nervous structures, both cerebral cortex and cranial nerves.Methods and ResultsIn this case report we describe a boy with a left temporal arachnoid cyst with a sudden onset of visual impairment 4 days after a mild trauma to the head. A perimetry test revealed a complete nasal hemianopia of the left eye, which normalized rapidly after an emergency craniotomy with cyst fenestration 3 hours after the patient experienced the reduced vision.ConclusionsThe observed, reversible effect on vision is most probably caused by pressure exerted by the medial part of the cyst on the left optic nerve. We believe this is the first time such a case has been described.     

Aprosencephaly: Review of the literature and report of a case with cerebellar hypoplasia,pigmented epithelial cyst and Rathke's cleft cyst

Summary Aprosencephaly is a very rare brain malformation that occurs in isolated and sydromatic forms. The syndromatic form has been named XK-aprosencephaly, and is characterized by near total absence of prosencephalon with a midline oculofacial defect similar to the most severe forms of holoprosencephaly, in association with limb and genital anomalies. We present a case of syndromatic aprosencephaly with absence of thumb and abnormal external genitalia. A previously undescribed finding was a Tathke's cleft cyst. Two other cystic structure were also identified — an ependymal cyst, which may represent a dorsal cyst as in holoprosencephaly, and a pigmented epithelial cyst, which may represent a rudimentary eye. Additional findings were extensive calcific vasculopathy in the rudimentary prosencephalon, absence of pituitary gland, forking of the aqueduct of Sylvius and marked cerebellar hypoplasia. Since calcific vasculopathy is a common accompaniment of other inflammatory diseases of the central nervous system, its presence in this case suggests that destructive processes may be involved in the genesis of some cases of aprosencephaly.     

Giant,calcified colloid cyst of the lateral ventricle

We report a patient with a giant, calcified colloid cyst in the left lateral ventricle. Colloid cysts are slow growing, benign lesions, commonly originating in the roof of the anterior third ventricle near the foramen of Monro. Many colloid cysts are small lesions which are either discovered incidentally or cause headache, visual changes, memory deficits, and/or syncope. Giant colloid cysts are rare. A 40-year-old man presented with a month long history of worsening headaches and was found to have a multiloculated 5 cm intraventricular mass with an anterior hyperdensity, suggestive of calcification, arising within the lateral ventricles. He underwent an interhemispheric transcallosal approach for resection of the mass. The pathology was consistent with a giant colloid cyst with calcification in the anterior cyst wall. Giant, calcified mass is a rare presentation of colloid cyst. Although rare, this diagnosis remains an important consideration in the differential diagnosis of any calcified, cystic intraventricular mass.