PARTIAL AGENESIS OF SEPTUM PELLUCIDUM WITH CAVE OF SEPTUM PELLUCIDUM; REPORT OF A CASE WITH REVIEW OF LITERATURE.
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A septum pellucidum cyst is defined as a cystic structure between the lateral ventricles, whose walls exhibit lateral bowing and are 10 mm apart or greater. This communication presents computerized tomography (CT) and magnetic resonance imaging (MRI) findings in six patients with septum pellucidum cysts, ages ranging from 1.5 to 47 yrs. The width of the cysts ranged from 13 to 23 mm, and their walls were laterally bowed. Only one patient had hydrocephalus, who was surgically treated. The remaining five patients only had intermittent headaches. It appears that further studies will be required to establish satisfactory diagnostic and therapeutic criteria for symptomatic or presumably symptomatic septum pellucidum cysts.
The cavum septum pellucidum is not a part of the brain's ventricular system and does not communicate with the lateral ventricles. However, under conditions of increased intraventricular pressure, cerebrospinal fluid may penetrate the septum and cause formation of a cavity. We report a neonate with pus accumulation in the cavum septum pellucidum after an episode of ventriculitis. The cavum septum pellucidum disappeared after medical and surgical management of the infection and increased intracranial pressure.
Absence of the septum pellucidum in the human is a rare congenital anomaly. Previous reports suggest it is almost always associated with other brain anomalies. However, MRI in two patients with absence of the septum pellucidum presented here, indicates that this anomaly may occur without associated anomalies. It may be one manifestation of a spectrum of developmental anomalies. One patient presented with schizophrenic psychosis; developmental disturbances in limbic areas are believed to be associated with schizophrenia. Agenesis of the septum pellucidum may indicate abnormal development of limbic structures and it may be associated with anomalies, such as cytoarchitectural disturbances of cortical layers, as yet undetectable by MRI.
Absence of the septum pellucidum may occur as an isolated malformation. Some of the common paediatric ultrasound and radiology textbooks mention this anomaly. However, only 12 case reports have been published so far. Absent septum pellucidum is also seen in combination with other cerebral malformations like septo-optic pituitary dysplasia (SOPD, de Morsier), agenesis of corpus callosum, holoprosencephaly etc. More often it is seen as a secondary injury due to an increasing internal hydrocephalus. Occurrence of this aplasia and its significance are often underestimated. At a paediatric centre in Germany the anomaly is expected to occur in 1: 1,000 to 1:3,000 cerebral ultrasound examinations. After a review of the literature four cases are presented. All of them were observed by ultrasound during the neonatal period. Using ultrasonography diagnosis of the isolated absence of septum pellucidum can be easily performed by the experienced physician, and will usually be of eminent relevance for the patient.
The cavum septum pellucidum is a fluid-filled cavity located between the membranes of the septi pellucidi, which should always be visualized sonographically in normal fetuses between 18 and 37 weeks gestation and in approximately 50% of term infants. The cavum vergae, a cavity within the septum pellucidum, is located posterior to an arbitrary vertical plane formed by the columns of the fornix. We present a case in which markedly dilated cavum septum pellucidum et vergae noted at 29 weeks gestation was associated with midgut malrotation and volvulus, and review the literature pertaining to this unusual prenatal ultrasonographic diagnosis.
Expanding cysts of the septum pellucidum, although rare, may be a cause of significant neurological dysfunction. Most become symptomatic as a result of obstruction of the interventricular foramina and produce headaches, papilledema, emesis, and loss of consciousness. Behavioral, autonomic, and sensorimotor symptoms occur when an expanding cyst impinges on the structures of the hypothalamoseptal triangle or impairs the deep cerebral venous drainage. Neuroophthalmological symptoms may develop as a consequence of hydrocephalus or direct compression of visual structures. The authors describe the case of a young boy with an expanding septum pellucidum cyst who presented with a sudden, severe headache and loss of consciousness. In addition, he had a history of hyperactivity and progressively declining school performance. All symptoms resolved following decompression of the cyst. Seventeen cases from the literature are reviewed. The pathophysiological mechanisms underlying the development of symptoms secondary to expanding septum pellucidum cysts are outlined, and the related clinical neuroanatomy is described. A model is proposed for the natural history of expanding septum pellucidum cysts that provides a rational basis for understanding their clinical behavior and response to intervention. In most cases, fenestration or shunting will relieve the obstructive hydrocephalus and mass effect caused by the cyst and will produce rapid symptomatic improvement.
Septum pellucidum agenesis is frequently associated with other cerebral malformations. Its isolated agenesis is quite rare. In the literature, all reported cases were diagnosed in early childhood. The authors present an unusual case of isolated septum pellucidum agenesis detected in adulthood with computed tomography and magnetic resonance imaging.
OBJECTIVE: Ten rare cases of symptomatic septum pellucidum cysts in patients who underwent endoscopic fenestration are described. The approaches and techniques used in the management of these cysts and the endoscopic surgical indications are discussed. CLINICAL PRESENTATION: In the past 5 years, 10 patients (age range, 3-60 yr) with symptomatic septum pellucidum cysts underwent neuroendoscopic fenestration. The most common symptom was intermittent headache (seven patients) accompanied by dizziness, vomiting, and epileptic seizures. Two patients presented with epileptic seizures. One patient presented with abnormally increased head circumference. Magnetic resonance imaging scans of 10 patients showed septum pellucidum cysts, two with hydrocephalus, and two with pituitary microadenoma. INTERVENTION: All 10 patients underwent endoscopic fenestration with a rigid endoscope via a frontal approach. Eight cases were performed freehand. Two cases were assisted by a frameless neuronavigation system. Postoperatively, the mass effect of the cysts and the symptoms resolved immediately, and computed tomographic or magnetic resonance imaging scans showed significant decrease in the cyst size and no recurrence during follow-up. Ventricular sizes in the two patients with hydrocephalus were normal. CONCLUSION: Neuroendoscopic pellucidotomy could be an effective, safe, and convenient therapeutic method for symptomatic septum pellucidum cysts. This approach might provide communication between the cyst and the ventricular system, thus avoiding shunting or craniotomy. We consider that it is appropriate to use the rigid endoscope via the frontal approach. It is helpful to fill the ventricles with lactated Ringer's solution and leave an external drain after surgery.
Tumours of the septum pellucidum (SP) are rare and seldom associated with memory impairment either before or after operation. A patient is described who developed amnesia after transcallosal excision of a tumour of the SP. Radiology did not show any major lesion of the brain areas traditionally associated with amnesia. Because septal nuclei could have been damaged during surgery their possible role in memory functions is discussed.
A persistent cavum septum pellucidum (CSP) is present in approximately 0.73% of adults, although its incidence ranges from 0.14 to 18.9% depending on the detection method. Cystic CSP is even rarer. A cyst causing mass within the CSP can obstruct the intraventricular foramen, leading to blockage of CSF flow and possible hydrocephalus, often justifying surgical intervention. We describe spontaneous decompression of a cystic CSP in a 36-year-old man. Initial MRI showed a cystic CSP with lateral bowing of the septal walls to 1.9 cm. Follow-up MRI 15 months later demonstrated no lateral bowing, and the septal wall width was 1.0 cm. This spontaneous decompression was not explained by the one previously described case report of cystic CSP regression.
We report the natural history of the closure of the cavum septum pellucidum in 47 premature infants. In this study, a cavum septum pellucidum was present in all patients at 25 to 26 weeks' postconceptual age, in keeping with previous reports. The data from this study suggest that premature delivery does not change the natural history of the normal closure of the cavum septum pellucidum in most infants by 36 to 40 weeks' postconceptual age. Although not statistically significant, there is a suggestion from these data that higher grades of intraventricular hemorrhage are more frequently associated with loss (early closure) of the cavum septum pellucidum. One particularly illustrative case with a grade 4 intraventricular hemorrhage and subsequent hydrocephalus suggests that increases in pressure and volume in the lateral ventricles can cause the laminae of the septum pellucidum to approximate and appear to fuse earlier than expected. However, the fact that the cavum septum pellucidum reappeared in this case after ventricular pressure was decreased (postventricular shunt) suggests that approximation is not the sole factor in definitive fusion of the laminae of the septum pellucidum.
During gross anatomy dissections of the brain, a developmental abnormality of the septum pellucidum was found in a 31-year-old male cadaver. Other parts of the central nervous system in this cadaver were normal in every aspect. Histological samples were taken from the neighboring areas of this abnormality, and they were examined under light microscope and scanning electron microscope. In this abnormality of the septum pellucidum, the two laminae of the septum pellucidum were fused together and there was a hole located 1 cm anterior to its apex. The maximum diameter of the hole was 0.5 cm in the sagittal plane and 0.6 cm in the vertical plane. In the light microscopic and scanning electron microscopic examinations, the free margin of this foramen was regular, and the surrounding tissue was intact and histologically unique to the septum pellucidum. Ependymal cells were present at the free margin of the foramen. Cavum vergae, cavum septum pellucidum, and agenesis of the septum pellucidum are described in the literature. These three abnormalities are seen in cadavers usually with histories of schizophrenia and other psychiatric or neurologic disorders. In a retrospective study, the cadaver with this abnormality had a history of schizophrenia and no history or signs of any kind of brain or head operation. As far as we could ascertain, the abnormality described here has not been reported previously.
Panic disorder is thought to involve dysfunction in the septohippocampal system, and the presence of a cavum septum pellucidum might indicate the aberrant development of this system. We compared the prevalence and size of cavum septum pellucidum in 21 patients with panic disorder and in 21 healthy controls by magnetic resonance imaging. The length of the cavum septum pellucidum was measured by counting the number of consecutive 1-mm coronal slices in which it appeared. A cavum septum pellucidum of >6 mm in length was rated as large. There was no significant difference in the proportion of patients (16 of 21 or 76.2%) and controls (18 of 21 or 85.7%) with a cavum septum pellucidum (P=0.35, Fisher's exact test, one-tailed), and no members of either group had a large cavum septum pellucidum. The mean cavum septum pellucidum rating in the patient and control groups was 1.81 (SD=1.50) and 2.09 (SD=1.51), respectively. There were also no significant differences between groups when we analyzed cavum septum pellucidum ratings as a continuous variable (U=196.5; P=0.54). Across all subjects there was a trend towards a higher prevalence of cavum septum pellucidum in males (100%, 10 of 10) than females (75%, 24 of 32; P=0.09, Fisher's exact test, one-tailed). Thus, we conclude that, while panic disorder may involve septo-hippocampal dysfunction, it is not associated with an increased prevalence or size of the cavum septum pellucidum.
Septum pellucidum hamartomas have not been reported previously in neurofibromatosis type I (NF1). In a retrospective study of 86 cases with NF1 we found three patients with septum pellucidum hamartomas (frequency = 3.4 %). These could be detected on especially fluid attenuated inversion recovery and proton density-weighted images on MRI. With respect to proton spectroscopy in a patient. a low Cho level with a normal NAA peak was found, excluding a neoplasm. MR spectroscopy findings resulted in a very high NAA/Cho ratio (= 2.91). and a low Cho/Cr ratio (= 0.53) compared to a control group of 8 normal age-matched individuals. In this control group. mean NAA/Cho ratio was I .86. and mean Cho/ Cr ratio was 0.78.
The anatomical foundations for the endoscopic fenestration of the septum pellucidum have not been presented previously in an isolated fashion. Using a surgical microscope, the microsurgical anatomy of the septum pellucidum, lateral ventricles, and surrounding structures was examined in ten adult brains injected with silicone rubber. The mean anteroposterior length of the septum pellucidum was 41 mm (range 40-42). Its mean height was 13.11 mm (range 12-15) at the level of the foramen of Monro; 10 mm (range 9-11) at the frontal horns, and 8 mm (range 7-10) at the ventricular atrium. One to three septal veins delimited 2 or 3 avascular areas in the septum pellucidum. We found an anterior area which was consistently avascular and large enough to be fenestrated safety. More posterior areas, while avascular, were inconsistent in their size and shape.
BACKGROUND: Tumor involving the septum pellucidum is uncommon. Surgery as the main therapeutic procedure for this lesion is a challenge to neurosurgeons. We analyzed the clinical characteristics and pathological features of septum pellucidum tumor in 41 patients and compared the curative effects of frontal transcortical, trans-sulcal and interhemispheric transcallosal approaches. METHODS: Clinical characteristics and the pathological features of septum pellucidum tumor were investigated retrospectively in 41 patients. The differences in postoperative residual rates, extents of tumors and resection of normal brain tissues after use of the three approaches in these patients were analyzed statistically. RESULTS: Septum pellucidum tumor is more likely to attack young or middle-aged persons. The tumor mainly presents itself as a central neurocytoma or cerebral low-grade glioma in pathology and manifests as intracranial hypertension clinically. No difference was found in the extent of tumor resection but significant difference in the extent of normal brain tissue resection and in postoperative disability rate among the three approaches. The transcortical approach brought about the most serious injury to brain tissue and the highest disability rate, Whereas the frontal transcallosal approach the lightest injury and the lowest disability rate. The injury to brain tissue and the disability rate brought about by the front trans-sulcus approach were between the above two approaches. CONCLUSIONS: Operation is still regarded the major treatment for septum pellucidum tumor. Transcallosal and trans-sulcus approaches are fit with the concept of minimally invasive surgery, and transcallosal approach is the first choice for septum pellucidum tumor.
Absence of the septum pellucidum is a rare developmental disorder of the human brain. Previous reports focused on the association with other cerebral anomalies. We present MRI scans showing an isolated absence of the septum pellucidum. The patient was suffering from a schizophrenic psychosis. Mental retardation or epileptic seizures, which are often found in more complex developmental brain disorders, were not observed. The septal area is part of the limbic system and aplasia of the septum pellucidum could indicate developmental anomalies of the limbic system. The concept of cerebral maldevelopment and the limbic system in schizophrenic psychosis is discussed.