Successful Endoscopic Surgery Under Stereotactic Navigation for a Symptomatic Interhemispheric Arachnoid Cyst Without the Agenesis of the Corpus Callosum in an Elderly Patient: A Case Report

Interhemispheric arachnoid cyst in the elderly: case report and review of the literature

IHACs (interhemispheric arachnoid cysts) are a rare kind of congenital arachnoid cyst that account for5% of all occurrences. The best surgical treatment for symptomatic IHAC is still debatable, and there are no clear standards. At the time of presentation, the median age was 13 months. Macrocrania with rapidly increasing head size, seizures, infantile spasms, and developmental delay were the most common clinical manifestations.Endoscopic cyst fenestration and shunt surgery are both safe treatments for IHAC. We present an 11-month-old male infant born to non consanguineous parents who has had a steady rise in head size since 6 months of age and delayed mile stones.A magnetic resonance image of the brain revealed a massive interhemispheric arachnoid cyst, as well as corpus callosum agenesis and hypoplasia in both frontal lobes. Baby underwent endoscopic cyst fenestration and cystoperitoneal shunt.

This article describes a classification and imaging diagnosis of intracranial midline cystic malformations based on neuroembryologic analysis. Midline cystic malformations are classified into two categories from an embryologic point of view. In one category, the cyst represents expansion of the roof plate of the brain vesicle, and in the other the cyst consists of extraaxial structures such as an arachnoid membrane or migrating ependymal cells. Infratentorial cysts, such as the Dandy-Walker cyst or Blake's pouch cyst, and supratentorial cysts, such as a communicating interhemispheric cyst with callosal agenesis or a dorsal cyst with holoprosencephaly, are included in the first category. Infratentorial arachnoid cavities, such as the arachnoid cyst, arachnoid pouch, and mega cisterna magna, are in the second category. Noncommunicating interhemispheric cysts, such as interhemispheric arachnoid cyst or ependymal cyst, with callosal agenesis are also in the second category. A careful review of embryologic development is essential for understanding these midline cysts and for making a more accurate radiologic diagnosis.

Since the beginning of the last century when agenesis of the corpus callosum was first described by Reil (cited by Hyndman and Penfield, 1937) a vast amount has been written about mid-line anomalies of the brain. The anomalies described have involved several categories: agenesis of the corpus callosum, agenesis of the corpus callosum associated with a mid-line lipoma, and cysts of the septum pellucidum, cavum vergae, and cavum septi veli interpositi. The radiological history of this group goes back to the 1930's. Dandy (1931) was the first to diagnose a cyst in the septum pellucidum radiologically. Davidoff and Dyke (1934) were the first to diagnose agenesis of the corpus callosum radiologically. Sosman (1939) was the first to diagnose lipoma of the corpus callosum radiologically. Since these pioneer descriptions of plain film findings and air studies a great many reports of other cases have appeared. Radiological history really begins again with the application of angiography to these entities. This is p...

Background:Interhemispheric arachnoid cysts (IHACs) are a rare type of congenital arachnoid cyst accounting for <5% of all cases. The optimum surgical management of symptomatic IHAC is still controversial, and there are no clear guidelines.Materials and Methods:Retrospective analysis of six pediatric patients of IHAC operated from 2012 to 2015 at our institute. There was definitive sex predisposition with all patients being males. Endoscopic cystoventriculostomy, cystocisternostomy, and cystoperitoneal shunt surgeries were performed in three patients each.Results:The median age at presentation was 13 months. The most common clinical presentations were macrocrania with rapidly increasing head size, seizures, infantile spasms, and developmental delay. The mean duration of follow-up was 24.16 months (range: 3–36 months). Cyst size decreased in all the patients on follow-up imaging. Head size stabilized in all the patients. None of the patients required a second surgical procedure till the last follow-up.Conclusions:Both endoscopic cyst fenestration and shunt surgery are safe and effective in management of IHAC.

Spectrum of corpus callosum agenesis

Edwards syndrome, also known as trisomy 18, is the second most common autosomal trisomic disorder after Down syndrome (trisomy 21). This disease is characterized by phenotypic features, abnormalities of the musculoskeletal system, cardiovascular system, digestive system and genitourinary system, as well as the central nervous system. In the central nervous system, the most common abnormalities are cerebellar hypoplasia, agenesis of the corpus callosum, microgyria, hydrocephalus and myelomeningocele. The article presents a clinical case of successful fenestration of a congenital arachnoid cyst in the right lateral ventricle in a child with Edwards syndrome. Several months after birth, the child’s cranial capacity increased to 39 cm, cranial sutures were spread, and fontanelles were bulging and enlarged. Based on the clinical picture and neuroimaging results, the child had occlusive hydrocephalus. Endoscopic fenestration of the cyst was performed. In our opinion, in this case, the use of endoscopic fenestration of the arachnoid cyst was performed to improve the child’s quality of life and showed good clinical results.

Interhemispheric arachnoid cysts are very rare, and they are often associated with complex brain malformations such as corpus callosum agenesis and hydrocephalus. Debate remains concerning the proper management of these lesions. Placement of shunts and microsurgical marsupialization of the cyst are the traditional options. Using endoscopic methods to create areas of communication between the cyst, the ventricular system, and/or the subarachnoid space is an attractive alternative to the use of shunts and microsurgery. Between 2000 and 2005, seven consecutive pediatric patients with interhemispheric arachnoid cysts underwent neuroendoscopic treatment involving cystoventriculostomy in two patients, cystocisternostomy in two, and cystoventriculocisternostomy in three. There were three cases of associated hydrocephalus, six cases of corpus callosum agenesis, and one case of corpus callosum hypogenesis. The follow-up period ranged from 12 to 49 months (mean 31.6 months). Endoscopic procedures were completely successful in all but two patients. In one of the remaining two patients, a repeated endoscopic cystocisternostomy was performed with success because of closure of the previous stoma. In the other, a subcutaneous collection of cerebrospinal fluid (CSF) was managed by insertion of an lumboperitoneal shunt. A subdural collection of CSF developed in three patients; it was treated with insertion of a subduroperitoneal shunt in one patient and managed conservatively in the other two patients, resolving spontaneously without further treatment. Neurodevelopmental evaluation performed in six patients showed normal intelligence (total intelligence quotient [IQ] > 80) in three patients, mild developmental delay (total IQ 50-80) in two, and severe developmental delay (total IQ < 50) in one. Endoscopic treatment of interhemispheric cysts can be considered a useful alternative to traditional treatments, even if some complications are to be expected, such as subdural or subcutaneous CSF collections and CSF leaks due to thinness of cerebral mantle and to the often-associated multifactorial hydrocephalus.

Background. Agenesis of the corpus callosum (ACC) is the total or partial absence of CC, one of the most common congenital brain malformations, with an incidence rate of 1.4 cases per 10,000 live births. Aim. To describe the clinical and diagnostic features of 4 patients with ACC. Materials and methods. Four patients with ACC aged 11, 12, 13, and 50 years were managed, of whom 3 were males, and 1 was a 13-year-old girl. All patients underwent a neurological examination, which assessed cognitive and mental disorders and electroencephalography. Patients underwent magnetic resonance imaging (MRI) in standard modes using a magnetic resonance imaging scanner with a magnetic field intensity of 1.5 T to detect damage to the brain's anatomical structure. Results. The disease was asymptomatic in 2 patients (a 50-year-old man and a 12-year-old boy). In the other 2 cases, there was an apparent neurological and cognitive deficit. The boy's parents and grandparents died of chronic alcoholism at the age of 11. During a neurological examination, he showed signs of damage to the pyramidal tract, as well as pronounced cognitive impairment with profound mental retardation, including delayed psycho-speech development. The 13-year-old girl suffers from severe mental retardation with speech impairment. In both cases, ACC was associated with epilepsy with a seizure frequency ranging from 6 times a year in the girl and up to 15 times a month in the boy. The gross neurological and cognitive deficits cause social difficulties since such patients need rehabilitation and ongoing care. In all cases, the diagnosis of ACC is based on the results of brain MRI, which is the method of choice. MRI enables assessment of the CC anatomical structure and the presence of other brain abnormalities. Complete agenesis was established in 3 cases, including a girl, and in one patient – a 12-year-old boy – partial agenesis with intact splenium was detected. In all patients, agenesis was combined with brain congenital malformations, namely with the absence of the septum pellucidum, interhemispheric and porencephalic cyst, basilar invagination, and venous malformation of the frontal lobe. Conclusion. ACC is a rare congenital brain malformation. According to the data, agenesis is more common in males. Complete ACC was diagnosed in 3 patients and partial ACC in 1. Risk factors include maternal alcohol consumption during pregnancy. The clinical presentation is diverse: from an asymptomatic course to severe cognitive impairment with severe and profound mental retardation, epilepsy, and autistic disorders with neurological deficits, including damage to the pyramidal tract. The primary diagnostic method is MRI, which detects anatomical changes in CC and other brain structures.

Altered Functional and Structural Connectivity in a Schizophrenia Patient With Complete Agenesis of the Corpus Callosum

Chudley McCullough syndrome is characterized by partial agenesis of the corpus callosum, interhemispheric cyst, cerebral and cerebellar cortical dysplasias, and hydrocephalus. This syndromic form of sensorineural hearing loss is rare. Our literature search has located 13 siblings in 6 families with this syndrome. We report a case of Chudley McCullough syndrome and discuss the relevant literature. It is amply clear from the review of literature that treatment of ventricular dilatation or drainage of arachnoid cysts in these cases will not improve the sensorineural hearing loss. A 14-month-old female child presented with bilateral profound sensorineural hearing loss. Neuroimaging revealed partial agenesis of the corpus callosum, colpocephaly, and an interhemispheric arachnoid cyst. These associations suggested a diagnosis of Chudley McCullough syndrome.

The aim of this study was to analyze the demographics, clinical presentation, and surgical outcome of intracranial arachnoid cysts and to review the surgical options. This is a retrospective observational study of 56 cases of symptomatic arachnoid cysts among all age groups treated between 2004 and 2020 at the Bombay Hospital, Mumbai. Endoscopic fenestration, microsurgical cyst excision, and shunt insertion were the interventions performed. Clinical presentation, cyst reduction post-intervention, and complications were studied. The follow-up period varied from 1 month to 16 years. Statistical analysis was done for 43 patients with a minimum of 3 years' follow-up. In all, 75% of patients were <18 years of age. Of these, the majority were between the age of 1 and 10 years. There were 14 cases of temporal, 13 cases of retrocerebellar, 10 cases of quadrigeminal cistern, and 7 cases each of interhemispheric and suprasellar arachnoid cysts. The most common clinical presentation was headache and vomiting. Concomitant hydrocephalus was seen on imaging in 24 cases. Endoscopic fenestration of cyst was the most routinely performed procedure (35 cases). Four patients of endoscopic fenestration underwent a redo endoscopic procedure on follow-up. Post-operative reduction in cyst size was found to be significantly better after endoscopic fenestration (p < 0.05). Though the best available treatment for arachnoid cysts remains controversial, surgery has been found to be beneficial in symptomatic cases. Endoscopic fenestration is considered the first-line surgical option and it may be followed by shunt, if necessary. Shunts may be preferred in very young children where there is associated hydrocephalus/macrocephaly.

GPSM2 Mutations Cause the Brain Malformations and Hearing Loss in Chudley-McCullough Syndrome

In children, interhemispheric arachnoid cysts (IHACs) are rare lesions often associated with corpus callosum dysgenesis. It is still controversial about surgical treatments for IHACs. We aim to report our experience with pediatric IHAC patients and evaluate surgical courses and neurological developments. Pediatric IHACs treated between 2001 and 2021 were reviewed retrospectively. IHAC was observed until they represented rapid cyst enlargement or neurological symptoms. Cyst fenestration was done by microscope or endoscope, depending on the IHAC's location. Cyst size and corpus callosum dysgenesis were evaluated with neuroimaging. Neurological development was assessed from medical records at the last follow-up. Fifteen children received cyst fenestration surgery (mean age 11.4months). Eleven patients (73.3%) under observation showed rapid cyst enlargement in a short period (median 5months). Cysto-ventriculostomy (CVS) and cysto-cisternostomy (CCS) regressed the cyst size significantly (p = 0.003). The median follow-up duration was 51months (range 14-178months). Corpus callosum dysgenesis was observed in eleven patients (73.3%, complete = 5, partial = 6). Among eight patients (53.3%) having developmental delay, five patients (33.3%) showed speech delay, including one patient with intractable seizures. Pediatric IHACs frequently present within 1year after birth, with rapid cyst enlargement. CVS and CCS were effective in regressing the cyst size. Corpus callosum dysgenesis accompanied by IHAC might have a risk of language achievement; however, development delay could rely on multifactorial features, such as epilepsy or other brain anomalies.

We sought to categorize the structural brain anomalies associated with abnormalities of the corpus callosum and anterior and hippocampal commissures in a large cohort. Brain MR images of adult and pediatric patients from our institution and from a national support organization (the ACC Network) were retrospectively evaluated for the type and severity of commissural anomalies and the presence and type of other structural abnormalities. Of 142 cases that were reviewed, 82 patients had agenesis of the corpus callosum (ACC), while 60 had hypogenesis of the corpus callosum (HCC). Of the overall cohort, almost all had reduced white matter volume outside the commissures, the majority had malformations of cortical development (most commonly heterotopia or abnormal sulcation), many had noncallosal midline anomalies (including abnormal anterior or hippocampal commissures and interhemispheric cysts and lipomas), and several patients had abnormalities of the cerebellum or brainstem. Sixty-six patients had Probst bundles, which were more common in patients with ACC than in those with HCC. Probst bundles were present in all four patients who had ACC or HCC but no other midline, cortical, or posterior fossa anomalies. Isolated commissural anomalies were rare in the populations of patients examined. Most cases of ACC and HCC were associated with complex telencephalic, diencephalic, or rhombencephalic malformations. Reduced cerebral hemispheric white matter volume and malformations of cortical development were seen in more than half of the patients, suggesting that many commissural anomalies are part of an overall cerebral dysgenesis. ACC and HCC appear to lie along a dysgenetic spectrum, as opposed to representing distinct disorders.