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Neurology International Apr 2024Sacral Tarlov cysts (TCs), often asymptomatic, can cause significant pain and severe neurological dysfunction. Conventional treatments are generally associated with high...
Sacral Tarlov cysts (TCs), often asymptomatic, can cause significant pain and severe neurological dysfunction. Conventional treatments are generally associated with high recurrence and complication rates. Specifically, the substantial recurrence rates, which can reach as high as 50%, significantly impact long-term outcomes. Recent evidence increasingly supports the hypothesis that the formation of Tarlov cysts (TCs) may be associated with inflammatory processes within the nerve root sheath, further exacerbated by elevated cerebrospinal fluid (CSF) pressure. This retrospective study explores thecaloscopy, combined with surgical techniques, as a more effective alternative. We observed a total of 78 patients, 48 of whom underwent endoscopic fenestration of the arachnoid sheath in addition to microsurgical resection of the TC. We found that the fenestration of the arachnoid sheath at the level of lumbosacral spinal nerve root entry led to a significantly decreased risk of developing recurrent TCs (5/48 vs. 9/30). Only one of the patients suffered from a persistent new bladder dysfunction after microsurgical resection. This presented technique provides a promising treatment path for the future management of TCs, offering a safe and more effective treatment option compared to previous methods. Additionally, the advantages of the thecaloscopy provide pathophysiological implications regarding the development of perineural cysts.
PubMed: 38668130
DOI: 10.3390/neurolint16020033 -
The Neuroradiology Journal Apr 2024Arachnoid cysts are the most common incidentally discovered intracranial lesions on imaging and the most common cystic intracranial lesions. They may be developmental or...
Arachnoid cysts are the most common incidentally discovered intracranial lesions on imaging and the most common cystic intracranial lesions. They may be developmental or secondary. A relative lack of recent literature and any comprehensive radiological review on arachnoid cysts has led to a general lack of awareness among radiologists of symptomatic or complicated arachnoid cysts. This is particularly concerning in pediatric patients. While arachnoid cysts are asymptomatic in most cases, they can cause clinical symptoms in a minority of cases, especially when they occur in unusual sites. These include intraventricular locations where they may cause hydrocephalus, the basal cisterns where they may compress cranial nerves, the cerebellopontine angle where they have to be differentiated from a number of cystic lesions, the cavum septum pellucidum or cavum velum interpositum, the choroid fissure where they can entrap the temporal horn and compress the hippocampus, the posterior fossa where they need to be differentiated from other posterior fossa cystic lesions, and within the spinal canal where there is a concern for cord or nerve root compression. Larger cysts are more prone to complications such as mass effect, hemorrhage, and rupture. Hemorrhage and rupture often present with acute symptoms. Ruptured cysts lose their characteristic imaging appearance and can mimic several ominous pathologies. It therefore becomes vital to accurately diagnose these cases as complications of pre-existing arachnoid cysts for appropriate management. A detailed review of all diagnostic imaging aspects of arachnoid cysts will help fill in the existing information void on this important entity.
PubMed: 38649153
DOI: 10.1177/19714009241248746 -
Neurological Sciences : Official... Apr 2024We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos....
We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos. Further investigation revealed additional clinical features including academic struggles, mood swings, and cutaneous findings, prompting consideration of a neurocutaneous syndrome. Magnetic Resonance Imaging (MRI) revealed features consistent with tuberous sclerosis complex (TSC), including radial migration lines in the subcortical white matter and an incidental arachnoid cyst. Notably, this case exhibited a unique presentation with absence of typical TSC findings such as subependymal nodules or cortical tubers. Additionally, precocious puberty, rarely associated with TSC, was observed, suggesting a potential link between hypothalamic lesions and hormonal imbalance. This case underscores the importance of comprehensive evaluation in pediatric patients presenting with seemingly unrelated symptoms, as it may unveil underlying conditions necessitating tailored management strategies.
PubMed: 38637341
DOI: 10.1007/s10072-024-07523-7 -
Child's Nervous System : ChNS :... Jul 2024Prenatally diagnosed complex arachnoid cysts are very rare. While the true prenatal incidence is still unknown, they account for approximately 1% of intracranial masses... (Review)
Review
OBJECTIVES
Prenatally diagnosed complex arachnoid cysts are very rare. While the true prenatal incidence is still unknown, they account for approximately 1% of intracranial masses in newborns. They rarely exhibit rapid growth or cause obstructive hydrocephalus, but if they increase to such a dimension during pregnancy, the ideal management is not well established. We present our detailed perinatal experience, covering prenatal diagnosis, a compassionate delivery process, and neonatal stabilization. Finally, a thorough postnatal neurosurgical intervention was performed. Initially, our focus was on the gradual reduction of cyst size as a primary effort, followed by subsequent definitive surgical treatment.
METHODS
This case series shows the treatment course of three fetuses with antenatally diagnosed large arachnoid cysts. We present pre- and postnatal management and imaging, as well as the surgical treatment plan and the available clinical course during follow-up.
RESULTS
Two girls and one boy were included in the current review. All three cases presented with prenatally diagnosed complex arachnoid cysts that increased in size during pregnancy. The mean gestational age at delivery was 35 weeks (range 32 to 37 weeks), and all patients were delivered by a caesarian section. Increasing head circumference and compression of brain structures were indications for delivery, as they are associated with a high risk of excess intracranial pressures and CSF diapedesis, as well as traumatic delivery and maternal complications. All cysts were supratentorial in location; one expanded into the posterior fossa, and one was a multicompartment cyst. All children underwent an initial surgical procedure within the first days of life. To relieve cyst pressure and achieve a reduction in head circumference, an ultrasound-guided or endoscopic-assisted internal shunt with drainage of the cyst to the ventricles or subdural/subarachnoid space was inserted. Definite surgical therapy consisted of cyst marsupialization and/or cysto-peritoneal shunt implantation. All children survived without severe neurodevelopmental impairments.
CONCLUSION
With the cases presented, we demonstrate that the slow reduction of immense cyst size as an initial procedure until optimal requirements for final surgical treatment were achieved has proven to be optimal for neurological outcome. Special emphasis has to be taken on the delicate nature of premature newborn babies, and surgical steps have to be thoroughly considered within the interdisciplinary team.
Topics: Female; Humans; Infant, Newborn; Pregnancy; Arachnoid Cysts; Neurosurgical Procedures; Prenatal Diagnosis
PubMed: 38602531
DOI: 10.1007/s00381-024-06385-w -
Journal of Community Hospital Internal... 2023Multiple cranial nerve palsies frequently accompany hypoglossal nerve palsy, potentially indicating malignancy, such as lymphoma, nasopharyngeal carcinoma, or...
Multiple cranial nerve palsies frequently accompany hypoglossal nerve palsy, potentially indicating malignancy, such as lymphoma, nasopharyngeal carcinoma, or metastases. However, when solely the hypoglossal nerve is affected, the causes may involve Chiari malformation, arachnoid cyst, or infectious mononucleosis, suggesting a positive prognosis. Craniocervical junction tuberculosis (TB), is an uncommon cause of isolated hypoglossal nerve palsy and has been reported infrequently in the literature. Craniocervical junction tuberculosis accounts for only 0.5% of TB cases overall and 6% of extra-pulmonary TB cases. We present here one such case of a 17-year-old male of Indian origin with a subacute history of tongue deviation and neck pain. Additionally, the patient reported loss of weight and appetite. The patient had significant posterior cervical lymphadenopathy. Neurological examination revealed findings suggestive of right peripheral hypoglossal nerve involvement. Blood investigations showed lymphocytosis along with an elevated erythrocyte sedimentation rate of 45 mm/h and elevated lactate dehydrogenase levels of 325 U/L. Tuberculin skin testing was positive and sputum acid-fast staining confirmed acid-fast bacilli. Magnetic Resonance Imaging of the cervical spine revealed a soft tissue component in the prevertebral space measuring 3.5×4.8 cm with a right paraspinal component adjoining the hypoglossal canal with peripheral contrast enhancement. Histological findings on the lymph node showed granulomatous lymphadenitis, suggestive of tuberculosis. The patient was started on 4-drug anti-tubercular therapy consisting of Isoniazid, Rifampicin, Pyrazinamide, and Ethambutol for a period of 18 months. He was subsequently followed up for 6 months till the resolution of palsy. This case emphasizes the importance of thorough evaluation and a meticulous workup to identify the underlying cause of hypoglossal nerve palsy and the importance of considering tuberculosis as a potential cause of isolated hypoglossal nerve palsy in everyday practice.
PubMed: 38596544
DOI: 10.55729/2000-9666.1267 -
Annals of Medicine and Surgery (2012) Apr 2024PHACE syndrome is a rare neurocutaneous disorder characterized by large segmental hemangiomas on the face and is associated with multiple developmental defects. PHACE...
INTRODUCTION AND IMPORTANCE
PHACE syndrome is a rare neurocutaneous disorder characterized by large segmental hemangiomas on the face and is associated with multiple developmental defects. PHACE stands for posterior fossa malformations, hemangiomas, arterial abnormalities, cardiac defects, and eye anomalies, with the most common manifestation being hemangioma in the cervico-facial region in early childhood.
CASE PRESENTATION
The authors report a case of a 15-year-female with complaints of facial hemangioma which on multisystemic imaging showed features of central nervous system (CNS) anomalies that led to the diagnosis of PHACE syndrome. The patient was started on propanolol which decreased the size of hemangioma in follow-up visits.
CLINICAL DISCUSSION
The hemangioma in the frontotemporal and frontonasal area of the face are associated more with CNS and cardiovascular anomalies needing a detailed multisystem approach. CNS anomalies include posterior cerebral fossa enlargement, cystic dilation of the fourth ventricle, arachnoid cyst, and cerebellar hypoplasia which were present in our case. Propanolol is considered the first-line drug for facial hemangioma with reported evidence of remarkable improvement and good tolerance. However, regular follow-up of the patient is needed to rule out any recurrence.
CONCLUSION
PHACE syndrome, although being a rare occurrence, must be kept as a differential diagnosis in infants and children with facial hemangioma. Imaging modalities like MRI/magnetic resonance arteriography must be used to rule out possible associations related to PHACE syndrome and focus on early treatment to prevent possible complications.
PubMed: 38576986
DOI: 10.1097/MS9.0000000000001759 -
Veterinary Radiology & Ultrasound : the... Apr 2024The conformation of spinal arachnoid diverticula (SAD) and their clinical implications are poorly characterized in dogs. This retrospective cross-sectional study...
The conformation of spinal arachnoid diverticula (SAD) and their clinical implications are poorly characterized in dogs. This retrospective cross-sectional study describes different SAD conformations in dogs and aims to identify if there is an association between SAD conformation and clinical features, localization, syringomyelia (SM) presence, concurrent vertebral condition, treatment option, and short as well as long-term outcome. Sixty-two dogs were included (12 cervical and 50 thoracolumbar SAD). All dogs with a cervical SAD had a cranial tethered conformation and were not included in the statistical analysis. Half of the dogs with a thoracolumbar SAD were cranial tethered, and the other half were caudal tethered. SM associated with SAD had a moderate prevalence in the cervical region (58.3%) and a high prevalence in the thoracolumbar region (82%). All dogs with the presence of SM and caudal tethered SAD had a cranial positioned SM, and all dogs with SM and a cranial tethered SAD had a caudal positioned SM. The SM absolute length and SM length/L2 ratio were significantly higher (P = .018, respectively) in the caudal tethered SAD compared with the cranial tethered SAD. The short-term outcome was statistically different (P = .045) between caudal and cranial tethered thoracolumbar SAD, but not the long-term outcome (P = .062). Multivariable logistic regression identified thoracolumbar caudal tethered SAD conformation had a better short-term outcome (P = 0.017, OR: 0.043, CI: 0.003-0.563), independently of SM length measurements. SAD conformation in dogs can influence SM formation. A possible link between short-term outcome and SAD conformation was found, but further research is warranted.
PubMed: 38572892
DOI: 10.1111/vru.13365 -
Cureus Feb 2024We report the case of a 33-year-old male patient with no past medical history presenting to our tertiary referral center with progressive (two years) deficit of lower...
We report the case of a 33-year-old male patient with no past medical history presenting to our tertiary referral center with progressive (two years) deficit of lower limb motor impairment (2/5 Medical Research Council [MRC] scale) and sensory impairment. T2- and T1-weighted MRI images clarified the nature of the cyst from T3 to T8. In our case, surgical management was warranted to relieve tension over the spinal cord, thus improving symptoms. Two multilevel laminectomies were performed, one centered on the proximal pole and the other on the distal pole; subsequently, the epidural cyst was gradually folded until it was totally extracted without complications. In the present study, we discuss a technique of extended spinal compressive arachnoid cyst. To the best of our knowledge, this technique has not been previously described in the existing body of literature. Here, we present a case of a successful procedure that seems both efficient and safe. Further study will be required to confirm its safety and efficacy.
PubMed: 38550475
DOI: 10.7759/cureus.54978 -
Child's Nervous System : ChNS :... May 2024
Topics: Humans; Arachnoid Cysts; Hydrocephalus; Decompression, Surgical; Seizures; Magnetic Resonance Imaging
PubMed: 38538974
DOI: 10.1007/s00381-024-06378-9 -
Pediatric Neurosurgery Mar 2024Introduction The association between trigonocephaly and sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature [1,2]. However, the real...
Introduction The association between trigonocephaly and sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature [1,2]. However, the real incidence of this association and its clinical relevance remain unknown. Methods The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS from January 2014 to June 2023. Results During the study period, 136 patients with trigonocephaly underwent surgery. Analysis of the clinical charts revealed that in 39.7% of the cases (54/136), pre-operative CT scan depicted the presence of a sylvian fissure arachnoid cyst. Of these, AC was bilateral in 23 cases and unilateral in the remaining 31. All unilateral ACs were on the left side. The ACs were classified as Galassi grade I in 52 cases (96.3%) and Galassi grade II in 2 cases (3.7%)[3]. Interestingly, in one case we reported a Galassi grade I AC enlargement during follow-up, thereby necessitating surgical fenestration. Conclusion ACs and trigonocephaly are well-known conditions for pediatric neurosurgeons; however, their association is poorly defined. Despite the lack of reports on the incidence and clinical significance of this association, it is worth knowing that radiological follow-up is essential be in monitoring AC evolution.
PubMed: 38531328
DOI: 10.1159/000538559