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Abstract: Background Intracranial aneurysms are rare in the pediatric age group, especially in neonates. This article presents a novel case of a neonate with a dissected and ruptured giant anterior cerebral artery aneurysm, focusing on diagnosis and endovascular treatment with a review of the literature. Case description A full-term female neonate presented with projectile vomiting, fever, and hyperkinetic movements. Diagnostic workup revealed a giant dissecting and partially thrombosed aneurysm along the anterior cerebral artery. The neonate underwent successful endovascular coil embolization. Results The intervention led to the cessation of symptoms, and the patient was discharged and followed with mild hypotonia as the only neurological deficit at 9 months. Conclusion This case highlights the rarity and complexity of diagnosing and treating giant dissected anterior cerebral artery aneurysms in neonates. It underscores the importance of considering intracranial aneurysms in differential diagnosis for neonates with relevant symptoms and suggests endovascular embolization as an effective treatment option. PubDate: 2024-08-09 DOI: 10.1007/s00381-024-06534-1
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Abstract: Purpose Unresectable pediatric low-grade gliomas (LGG) usually need adjuvant therapy, and carboplatin hypersensitivity reaction (HR) commonly leads to premature treatment cessation of a standard chemotherapy regimen. In the molecular era, advances in understanding tumor genetic characteristics allowed the development of targeted therapies for this group of tumors; however, cost-effectiveness assessment of treatments, especially in low-income countries, is crucial. The aim is to describe the results of carboplatin desensitization protocol in a single center in a middle-income country. Method Prospective analysis of children with LGG submitted to carboplatin desensitization from December 2017 to June 2020 with follow-up until April 2024. Results Nine patients were included. The mean age was 11 years. Five patients were male. Seven had optic pathway and two cervicomedullary location. Six had histologic diagnosis and four molecular analyses. The incidence of carboplatin reactions during the study period was 39.1%. Six patients underwent skin prick test, three with positive results. The first HR occurred, on average, around the 9th cycle of treatment. All patients had cutaneous symptoms, and five out of nine had anaphylaxis as the first reaction. 77.7% of the patients completed the protocol, and the clinical benefit rate (stable disease and partial response) was 88.8%. Six patients further required other lines of therapy. Monthly, the total cost for carboplatin was $409.09, and for target therapies (dabrafenib plus trametinib), $4929.28 to $5548.57. Conclusion Our study presented an interesting and cost-effective option where desensitization allowed children with HR to be treated with first-line therapy, avoiding the discontinuation of an effective treatment. PubDate: 2024-08-07 DOI: 10.1007/s00381-024-06564-9
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Abstract: Purpose Endoscopic third ventriculostomy (ETV) is an effective treatment for obstructive hydrocephalus. Secondary stoma closure may be life threatening and is the most common reason for late ETV failure, mostly secondary to local scarring. Local stents intended to maintain patency are rarely used. In this study, we summarize our experience using stented ETV (sETV), efficacy, and safety. Material and methods Data was retrospectively collected from all consecutive patients who underwent ETV with stenting at four centers. Collected data included indications for using sETV, hydrocephalic history, surgical technique, outcomes, and complications. Results Sixty-seven cases were included. Forty had a primary sETV, and 27 had a secondary sETV (following a prior shunt, ETV, or both). The average age during surgery was 22 years. Main indications for sETV included an adjacent tumor (n = 15), thick or redundant tuber cinereum (n = 24), and prior ETV failure (n = 16). Fifty-nine patients (88%) had a successful sETV. Eight patients failed 11 ± 8 months following surgery. Reasons for failure included obstruction of the stent, reabsorption insufficiency, and CSF leak (n = 2 each), and massive hygroma and tumor spread (n = 1 each). Complications included subdural hygroma (n = 4), CSF leak (n = 2), and stent malposition (n = 1). There were no complications associated with two stent removals. Conclusion Stented ETV appears to be feasible and safe. It may be indicated in selected cases such as patients with prior ETV failure, or as a primary treatment in cases with anatomical alterations caused by tumors or thickened tuber cinereum. Future investigations are needed to further elucidate its role in non-communicating hydrocephalus. PubDate: 2024-08-05 DOI: 10.1007/s00381-024-06566-7
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Abstract: Background Cerebral aneurysms in children have a low incidence and accounts for less than 4% of all cerebral aneurysms. These aneurysms have been linked to various factors. Severe headache, seizures, and motor-sensory deficits are common presentations. Case report We describe the case of a 2-month-old male patient who presented with generalized tonic–clonic seizures for 4 days. At the hospital, he was stabilized with ventilatory support, sedation, and antiepileptic drugs. A NCCT (Head) showed intraparenchymal hemorrhage in the left fronto-parieto-temporal lobe and subarachnoid hemorrhage. Subsequently a CT angiogram revealed an aneurysm of the left M3 segment of MCA. Successfully, the patient underwent microsurgical clipping of aneurysm and evacuation of hematoma. Conclusions Pediatric cerebral aneurysms differ from their adult counterparts, mainly in their etiology and evolution. As per literature, aneurysmal clipping and neurological endovascular therapy have shown similar results. PubDate: 2024-08-02 DOI: 10.1007/s00381-024-06558-7
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Abstract: Purpose We examined differences in long-term morphometric outcomes of spring-mediated cranioplasty (SMC) for various forms of isolated nonsyndromic sagittal craniosynostosis. Methods A retrospective review was performed of children who underwent SMC from 2011 to 2020 at the Children’s Hospital of Philadelphia. Cephalic indices (CI), Whitaker grades, parietal bone thickness, and degree of suture fusion were assessed. Frontal bossing and vertex-nasion-opisthocranion (VNO) angles were compared to a normal control group. Results Fifty-four subjects underwent surgery at age 3.6 ± 1.0 months with follow-up of 6.3 ± 1.8 years. Mean CI was 75.2 ± 4.1 at 5.9 ± 2.0 years postoperatively. Mean CI were 75.8 ± 4.1 (n = 32), 76.4 ± 4.0 (n = 22), and 77.1 ± 4.8 (n = 11) at 5, 7, and 9+ years postoperatively, respectively. Three (5.6%) required reoperation for persistent scaphocephalic cranial deformity. Fifty-one (94.4%) were Whitaker Grade I. On physical examination, 12 (22.2%) demonstrated craniofacial abnormalities. At long-term follow-up, there were no differences in frontal bossing angle (102.7 ± 5.2 degrees versus 100.7 ± 5.6 degrees, p = .052) and VNO angle (44.9 ± 3.3 degrees versus 43.9 ± 2.2 degrees, p = .063) between study and control groups. Younger age at surgery predicted a lower Whitaker grade, more normalized VNO angle, and greater change in CI during active expansion. Increased percentage fused of the posterior sagittal suture predicted a higher Whitaker grade, while decreased anterior fusion was associated with frontal bossing and temporal hollowing. Conclusions Overall, children undergoing spring-mediated cranioplasty for sagittal craniosynostosis demonstrated maintenance of CI, favorable cosmetic outcomes, and a low reoperation rate at mid-term follow-up. Early intervention is associated with improved aesthetic outcomes, and regional fusion patterns may influence long-term craniofacial dysmorphology. PubDate: 2024-08-02 DOI: 10.1007/s00381-024-06531-4
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Abstract: Abstract Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06410-y
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Abstract: Purpose Access to the pineal region has always represented a fascinating challenge to the neurosurgeons. Almost equally thrilling is the historical evolution from the hard beginnings with extremely high mortality rates to the current surgical outcomes, with excellent resection rates without long-term morbidity for most of the patients. The purpose of this paper is to provide an overview of the historical evolution of the occipital interhemispheric transtentorial (OITT) approach and its role in the development of access to the pineal region. Methods Review of the literature highlights the occipital transtentorial approach from the historical context prior its description and the beginning to the current modifications and new recent insights. Results The occipital transtentorial approach described initially by Poppen in 1966 has played a key role in the progress and success accessing the pineal area. Conclusion This historical review aims to highlight the extraordinary effort of those neurosurgeons who guided and led the development of one of the most important approaches to the pineal region. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06508-3
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Abstract: Abstract An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06423-7
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Abstract: Objective To compare cases of dysraphism with ventral tethering of cord with those with dorsal tethering and to find out any differences in the outcome of surgery in them. Methods We collected the data of 188 consecutively operated tethered cord patients at our institute in the past 7 years and divided them into ventral tethering and dorsal tethering groups. Those that we felt had both dorsal and ventral tethering were excluded. Their preoperative clinical, radiological, and baseline neurophysiological parameters as well as postoperative clinical and radiological parameters were analyzed in a retrospective study. Results Among the 188 tethered cord patients, 52 (28%) had ventral tethering and 136 (72%) had posterior tethering. Preoperative neurodeficit and cord signal changes as well as absent baseline MEP (of any one muscle) were significantly more associated with ventral tethered cord than the dorsal tethered cord. The neurological deterioration after surgery occurred significantly in the ventral tethered cord group than in the dorsal tethered cord group. Also, the postoperative MRI had more incomplete detethering cases in the ventral group than in the dorsal tethered cord group. Conclusion Ventral tethered cord is more likely to present with preoperatively neurological deficits. It should be carefully identified in the preoperative MRI, so that the intraoperative difficulties in complete detethering and postoperative deterioration can be anticipated. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06438-0
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Abstract: Abstract Pallister–Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently caused by mosaic supernumerary isochromosome 12p. Here, we report a 27-month-old girl with a prenatal diagnosis of PKS and a histopathological diagnosis of pineocytoma. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06426-4
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Abstract: Background Intracranial arachnoid cysts are benign collections of cerebrospinal fluid that are often asymptomatic and discovered incidentally. An interhemispheric location of these lesions is rare, with only a few such cases reported in the literature. Though spontaneous regression of arachnoid cysts has been described in other locations, to date this phenomenon has not been reported in interhemispheric fissure cysts. Observations In this report, we describe a patient with a large, multiloculated interhemispheric arachnoid cyst diagnosed on prenatal ultrasound. She did not exhibit neurologic deficits or signs of increased intracranial pressure and was observed with serial imaging. After several years of observation, imaging revealed spontaneous and progressive decrease in the cyst size. Lessons We illustrate a case of regression of an interhemispheric arachnoid cyst in a pediatric patient. To our knowledge, this is the first reported case of spontaneous shrinkage of an arachnoid cyst in this location. Although the current presentation is rare, this reporting adds to the current understanding of natural history of arachnoid cysts and provides an example of radiographical improvement without intervention of a cyst located within the interhemispheric fissure. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06464-y
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Abstract: Purpose To describe the relation of the sphenoid ridge (SR) with the surrounding anatomical structures in healthy children when approaching the anterior and middle fossae. Methods Computed tomography of 180 pediatric patients (90 males / 90 females), aged 1–18 years were included the study. The size of the lesser wing (LW) and the distances of the LW to certain landmarks were measured. Results The LW length was 28.48 ± 8.15 mm. The LW widths at the midline and the midpoint and lateral point of the SR were 7.78 ± 1.74 mm, 2.84 ± 0.81 mm, and 1.91 ± 0.64 mm, respectively. The distance between the midpoint of the SR and the crista galli was 28.22 ± 5.56 mm, and the distance between the crista alaris and internal auditory meatus was 51.73 ± 5.79 mm. The linear function was calculated as y = 18.748 + 1.024 × age for SR length, y = 6.046 + 0.182 × age for the midline width of SR, y = 2.367 + 0.050 × age for the midpoint width of SR, y = 1.249 + 0.069 × age for the crista alaris width, y = 21.727 + 0.683 × age for the distance between the SR midpoint and the crista galli, and y = 43.614 + 0.855 × age for the distance between the crista alaris and internal auditory meatus. Conclusion All measured parameters increased irregularly with advancing age. Furthermore, our regression equations representing the growth dynamics of SR may be used to estimate these parameters. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06391-y
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Abstract: Abstract A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case report, we describe a female newborn with an extremely rare mature cystic teratoma in the thoracolumbar region. She presented prenatally with a preliminary diagnosis of meningomyelocele, diastematomyelia, and Chiari II malformation and a possible teratoma. However, a mass containing solid glandular tissues and bony calcifications approximately 3 × 4 cm in size was observed in the thoracolumbar region upon birth. During surgical resection, no nerve roots were found in the associated meningocele. The patient retained full lower body function postoperatively following surgical excision of the thecal sac and teratoma. PubDate: 2024-08-01 DOI: 10.1007/s00381-024-06396-7
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