scholarly journals The surgical resection of dysplastic cerebellar gangliocytoma assisted by intraoperative sonography: illustrative case

2021 ◽  
Vol 2 (14) ◽  
Author(s):  
Yiming Li ◽  
Jiahe Guo ◽  
Huijie Wei ◽  
Cuiyun Sun ◽  
Yan Chai ◽  
...  

BACKGROUND Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is an extremely rare, slow-growing hereditary mass lesion that is mainly characterized by both specific neuroradiological features and secondary hydrocephalus. Patients may present with symptoms of cerebellar mass lesion and increased intracranial pressure. As an important part of Cowden syndrome, Lhermitte-Duclos disease in adults is typically marked by PTEN gene mutation. OBSERVATIONS The clinical management of a 31-year-old woman who suffered Lhermitte-Duclos disease was introduced in this case report. Subtotal resection was performed with the assistance of intraoperative sonography to relieve obstructive hydrocephalus, and prophylactic C1 laminectomy was performed to prevent possible postoperative progression of the residual lesion. Perioperative care and surgical process were clearly revealed in an accompanying video. Intraoperative sonography of Lhermitte-Duclos disease presents hyperechoic distorted thickening cortices surrounded by hypoechoic edema belt. The patient did not report any significant neurological complications or sequelae after the lesion resection. LESSONS The authors first reported the use of intraoperative sonography in resection of adult-onset Lhermitte-Duclos disease. Hopefully, the educative case report can provide a feasible experience in the diagnosis and treatment of Lhermitte-Duclos disease.

2019 ◽  
Vol 38 (04) ◽  
pp. 319-323
Author(s):  
Mylena Miki Lopes Ideta ◽  
Mylla Christie Oliveira Paschoalino ◽  
Louise Makarem Oliveira ◽  
Nelson Brancaccio dos Santos ◽  
Marco Rodrigo Valdivia Sanz ◽  
...  

AbstractLhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group of genetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are considered major criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.


2015 ◽  
Vol 16 (1) ◽  
pp. 54-57 ◽  
Author(s):  
Maria M. Santos ◽  
Mark M. Souweidane

The authors report an illustrative case of a purely endoscopic surgical approach to successfully remove a solid choroid plexus papilloma of the third ventricle in an infant. A 10-week-old male infant first presented with transient episodes of forced downward gaze, divergent macrocephaly, a tense anterior fontanel, diastasis of the cranial sutures, and papilledema. Brain MRI revealed a small, multilobulated contrast-enhancing mass situated within the posterior third ventricle, with resultant obstructive hydrocephalus. A purely endoscopic removal of the tumor was performed through a single right frontal bur hole. Intraoperatively, a unique vascular tributary was recognized coming from the tela choroidea and was controlled with coagulation and sharp dissection. Postoperative MRI confirmed complete tumor removal, and the tumor was classified as a choroid plexus papilloma. There has been no evidence of tumor recurrence over 42 months of follow-up. With this case report the authors intended to show that endoscopic surgery can be an additional tool to consider when planning a choroid plexus tumor approach. It seems to be of particular interest in selected cases in which there are concerns about the patient's total blood volume, as in infants with potential hemorrhagic tumors and when it is possible to preoperatively identify a single vascular pedicle that can be approached early in the surgery.


1986 ◽  
Vol 26 (4) ◽  
pp. 391-394 ◽  
Author(s):  
C.L. Pappas ◽  
R.G. Quisling ◽  
W.E. Ballinger ◽  
L.C. Love

2013 ◽  
Vol 21 (3-4) ◽  
pp. 139-140
Author(s):  
Sunita Shere ◽  
Anjali Kulkarni ◽  
Shubhjyoti Pore ◽  
Rajan Bindu

Testicular fibroma of gonadal stromal origin is a rare benign tumor of testis, which usually presents as a slow growing testicular mass. Intratesticular fibroma of gonadal stromal origin, with or without minor sex cord elements, must be considered, analogous to similar tumors in ovary, as a benign tumor. Until now, only 25 cases of testicular fibroma have been reported in the literature. We reported a case of testicular fibroma in a 20 years male who presented with painless right testicular enlargement since two years. Ultrasonography (USG) showed heterogeneous mass in right scrotum suggestive of testicular malignancy. Right orchidectomy was done. Histopathological diagnosis was testicular fibroma, which was confirmed by immunohistochemistry.


Author(s):  
Sebastião Silvério Sousa-Neto ◽  
José Alcides Almeida de Arruda ◽  
Allisson Filipe Lopes Martins ◽  
Lucas Guimarães Abreu ◽  
Ricardo Alves Mesquita ◽  
...  

2021 ◽  
Author(s):  
Han Na Jang ◽  
Sa Hong Kim ◽  
Young Min Cho ◽  
Do Joong Park

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuyan Nie ◽  
Weimin Zhou ◽  
Shaoqiang Huang

Abstract Background The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia. Case presentation Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. She had high risks of difficult airway and aspiration. The injection of bolus drugs through the spinal or epidural needle may worsen the previous neurological complications. The patient was well evaluated with a multidisciplinary technique before surgery and the anesthesia was provided by a skilled anesthesiologist with slow spinal injection. Conclusions An interdisciplinary team approach is needed to weigh risks and benefits for patients with CAAD and ACM-1 undergoing cesarean delivery. Therefore, an individual anesthetic plan should be made basing on the available anesthetic equipments and physicians’ clinical experience on anesthetic techniques.


Disabilities ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 1-22
Author(s):  
Roslyn Livingstone ◽  
Ginny Paleg

Recent advances in medical interventions have changed the prognosis for children with infantile-onset spinal muscular atrophy (SMA-1); however, little has been published regarding rehabilitation management. A rapid scoping review was conducted in November 2020 using Medline and CINAHL databases. Evidence supporting use of assistive devices and equipment to enhance participation, mobility, function, and posture in lying, sitting, and standing positions was sought. From 239 articles, only five studies (describing use of augmentative communication, manual and power mobility, supported standing and orthotic devices) met inclusion criteria. Results are presented alongside a case report of a 5-year-old boy (treated with Nusinersen since 7 months-of-age) who uses a variety of devices to enhance his activity and participation in family life. While reclined and tilted sitting positions as well as power mobility were previously considered for children with SMA-1, this child has progressed to supported upright standing, self-propelling a lightweight manual wheelchair indoors, communicating using multiple methods and taking steps in a dynamic mobility device. Power mobility was introduced in a switch-adapted cart at 11 months and he was independently exploring indoors and outside in his power wheelchair before 20 months. Research evidence is limited, but alongside the case report highlights the importance of a comprehensive and proactive approach to enhancing function, fun and participation with family and friends through adaptive equipment for children with significant and life-limiting disabilities.


2007 ◽  
Vol 43 (6) ◽  
pp. 526-530 ◽  
Author(s):  
Marcus C. Korinth ◽  
Alexandra Kapser ◽  
Martin R. Weinzierl

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