Brain Aneurysms in the Pediatric Population of Slovenia: A Case Series

2019 ◽  
Vol 50 (03) ◽  
pp. 188-192
Author(s):  
Saša Ilovar ◽  
Mirjana Benedik ◽  
Tina Vesnaver ◽  
Damjan Osredkar
Keyword(s):  
Clinical Presentation ◽  
Pediatric Population ◽  
Case Series ◽  
Medical Documentation ◽  
Brain Aneurysm ◽  
Male Predominance ◽  
Presenting Symptoms ◽  
Asymptomatic Patients ◽  
Brain Aneurysms

Background Brain aneurysms are rare in the pediatric population. The diagnosis of a brain aneurysm in a child may be difficult because of its infrequency and often subtle or nonspecific clinical presentation. Endovascular therapy and microsurgical treatment are increasingly used approaches in treating children, possibly contributing to favorable outcomes if patients are treated in a timely manner. Objective We were interested in the clinical presentation, symptoms, diagnostics, treatment, and follow-up of pediatric patients with brain aneurysms in Slovenia. Methods This was a retrospective review of medical documentation of children with intracranial aneurysms treated at the University Children's Hospital in Ljubljana, Slovenia, from January 1998 to December 2017. Results We identified a cohort of eight children (median age: 14.9 years; range: 2.8–17.7). The estimated incidence of pediatric brain aneurysms in Slovenia is 0.12/100,000 children per year. We observed a male predominance (1.7:1). Half of the patients presented with acute onset of neurologic symptoms and three with subarachnoid hemorrhage. One of the patients had a related stroke. The presenting symptoms were tonic–clonic seizures, hemiparesis, paresthesias, speech disturbance, and cranial nerve palsy. The other half of aneurysms were identified incidentally. Five patients had anterior circulation aneurysms; the most prevalent location was the internal carotid artery. One patient was treated with surgical procedures, four patients were treated with endovascular procedures, and three patients were treated conservatively. Outcome was excellent in all patients. Conclusion Endovascular interventions and microsurgical procedures appear to be safe and effective in the treatment of brain aneurysms in the pediatric population. Asymptomatic patients with brain aneurysms need close follow-up.

scholarly journals Transnasal endoscopic approach for pediatric skull base lesions: a case series

2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  
Keyword(s):  
Skull Base ◽  
Pediatric Population ◽  
Case Series ◽  
Length Of Hospital Stay ◽  
Skull Base Tumors ◽  
Presenting Symptoms ◽  
Institutional Experience ◽  
Transnasal Endoscopic Approach ◽  
Skull Base Lesions

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.

Oral Management for Pediatric Lyme Meningitis

2018 ◽  
Vol 8 (3) ◽  
pp. 272-275
Author(s):  
Santiago M C Lopez ◽  
Brian T Campfield ◽  
Andrew J Nowalk
Keyword(s):  
Oral Therapy ◽  
Chart Review ◽  
Clinical Presentation ◽  
Pediatric Population ◽  
Case Series ◽  
Parenteral Therapy ◽  
Oral Antibiotics ◽  
Serious Adverse Events ◽  
Parenteral Route

Abstract Background Guidelines for pediatric Lyme meningitis recommend treatment with parenteral therapy [1, 2]. Adult studies suggest that Lyme meningitis can be successfully treated with oral therapy. Our objective was to evaluate the clinical response, side effects and outcome of oral therapy for Lyme meningitis in the pediatric population compared with parenteral therapy in an area endemic for Lyme disease. Methods We conducted a case series chart review from January 2012 to May 2017 of pediatrics patient diagnosed and treated for Lyme meningitis. We recorded clinical presentation, laboratory values, antimicrobial therapy and follow up after therapy to compare the efficacy of oral versus parenteral route of therapy. Results We identified 38 patients diagnosed with Lyme meningitis. Thirty-two patients were discharge with exclusively oral therapy with: doxycycline and amoxicillin. We had only 2 patients developed potential adverse effects from oral doxycycline therapy. All patients treated with oral antibiotics had resolution of symptoms on follow up appointments. Conclusions Oral therapy for Lyme meningitis yields no serious adverse events, was well tolerated and showed resolution of symptoms.

P4657Cardiac myxomas: are we dealing with distinct clinical entities?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Sa Mendes ◽  
J Abecasis ◽  
A Ferreira ◽  
R Ribeiras ◽  
C Reis ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Significant Proportion ◽  
Case Series ◽  
Surgical Excision ◽  
Non Invasive ◽  
Cardiac Tumours ◽  
Asymptomatic Patients ◽  
Grade 5 ◽  
Cardiac Myxomas

Abstract Background Cardiac myxomas are rare, despite being the most common primary cardiac tumours. A significant number of myxomas are discovered accidentally in asymptomatic patients (pts), as there is increased use of non-invasive cardiac imaging. Our aim was to describe the experience of a cardiac surgery centre managing cardiac myxomas during the last 28 years. Methods Single-center retrospective study of consecutive pts admitted with the diagnosis of a cardiac myxomas between 1990 and 2018. Registry data concerning clinical presentation, non-invasive imaging assessment and definitive histopathology were collected. Results From 154 pts with the diagnosis of cardiac tumours, we identified 106 (68.8%) myxomas (67% females; mean age at diagnosis 61,5±13,1 years). Myxoma diagnosis increased throughout the 3 decades (27 cases until 2000; 26 cases in the second decade; 52 cases from 2010 until present). 30% of the pts were asymptomatic at diagnosis. Obstructive symptoms (heart failure and syncope) and embolic events were the most common complaints among symptomatic pts. Transthoracic echocardiography firstly identified the tumours in 88% of the cases. Cardiac magnetic resonance and computed tomography were performed for further investigation in 7% of the cases. Presumptive pre-operative diagnosis was correct in 83.8% pts. Surgical excision was successfully achieved in all cases. 89% of the tumours were located in the left atrium with inter-atrial septum implantation (13 in right chambers; 1 valvular tumour). There were 10 multifocal tumours. At histopathology myxomas were grossly described as mucous jelly appearance (80%), solid (15%) and mixed type lesions (5%). Rare histologic findings were described in 30% of the cases (8 tumours with bone tissue; 1 with forming bone marrow; 4 with endocrine type glandular epithelium; 16 with lympho-plasmocytic infiltrates; 3 with high mitotic grade; 5 with concomitant thrombus). For a median follow up of 86 [31–214] months there were 15 deaths (2 of them with tumour related deaths). There were 3 recurrences (2 with high mitotic grade histology), mostly occurring 3 years after the first intervention. Conclusion In this case series cardiac myxomas are the most common cardiac tumours, with a significant proportion of asymptomatic lesions. Clinical heterogeneity followed polymorphic histology, with recognized differences when compared to classical descriptions of this kind of tumour.

scholarly journals A Case Series on Genotype and Outcome of Liver Transplantation in Children with Niemann-Pick Disease Type C

Children ◽  
2021 ◽  
Vol 8 (9) ◽  
pp. 819
Author(s):  
Line Modin ◽  
Vicky Ng ◽  
Paul Gissen ◽  
Julian Raiman ◽  
Eva Doreen Pfister ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Genetic Testing ◽  
Clinical Presentation ◽  
Case Series ◽  
Presenting Symptoms ◽  
Niemann Pick Disease ◽  
Type C ◽  
Niemann Pick ◽  
Pick Disease

Background: To report on clinical presentation and outcomes of children who underwent liver transplantation (LTx) and were subsequently diagnosed to have Niemann-Pick type C (NPC). Methods: Retrospective, descriptive, multi-centre review of children diagnosed with NPC who underwent LTx (2003–2018). Diagnosis was made by filipin skin test or genetic testing. Results: Nine children were identified (six centres). Neonatal acute liver failure was the most common indication for LTx (seven children). Median age at first presentation: 7 days (range: 0–37). The most prevalent presenting symptoms: jaundice (8/9), hepatosplenomegaly (8/9) and ascites (6/9). 8/9 children had a LTx before the diagnosis of NPC. Genetic testing revealed mutations in NPC1 correlating with a severe biochemical phenotype in 5 patients. All 9 children survived beyond early infancy. Seven children are still alive (median follow-up time of 9 (range: 6–13) years). Neurological symptoms developed in 4/7 (57%) patients at median 9 (range: 5–13) years following LTx. Conclusion: Early diagnosis of NPC continues to be a challenge and a definitive diagnosis is often made only after LTx. Neurological disease is not prevented in the majority of patients. Genotype does not appear to predict neurological outcome after LTx. LTx still remains controversial in NPC.

Symptomatic spontaneous vertebrobasilar dissections in children: review of 29 consecutive cases

2010 ◽  
Vol 6 (3) ◽  
pp. 233-243 ◽  
Author(s):  
Dittapong Songsaeng ◽  
Kittipong Srivatanakul ◽  
Timo Krings ◽  
Sasikhan Geibprasert ◽  
Augustin Ozanne ◽  
...  
Keyword(s):  
Basilar Artery ◽  
Clinical Presentation ◽  
Clinical Symptoms ◽  
Pediatric Population ◽  
Morphological Characteristics ◽  
Clinical History ◽  
Mass Effect ◽  
Male Predominance ◽  
Presenting Symptoms ◽  
Imaging Appearance

Object The purpose of this study is to analyze the clinical presentation, morphological characteristics, angio-architecture, and outcome of vertebrobasilar dissection (VBD) in the pediatric population. Methods The authors retrospectively reviewed 29 consecutive cases involving children younger than 16 years of age who were diagnosed with symptomatic VBDs. Data were gathered with respect to the patient's age, sex, clinical history, associated underlying disease, and symptoms (headache, vertigo) as well as the location of the dissection and the imaging appearance. Results The patients' mean age was 8.24 years (range 2 months–15 years). There was an overall 3:1 male predominance, although among children older than 8 years, girls and boys were similarly affected. Hemorrhagic dissections occurred in 10 of 29 cases. In nonhemorrhagic dissections, stroke occurred in 16 cases, with the most common presenting symptoms being headaches and vertigo; in the other 3 cases, mass effect due to a chronic dissecting aneurysm was present. In 7 children an underlying vessel wall disease was found. The location of the dissection was extradural in 11 cases and intradural in the remainder. There was no preference with respect to side. The basilar artery was affected in 9 patients. Conclusions The imaging appearance and clinical presentation of symptomatic VBDs in the pediatric population differs from that in adults. Boys are more often affected, especially at younger ages, and hemorrhagic presentation is more common, presumably owing to the fact that the basilar artery is more commonly involved. Depending on the pathogenetic mechanism underlying the dissection, different clinical symptoms will evolve, necessitating individually tailored treatment.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Treatment and therapeutic strategies for pituitary apoplexy in pregnancy: a case series

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga
Keyword(s):  
Pituitary Apoplexy ◽  
Clinical Symptoms ◽  
Case Series ◽  
Optimal Timing ◽  
Presenting Symptoms ◽  
Pathological Evaluation ◽  
Mother And Child ◽  
Life Threatening ◽  
In Pregnancy

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.

scholarly journals A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation

2021 ◽  
Vol 50 (1) ◽  
Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Clinical Presentation ◽  
Tertiary Care ◽  
Choanal Atresia ◽  
Case Series ◽  
Retrospective Chart Review ◽  
National Study ◽  
Feeding Difficulties ◽  
Presenting Symptoms ◽  
Tertiary Care Centers

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract

Secretory Carcinoma in Children and Young Adults: A Case Series

2021 ◽  
pp. 109352662110469
Author(s):  
Caroline T Simon ◽  
Jonathan B McHugh ◽  
Raja Rabah ◽  
Amer Heider
Keyword(s):  
Case Series ◽  
Salivary Gland Neoplasm ◽  
Low Grade ◽  
Secretory Carcinoma ◽  
Patients Âgés ◽  
Male Predominance ◽  
Small Series ◽  
Children And Young Adults ◽  
Grade Malignancy

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.

scholarly journals Index event of cerebral amyloid angiopathy (CAA) determines long-term prognosis and recurrent events (retrospective analysis and clinical follow-up)

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Andrea Wagner ◽  
Christiane Groetsch ◽  
Sibylle Wilfling ◽  
Karl-Michael Schebesch ◽  
Mustafa Kilic ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Recurrent Events ◽  
Clinical Presentation ◽  
Secondary Prophylaxis ◽  
Amyloid Angiopathy ◽  
Index Event ◽  
Asymptomatic Patients ◽  
Cerebral Amyloid

Abstract Background The modified Boston criteria (mBC) define the probability for the diagnosis of cerebral amyloid angiopathy (CAA). Its initial clinical presentation differs from asymptomatic cerebral microbleedings (cMBs), acute ischemic stroke (AIS), cortical hemosiderosis (cSS), to lobar ICH (lICH). Methods Retrospective analyses and clinical follow-ups of individuals with at least mBC “possible” CAA from 2005 to 2018. Results 149 patients were classified in subgroups due to the index event: lICH (n = 91), AIS (n = 32), > 3 cMBs only (n = 16) and cSS (n = 10). Patients in the lICH subgroup had a significantly higher percentage of single new lICHs compared to other groups, whereas patients in the AIS-group had a significantly higher percentage of multiple new AIS. cMBs as index event predisposed for AIS during follow up (p < 0.0016). Patients of the cMBs- or cSS-group showed significantly more TFNEs (transient focal-neurological episodes) and lower numbers of asymptomatic patients (for epilepsy and TFNEs) at the index event than patients with lICH or AIS (p < 0.0013). At long-term follow-up, the cMBs- and cSS-group were characterized by more TFNEs and fewer asymptomatic patients. Conclusions A new classification system of CAA should add subgroups according to the initial clinical presentation to the mBCs allowing individual prognosis, acute treatment and secondary prophylaxis.

Sign in / Sign up

Export Citation Format

Share Document