scholarly journals Rathke cleft cysts in pediatric patients: presentation, surgical management, and postoperative outcomes

2011 ◽  
Vol 31 (1) ◽  
pp. E3 ◽  
Author(s):  
Arman Jahangiri ◽  
Annette M. Molinaro ◽  
Phiroz E. Tarapore ◽  
Lewis Blevins ◽  
Kurtis I. Auguste ◽  
...  

Object Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC. Methods The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008. Results In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5). Conclusions The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.

2014 ◽  
pp. 39-44 ◽  
Author(s):  
Catalina María Vélez Echeverri ◽  
Lina María Serna-Higuita ◽  
Ana Katherina Serrano Gayubo ◽  
Carolina Ochoa-García ◽  
Luisa Rojas Rosas ◽  
...  

Introduction: Urinary tract infection (UTI) is one of the most common bacterial infections in childhood and causes acute and chronic morbidity and long-term hypertension and chronic kidney disease. Objectives To describe the demographic characteristics, infectious agents, patterns of antibiotic resistance, etiologic agent and profile of susceptibility and response to empirical treatment of UTI in a pediatric population. Methods: This is a descriptive, retrospective study. Results: Included in the study were 144 patients, 1:2.06 male to female ratio. The most common symptom was fever (79.9%) and 31.3% had a history of previous UTI. 72.0% of the patients had positive urine leukocyte count (>5 per field), urine gram was positive in 85.0% of samples and gram negative bacilli accounted for 77.8% for the total pathogens isolated. The most frequent uropathogens isolated were Escherichia coli and Klebsiella pneumoniae. Our E.coli isolates had a susceptibility rate higher than 90% to most of the antibiotics used, but a resistance rate of 42.6% to TMP SMX and 45.5% to ampicillin sulbactam. 6.3% of E. coliwas extended-spectrum beta-lactamases producer strains. The most frequent empirical antibiotic used was amikacin, which was used in 66.0% of the patients. 17 of 90 patients who underwent voiding cistouretrography (VCUG) had vesicoureteral reflux. Conclusion: This study revealed that E. coli was the most frequent pathogen of community acquired UTI. We found that E. coli and other uropathogens had a high resistance rate against TMP SMX and ampicillin sulbactam. In order to ensure a successful empirical treatment, protocols should be based on local epidemiology and susceptibility rates.


2018 ◽  
Vol 21 (3) ◽  
pp. 308-314 ◽  
Author(s):  
Matthew J. Shepard ◽  
Mohamed A. Elzoghby ◽  
Erin N. Kiehna ◽  
Spencer C. Payne ◽  
John A. Jane

OBJECTIVERathke cleft cysts (RCCs) are sellar lesions that are commonly encountered in adults but infrequently diagnosed in the pediatric population. As a result, the optimal management of pediatric RCCs remains a subject of controversy. Only 2 prior surgical series have been published on pediatric RCCs and no study has compared the presentation and outcomes of surgically versus conservatively managed cases. The authors therefore performed a comparative analysis of pediatric cases of RCC in which patients were treated with surgery or managed in a conservative manner.METHODSAll cases involving pediatric patients diagnosed with an RCC at the University of Virginia between 2000 and 2016 were included in this study. Patient medical records, operative notes, and neuroimaging findings were reviewed. Patients who developed visual field deficits, radiographic evidence of chiasmal compression, or medically refractory headaches were considered candidates for surgical intervention. All patients who were selected for surgery underwent an endoscopic endonasal approach with cyst fenestration.RESULTSA total of 24 pediatric patients were diagnosed with an RCC over a 16-year period. Seven patients ultimately underwent transsphenoidal cyst fenestration, and 17 were managed conservatively. The patients’ age at diagnosis, cyst size, and pituitary function at the time of RCC diagnosis were similar in the conservatively and surgically managed cohorts. At diagnosis, 19 of 24 patients endorsed headaches that led to neuroimaging. All patients in the surgical cohort endorsed severe headaches at diagnosis compared with 71% in the conservative group. For the 7 patients treated with surgery, complete cyst evacuation was achieved in 86% of cases. Transient postoperative endocrinopathy occurred in 4 (57%) of 7 surgically treated individuals and resolved in all cases. In the conservative cohort, 1 patient developed a delayed pituitary-related endocrinopathy. Headache resolution occurred in 5 (71%) of the 7 patients who underwent surgery and 7 (58%) of the 12 who were treated without surgery. Cyst recurrence was documented in 1 individual in the surgical cohort who underwent a subtotal cyst fenestration that ultimately required re-intervention. In the conservative cohort, spontaneous cyst shrinkage occurred in 35% of patients with a median time to regression of 23.5 months.CONCLUSIONSPediatric RCCs are benign sellar lesions that often present with headaches. While cyst fenestration mitigates headaches in most patients, the majority of conservatively managed pediatric patients with RCCs will have spontaneous headache resolution. Furthermore, spontaneous RCC regression occurs in a substantial number of individuals. Thus, in the absence of optic compression, visual field deficit, or diagnostic uncertainty, many pediatric cases of RCC can be managed conservatively.


2013 ◽  
Vol 12 (4) ◽  
pp. 344-350 ◽  
Author(s):  
Peter J. Madsen ◽  
Shih-Shan Lang ◽  
Jared M. Pisapia ◽  
Phillip B. Storm ◽  
Robert W. Hurst ◽  
...  

Object Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature. Methods A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012. Results Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both N-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit. Conclusions Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.


2020 ◽  
Vol 29 (02) ◽  
pp. 091-095
Author(s):  
Jaime Perez ◽  
Julián Chavarriaga ◽  
Paula Peña ◽  
Gustavo Ramos ◽  
Lynda Torres ◽  
...  

Abstract Introduction Transitional cell carcinoma of the bladder (TCCB) is uncommon in the pediatric population, and its etiology, natural history and epigenetics remain poorly understood. We aim to describe six cases of TCCB in pediatric patients and discuss the state of the art in the management and follow-up of the patients with this uncommon early presentation. Methods The clinicopathological data of 6 patients with TCCB who underwent transurethral resection of bladder tumor (TURBT) were obtained from our institutional database. The patient data were collected retrospectively. A review of the literature was performed, and the most relevant and trending data were analyzed. Results A total of 6 patients (4 female, 2 male) were treated at our institution between 2004 and 2019. The mean age of the sample was 12 years, and the presenting symptoms were macroscopic hematuria (3 cases), suprapubic pain (2 cases), and 1 case was an incidental finding during pelvic ultrasonography. The long-term follow-up (median follow-up of 61 months) did not reveal recurrence. Conclusion Transitional cell carcinoma of the bladder rarely presents in the pediatric population. Genetic and epigenetic anomalies have been proposed as causes, as well as carcinogenic exposure. The reported cases tend to have a good prognosis, and most are non-invasive at the diagnosis. Follow-up protocols are still lacking, as well as molecular insights on tumor development and prognostic markers.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Monica F Stecchini ◽  
Ana Carolina Bueno ◽  
Leandra N Z Ramalho ◽  
Fernando S Ramalho ◽  
Rodrigo T Calado ◽  
...  

Abstract Background: The pathogenesis of adrenocortical tumors (ACTs) in the pediatric population is partially known, and few prognostic factors have been identified in this age group. Recently, ATRX and DAXX have been implicated in the pathogenesis and prognosis of a variety of cancers. Their altered function has been shown to affect telomere length through a telomerase-independent mechanism. Objective: To investigate ATRX and DAXX gene expression, ATRX and DAXX protein expression, and telomere length, as well as their clinical significance, in ACT samples from pediatric patients. Methods: The records of 110 pediatric patients with available ACT samples were reviewed. ATRX, DAXX, TERT and TERC gene expression was assessed by qPCR (n = 100 ACTs; n = 12 normal adrenals). ATRX and DAXX protein expression was assessed by IHC (n = 45 ACTs). Telomere length was assessed by qPCR (n = 64 ACTs). For survival analysis, Kaplan-Meier curves were obtained. For association analysis, simple linear regression models were adjusted. Results: Most patients were female (70.9%) and harbored germline TP53 mutations (90.2%). Median age at diagnosis was 21.1 months (2.1 – 199). Younger patients (< 3 years) had better survival (p < 0.01), while those with metastasis at diagnosis and carcinomas (classified by the Wieneke score) had worse survival (p < 0.01). ATRX gene expression was decreased (p < 0.01), while DAXX gene expression was increased (p < 0.01) in ACTs, compared to normal adrenals. ATRX gene expression was even lower in the context of the germline TP53 (R337H) mutation (p < 0.01). TERT expression was not detected in ACTs or normal adrenals, and TERC expression was not altered (p = 0.69). ATRX protein expression was lost in the majority of ACTs (95.6%), while DAXX was lost in a minority (21.1%). There was no association between gene or protein expression and disease-free or overall survival. There was a significant association between decreased ATRX and DAXX gene expression and increased telomere length (p < 0.01 and p = 0.03, respectively). Conclusion: In pediatric ACTs, decreased ATRX and DAXX gene expression was associated with increased telomere length, independently of TERT or TERC expression. In these tumors, ATRX gene expression was decreased and ATRX protein expression was overall lost, while DAXX gene expression was increased and DAXX protein expression was overall retained. No significant association between these alterations and prognosis was found in this cohort. These findings suggest that ATRX and DAXX altered function may be more involved in the pathogenesis of pediatric ACTs than in the prognosis of the affected patients.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Cherif Ines ◽  
Wafa Triki ◽  
Affes Hassen ◽  
Hanene Ferjani ◽  
Kaouther Maatallah ◽  
...  

Abstract Background Hip disorders are highly common among pediatric patients. The diagnosis is based primarily on the clinical findings, the context and the personal and family history of the child. It is in most cases steered by the biological and imaging findings. The latter are usually derived from radiographs, ultrasound imaging and, in some cases, MRI. The main purpose of this study is to evaluate assess the diagnostic delay for hip disorders in the pediatric population. Methods We conducted a retrospective study including pediatric patients from the rheumatology and the orthopedics department, who consulted or were referred to orthopeadics for a suspected hip disorder. For all patients, we collected the following data: age, clinical findings, imaging findings, the number of consultations in general medicine or emergency medicine and the number of consultations in orthopedics department before the diagnosis is made, the final retained diagnosis and, ultimately, the diagnostic delay since onset of symptoms and first consultation. Results We included 81 children (48 boys and 33 girls) with a mean age of 9.9 years old [1.5–16]. Sixty-two patients were symptomatic at admission. The pain was located in the hip in 55.5% of patients (n = 45), in the groin in 7.4% of patients (n = 6) and in the knee in 12.34% of patients (n = 10). One child reported unilateral buttock pain. The other reasons for consultation were limping in 3.7% of cases (n = 3), deformity of the lower limb in 1.23% of cases (n = 1), a crying baby in 1.23% of cases (n = 1) and swelling of the knee in 1.23% of cases (n = 1). On clinical examination, 58 patients had a limitation of the hip motion. Twenty-nine percent of the children had a biological inflammatory syndrome at admission. In total, over one hundred and seventy-one imaging examinations were performed in order to adjust the diagnosis; among them, 7 CT pelvic scan and 23 MRI. The different retained diagnoses of hip involvement are summarized in Table 1. The mean number of consultations in general medicine or emergency medicine before the diagnosis is made was 0,58 ranging from 0 to 2. The mean diagnostic delay since the onset of symptoms was 9,12 months ranging from 3 days to 13 years. The mean diagnostic delay since the first medical check -up was 3,5 months ranging from 0 to 10 years. Conclusion Our study showed that the patient lost an average of 9 months from symptom onset to retaining the final diagnosis. The hip being a prognosis joint, this delay could alter the child's future functional abilities.


2012 ◽  
Vol 9 (4) ◽  
pp. 432-441 ◽  
Author(s):  
Aaron E. Bond ◽  
Gabriel Zada ◽  
Ira Bowen ◽  
J. Gordon McComb ◽  
Mark D. Krieger

Object The goal of this study was to review all cases of pediatric spinal arachnoid cysts (SACs) surgically treated at the authors' institution between 1992 and 2008 and to compare these cases to the published literature for the general population. Methods The charts of all pediatric patients with SACs were reviewed for demographics, medical history, presenting symptoms, imaging findings, operative procedure(s), complications, and outcomes. Following a complete literature review, the pediatric data were compared with data from the general population and unique findings associated with pediatric patients were identified. Results Thirty-one pediatric patients (median age 6.9 years) underwent operative intervention for SACs between 1992 and 2008 (median duration of follow-up 4.2 years). There were 17 female patients (55%) and 14 male patients (45%). Twenty-one patients (68%) presented with symptoms of radiculopathy or myelopathy. The most common presenting symptoms were pain (42%), lower-extremity weakness (39%), gait instability (32%), spasticity (19%), sensory loss (10%), and bladder dysfunction (7%). In 3 patients (10%) SACs were incidental findings. Intradural SACs were more common (18 patients, 58%) than extradural SACs (11 patients, 36%). One patient (3%) had extradural and intradural components. One patient (3%) had a purely intramedullary cyst, and 1 patient (3%) had both an intradural and intramedullary component. Of the 18 intradural SACs, 9 (50%) were located ventral to the spinal cord and 9 (50%) were dorsally situated. One dorsal intradural SAC had an intramedullary component. All extradural SACs were located dorsal to the spinal cord. Intradural SACs were primarily concentrated in the cervical and thoracic regions (67%), whereas extradural cysts were more evenly distributed between the thoracic, lumbar, and sacral regions. Of the 18 patients with intradural SACs, 13 (72%) had significant previous CNS abnormalities, compared with 3 (27%) of 11 patients with extradural SACs. There were 2 operative complications. One patient had a CSF leak treated with a lumbar drain, and the second patient had a pseudomeningocele. No patients had neurological deterioration as a result of surgical intervention. Twenty-one patients (68%) had complete remission of symptoms, 6 (19%) had improvement, 3 (10%) were stable, and 1 (3%) has worsening of symptoms with recurrence that ultimately required cystoperitoneal shunting, despite multiple failed attempts at fenestration. Conclusions Spinal arachnoid cysts are rare lesions in the pediatric population. Affected patients present with back pain, weakness, and/or gait instability. In children, SACs predominantly develop in the thoracic region and are more likely to occur intradurally, compared with SACs in the general population. Overall outcomes following surgical fenestration or excision of SACs are excellent, with complete remission or improvement of symptoms achieved in 87% of cases.


2016 ◽  
Vol 17 (6) ◽  
pp. 639-644 ◽  
Author(s):  
Babatunde J. Akinpelu ◽  
Scott L. Zuckerman ◽  
Stephen R. Gannon ◽  
Ashly Westrick ◽  
Chevis Shannon ◽  
...  

OBJECTIVE Isolated transverse and spinous process fractures (TPFx and SPFx) in the thoracic and/or lumbar region have been deemed clinically insignificant in the adult population. This same rule is often applied to the pediatric population; however, little evidence exists in this younger group. The goal of this study was to describe the clinical, radiographic, and long-term data on isolated TPFx and SPFx in an exclusively pediatric population. METHODS A retrospective chart review at Monroe Carell Jr. Children's Hospital at Vanderbilt University identified 82 pediatric patients with isolated TPFx and/or SPFx following a traumatic event between January 2000 and December 2013. Patient demographic information, presenting symptoms, radiographic characteristics, and follow-up data were collected. Follow-up was used to determine the outcome (presence of neurological deficits) of such injuries via complete physical examination and, when available, radiographic evidence. RESULTS In the 82 identified patients, the mean age was 15.5 ± 3.1 years (mean is expressed ± SD throughout), with 72 injuries (87.8%) resulting from a motor vehicle, motorcycle, or all-terrain vehicle accident. There was a mean of 1.7 ± 1.0 fractured vertebral levels involved and a mean of 1.8 ± 1.1 fractures was identified per patient. Seventy-one patients (86.6%) needed bedside pain control, 7 (8.5%) were prescribed a brace, and 4 patients (4.9%) received a collar. Physical therapy was recommended for 12 patients (14.6%). A total of 84.1% had follow-up, and the mean length of follow-up was 19 ± 37 months. No patients had true neurological deficits at presentation or follow-up as a result of their isolated fractures, whereas 95.1% had other associated system injuries. CONCLUSIONS These data shows that there is no appreciable long-term complication associated with isolated thoracic and/or lumbar TPFx and/or SPFx in an exclusively pediatric population. Because these fractures are, however, associated with high-energy blunt trauma, they often result in associated soft-tissue or other skeletal injury. All pediatric patients in the cohort benefited from conservative management and aggressive treatment of their comorbidities.


Medwave ◽  
2021 ◽  
Vol 21 (01) ◽  
pp. e8107-e8107
Author(s):  
Jesús Domínguez Rojas ◽  
Matilde Estupiñan Vigil ◽  
Raquel Garcés-Ghilardi ◽  
Giancarlo Alvarado-Gamarra ◽  
Olguita del Águila ◽  
...  

Introduction Coronavirus 2019 (SARS-CoV-2) infection in children occurred in Peru as of March 2020, leading to pediatric patients' hospitalization in areas adapted for this purpose at the Edgardo Rebagliati Martins National Hospital. In the beginning, the demand for hospitalization was low, but it increased gradually. Consistent with international reports, the majority of patients presented mild or moderate symptoms. Nonetheless, there were also severe cases, even fatal ones. Objectives To describe the characteristics and clinical outcome of pediatric patients with COVID-19 hospitalized in a referral hospital in Lima, Peru, between March and August 2020. Methods A descriptive and inferential cross-sectional study was carried out. The population includes all hospitalized patients in the Department of Pediatrics, with clinical and surgical diagnoses associated with COVID-19. Results We included 100 patients, with an average age of 83.4 ± 54 months, with a predominance of male patients (55%). Hospitalized patients were grouped into five categories: respiratory failure (17%), multisystemic inflammatory syndrome (MIS-C) (31%), neurological presentation (19%), acute abdomen (20%), and patients with oncological problems (13%). Most of the patients (74%) had comorbidities. Regarding the presenting symptoms, intestinal pain predominated in the appendicitis group (90%, p < 0.001), fever was present in most patients with respiratory failure (64.7%); multisystemic inflammatory syndrome (90.3%), neurological manifestations (15.8%), acute abdomen (50%) and oncological conditions (61.5%) were also present in these patients. Kawasaki symptoms were found in 38.7% of the patients with multisystemic inflammatory syndrome. Mortality was 4%. Respiratory problems (29.4%) and multisystemic inflammatory syndrome (22.6%) required admission to intensive care, more frequently than the other presentations (p = 0.008). Conclusions We conclude that the vulnerability in the pediatric population is the one that has preexisting conditions. We divided our patients according to presentation, diagnosis, and complications, which were predominantly respiratory. We also had oncological patients with COVID-19.


2020 ◽  
Vol 11 ◽  
pp. 246
Author(s):  
Annelise Sprau ◽  
Anil Mahavadi ◽  
Michael Zhang ◽  
Micah Saste ◽  
Michael Deftos ◽  
...  

Background: Rathke’s cleft cysts (RCCs) are benign, typically asymptomatic sellar lesions found incidentally in adults, with a dramatically lower incidence in pediatric patients (<18 years). We present a case of RCC with xanthogranulomatous change (XGC) – an even less common subtype of RCC – treated by endoscopic endonasal surgical resection. This is the second reported instance of an RCC with XGC occurring in a pediatric patient. Case Description: The patient is a 17-year-old male with delayed puberty who presented with bitemporal hemianopsia and was found to have a 2.6 cm lesion, initially thought to be a craniopharyngioma. He subsequently underwent uncomplicated transsphenoidal endoscopic endonasal resection. Histology confirmed the diagnosis of RCC and demonstrated marked degenerative XGCs with squamous metaplasia. The patient tolerated the procedure well with improvement in visual symptoms. Conclusion: RCC with XGC is a very rare pathology, particularly in the pediatric population. These lesions, while benign, can manifest clinically with significant symptoms. While treatment paradigms are not fully established with a small cohort of cases, endoscopic endonasal approaches have made surgical resection of these lesions a safe and effective treatment strategy, even in the pediatric population.


Sign in / Sign up

Export Citation Format

Share Document