scholarly journals Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts

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.

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.


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.


2017 ◽  
Vol 83 (12) ◽  
pp. 1386-1389 ◽  
Author(s):  
David J. Hiller ◽  
Jaime L. Bohl ◽  
Kristen A. Zeller

Rectal prolapse is the protrusion of the rectum out of the anus. Surgical correction can be accomplished via open and minimally invasive abdominal approaches, as well as from the perineum. Robotic rectopexy is an option for minimally invasive treatment of rectal prolapse. There are no studies that have established the efficacy of robotic rectopexy for rectal prolapse in the pediatric population. The aim of this study was to review the experience of robotic rectopexy at a single institution. This is a retrospective review of our pediatric robotic rectopexy experience from 2012 to 2015. Information was obtained from chart review of both operative notes and clinic visits. Four pediatric patients underwent a robotic rectopexy for rectal prolapse from 2012 to 2015. Three patients were male and one was female. The mean age was 15.5 years (range 13–17). Two patients had rectal prolapse with chronic constipation. One patient had rectal prolapse from Ehlers Danlos syndrome, and the last had rectal prolapse after imperforate anus repair as an infant. Three patients received a bowel preparation. Three patients were completed robotically, and one patient required conversion to an open procedure. The average postoperative length of stay was 3.25 days (range 2–4). There were no episodes of recurrent prolapse. Two patients had improvement in constipation, one had no improvement, and one had no documented change. Average postoperative follow-up was 11.5 months (range 3–29). This study was a review of one institution's experience with pediatric robotic rectopexy. With short-term follow-up, there was no recurrence of prolapse. Robotic rectopexy provided a safe, reliable, and short-term resolution of rectal prolapse in pediatric patients.


2018 ◽  
Vol 79 (06) ◽  
pp. 574-579 ◽  
Author(s):  
Alexander Schupper ◽  
Adam Deconde ◽  
Michael Levy ◽  
Javan Nation

Introduction The fully endoscopic expanded endonasal approach (EEA) has been shown to be safe and efficacious in pediatric patients. However, in the very young patient (ages six and under), the anatomical challenge of working through a small nasal corridor is problematic. The ability to repair the skull base and use a nasoseptal flap (NSF) has also been called into question. Here, we review skull base resections using EEA in patients aged 6 years and younger. Methods A retrospective chart review was conducted on pediatric patients age 6 years and younger who underwent EEA skull base resections over a 3-year period (June 2014 through June 2017). Results Eight children aged 6 and under with seven tumor pathologies underwent an EEA for the resection of their skull base tumors, with a mean follow-up of 1.45 years. Only chordoma cases required multiple-staged resections. There are no tumor recurrences to date. A high-flow intraoperative cerebrospinal fluid (CSF) leak was encountered in three cases, and there were no postoperative CSF leaks. CSF leaks were effectively repaired with NSFs. One patient suffered from postoperative hypopituitarism, one patient experienced recurrent epistaxis secondary to turbinate destruction by the tumor, and one patient expired 8 months postresection secondary to metastatic disease. Conclusion In our case series, EEA for skull base tumors in children aged 6 and under was a safe and effective surgical approach. Skull base defects are able to be effectively addressed with typical repairs including NSFs. Larger studies are warranted to further investigate this technique in this pediatric population.


2021 ◽  
pp. 014556132110079
Author(s):  
Melonie Anne Phillips ◽  
Meredith Lind ◽  
Gerd McGwire ◽  
Diana Rodriguez ◽  
Suzanna Logan

Head and neck tumors are rare in pediatric patients but should be kept in the differential when a patient presents with a new swelling or mass. One of these tumors is a myxoma, which is an insidiously growing, benign mass originating from the mesenchyme. They most commonly arise in the myocardium but can also develop in facial structures, particularly in the maxilla and mandible. When arising in facial structures, ocular, respiratory, and digestive systems can be affected based on local invasion. Complete surgical resection is curative but can lead to significant morbidity as well. Here, we present a case of a 15-month-old toddler presenting with a paranasal mass, which was ultimately diagnosed as a maxillary myxoma. This tumor is very rare in the pediatric population, especially in the toddler age-group, reminding clinicians to broaden the differential diagnosis when a patient’s course is atypical.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S684-S684
Author(s):  
Victoria Konold ◽  
Palak Bhagat ◽  
Jennifer Pisano ◽  
Natasha N Pettit ◽  
Anish Choksi ◽  
...  

Abstract Background To meet the core elements required for antimicrobial stewardship programs, our institution implemented a pharmacy-led antibiotic timeout (ATO) process in 2017 and a multidisciplinary ATO process in 2019. An antibiotic timeout is a discussion and review of the need for ongoing empirical antibiotics 2-4 days after initiation. This study sought to evaluate both the multidisciplinary ATO and the pharmacy-led ATO in a pediatric population, compare the impact of each intervention on antibiotic days of therapy (DOT) to a pre-intervention group without an ATO, and to then compare the impact of the pharmacy-led ATO versus multidisciplinary ATO on antibiotic days of therapy (DOT). Methods This was a retrospective, pre-post, quasi-experimental study of pediatric patients comparing antibiotic DOT prior to ATO implementation (pre-ATO), during the pharmacy-led ATO (pharm-ATO), and during the multidisciplinary ATO (multi-ATO). The pre-ATO group was a patient sample from February-September 2016, prior to the initiation of a formal ATO. The pharmacy-led ATO was implemented from February-September 2018. This was followed by a multidisciplinary ATO led by pediatric residents and nurses from February-September 2019. Both the pharm-ATO and the multi-ATO were implemented as an active non-interruptive alert added to the electronic health record patient list. This alert triggered when new antibiotics had been administered to the patient for 48 hours, at which time, the responsible clinician would discuss the antibiotic and document their decision via the alert workspace. Pediatric patients receiving IV or PO antibiotics administered for at least 48 hours were included. The primary outcome was DOT. Secondary outcomes included length of stay (LOS) and mortality. Results 1284 unique antibiotic orders (n= 572 patients) were reviewed in the pre-ATO group, 868 (n= 323 patients) in the pharm-ATO and 949 (n= 305 patients) in the multi-ATO groups. Average DOT was not significantly different pre vs post intervention for either methodology (Table 1). Mortality was similar between groups, but LOS was longer for both intervention groups (Table 1). Impact of an ATO on DOT, Mortality and LOS Conclusion An ATO had no impact on average antibiotic DOT in a pediatric population, regardless of the ATO methodology. Disclosures All Authors: No reported disclosures


2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Pi-Chang Lee ◽  
Betau Hwang ◽  
Wen-Jue Soong ◽  
C. C. Laura Meng

Background.The prevalence of obstructive sleep apnea (OSA) in the pediatric population is currently estimated at 1-2% of all children. The purpose of this study was to investigate the clinical and hemodynamic characteristics in pediatric patients with cor pulmonale and OSA.Methods.Thirty children with the diagnosis of OSA were included. These patients consisted of 26 male and 4 female children with a mean age of 7 ± 4 years old. Five of those children were found to be associated with cor pulmonale, and 25 had OSA but without cor pulmonale.Results.The arousal index was much higher in children with OSA and cor pulmonale. The children with OSA and cor pulmonale had much lower mean and minimal oxygen saturation and a higher incidence of bradycardia events. All 5 patients with OSA and cor pulmonale underwent an adenotonsillectomy, and the pulmonary arterial pressure dropped significantly after the surgery.Conclusion.This study demonstrated that the OSA pediatric patients with cor pulmonale had the different clinical manifestations and hemodynamic characteristics from those without cor pulmonale. The adenotonsillectomy had excellent results in both the OSA pediatric patients with and without cor pulmonale.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rafael Peñalver Penedo ◽  
Marta Rupérez Lucas ◽  
Luis Antonio Álvarez-Sala Walther ◽  
Alicia Torregrosa Benavent ◽  
María Luisa Casas Losada ◽  
...  

Abstract Background Midregional-proadrenomedullin (MR-proADM) is a useful prognostic peptide in severe infectious pathologies in the adult population. However, there are no studies that analyze its utility in febrile urinary tract infection (fUTI) in children. An accurate biomarker would provide an early detection of patients with kidney damage, avoiding other invasive tests like renal scintigraphy scans. Our objective is to study the usefulness of MR-proADM as a biomarker of acute and chronic renal parenchymal damage in fUTI within the pediatric population. Methods A prospective cohort study was conducted in pediatric patients with fUTI between January 2015 and December 2018. Plasma and urine MR-proADM levels were measured at admission in addition to other laboratory parameters. After confirmation of fUTI, renal scintigraphy scans were performed during the acute and follow-up stages. A descriptive study has been carried out and sensitivity, specificity and ROC curves for MR-proADM, C-reactive protein, and procalcitonin were calculated. Results 62 pediatric patients (34 female) were enrolled. Scintigraphy showed acute pyelonephritis in 35 patients (56.5%). Of those patients, the median of plasmatic MR-proADM (P-MR-proADM) showed no differences compared to patients without pyelonephritis. 7 patients (11.3%) developed renal scars (RS). Their median P-MR-proADM levels were 1.07 nmol/L (IQR 0.66–1.59), while in patients without RS were 0.48 nmol/L (0.43–0.63) (p < 0.01). The AUC in this case was 0.92 (95% CI 0.77–0.99). We established an optimal cut-off point at 0.66 nmol/L with sensitivity 83.3% and specificity 81.8%. Conclusion MR-ProADM has demonstrated a poor ability to diagnose pyelonephritis in pediatric patients with fUTI. However, P-MR-proADM proved to be a very reliable biomarker for RS prediction.


2021 ◽  
pp. 088307382110045
Author(s):  
Eric Strong ◽  
Emily Linda Pierce ◽  
Raquel Langdon ◽  
Jeffery Strelzik ◽  
William McClintock ◽  
...  

Introduction: New daily persistent headache (NDPH) is a primary headache disorder characterized by an intractable, daily, and unremitting headache lasting for at least 3 months. Currently, there are limited studies in the pediatric population describing the characteristics of NDPH. Objective: The objective of the current study is to describe the characteristics of NDPH in pediatric patients presenting to a headache program at a tertiary referral center. Methods: The participants in the current study were pediatric patients who attended the Headache Clinic at Children’s National Hospital between 2016 and 2018. All patients seen in the Headache Clinic were enrolled in an institutional review board–approved patient registry. Results: Between 2016 and 2018, NDPH was diagnosed in 245 patients, representing 14% of the total headache population. NDPH patients were predominantly female (78%) and white (72%). The median age was 14.8 years. The median pain intensity was 6 of 10 (standard deviation = 1.52). Most patients reported experiencing migrainous features, namely, photophobia (85%), phonophobia (85%), and a reduced activity level (88%). Overall, 33% of patients had failed at least 1 preventive medication, and 56% had failed at least 1 abortive medication. Furthermore, 36% of patients were additionally diagnosed with medication overuse headache. Conclusion: NDPH is a relatively frequent disorder among pediatric chronic headache patients. The vast majority of these patients experience migrainous headache characteristics and associated symptoms and are highly refractory to treatment—as evidenced by a strong predisposition to medication overuse headache and high rates of failed preventive management.


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