scholarly journals History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis

2015 ◽  
Vol 173 (6) ◽  
pp. 853-862 ◽  
Author(s):  
Anika Hoffmann ◽  
Svenja Boekhoff ◽  
Ursel Gebhardt ◽  
Anthe S Sterkenburg ◽  
Anna M M Daubenbüchel ◽  
...  

ObjectiveChildhood craniopharyngiomas (CP) are often diagnosed after a long duration of history (DOH). Tumor size, hypothalamic involvement (HI), and obesity are associated with reduced overall survival (OS) and functional capacity (FC). The effect of DOH and specific symptoms in history on presentation at initial diagnosis and long-term prognosis are unknown.DesignRetrospective analysis of patients' records and prospective longitudinal follow-up.MethodsHistories of 411 CP patients recruited in HIT Endo, KRANIOPHARYNGEOM 2000 were retrospectively evaluated for DOH, symptoms, and characteristics. The effect of specific manifestations and DOH on clinical presentation and tumor characteristics at time of initial CP diagnosis and long-term outcome were analyzed. Main outcome measures were 10-year OS and progression-free survival (PFS), FC, and BMI during longitudinal follow-up.ResultsMedian DOH was 6 months (range: 0.1–108 months) and correlated with age at diagnosis. Tumor size, HI, degree of resection, and BMI at diagnosis were not related to DOH. In multivariate analysis adjusted for age at diagnosis, only hydrocephalus was found to have a relevant influence on DOH. Visual and neurological deficits were associated with larger initial tumor size and impaired 10-year OS. Weight gain and growth failure were observed with longest DOH. PFS and FC were not related to any specific symptom. Endocrine deficits at diagnosis were associated with long DOH.ConclusionsCP is frequently diagnosed after long DOH, especially in older children. However, DOH was not associated with tumor size, HI, survival, or FC. Visual and neurological deficits necessitate rapid diagnostic workup.

Neurosurgery ◽  
2012 ◽  
Vol 72 (4) ◽  
pp. 573-589 ◽  
Author(s):  
Paritosh Pandey ◽  
Erick M. Westbroek ◽  
Peter A. Gooderham ◽  
Gary K. Steinberg

Abstract BACKGROUND: Cavernous malformations (CMs) in deep locations account for 9% to 35% of brain malformations and are surgically challenging. OBJECTIVE: To study the clinical features and outcomes following surgery for deep CMs and the complication of hypertrophic olivary degeneration (HOD). METHODS: Clinical records, radiological findings, operative details, and complications of 176 patients with deep CMs were reviewed retrospectively. RESULTS: Of 176 patients with 179 CMs, 136 CMs were in the brainstem, 27 in the basal ganglia, and 16 in the thalamus. Cranial nerve deficits (51.1%), hemiparesis (40.9%), numbness (34.7%), and cerebellar symptoms (38.6%) presented most commonly. Hemorrhage presented in 172 patients (70 single, 102 multiple). The annual retrospective hemorrhage rate was 5.1% (assuming CMs are congenital with uniform hemorrhage risk throughout life); the rebleed rate was 31.5%/patient per year. Surgical approach depended on the proximity of the CM to the pial or ependymal surface. Postoperatively, 121 patients (68.8%) had no new neurological deficits. Follow-up occurred in 170 patients. Delayed postoperative HOD developed in 9/134 (6.7%) patients with brainstem CMs. HOD occurred predominantly following surgery for pontine CMs (9/10 patients). Three patients with HOD had palatal myoclonus, nystagmus, and oscillopsia, whereas 1 patient each had limb tremor and hemiballismus. At follow-up, 105 patients (61.8%) improved, 44 (25.9%) were unchanged, and 19 (11.2%) worsened neurologically. Good preoperative modified Rankin Score (98.2% vs 54.5%, P = .001) and single hemorrhage (89% vs 77.3%, P < .05) were predictive of good long-term outcome. CONCLUSION: Symptomatic deep CMs can be resected with acceptable morbidity and outcomes. Good preoperative modified Rankin Score and single hemorrhage are predictors of good long-term outcome.


Author(s):  
Monika Scheer ◽  
Christian Vokuhl ◽  
Sebastian Bauer ◽  
Jörg Fuchs ◽  
Steffan Loff ◽  
...  

Abstract Background The benefit of adjuvant therapy in synovial sarcoma (SS) treatment is under debate. Long-term follow-up data are missing. Methods SS patients treated in the consecutive trials CWS-81, CWS-86, CWS-91, CWS-96, CWS-2002-P, and the SoTiSaR-registry till 2013 were analyzed. Results Median age of 185 patients was 13.9 years (0.1–56)—with median follow-up of 7.4 years for 163 survivors. Most tumors (76%) were located in extremities. Size was < 3 cm in 58 (31%), 3–5 cm in 59 (32%), 5–10 cm in 42 (23%), and > 10 cm in 13 (7%) (13 missing). In 84 (45%) tumors, first excision was complete (R0 corresponding to IRS-I-group) and in 101 (55%) marginal (R1 corresponding to IRS-II-group). In a subsequent surgical intervention during chemotherapy, R0-status was accomplished in 23 additional IRS-II-group patients with secondary surgery. Radiotherapy was administered to 135 (73%), thereof 62 with R0-status and 67 R1-status (6 missing information). Adjuvant chemotherapy was administered to all but six patients. 5-year event-free (EFS) and overall survival (OS) was 82.9% ± 5.7 (95%CI) and 92.5% ± 3.9. Local and metastatic relapse-free survival was 91.3% ± 4.3 and 92.3% ± 4.1 at 5 years, respectively. In the multivariate analysis, tumor size and no chemotherapy were independently associated with EFS. Size and site were associated with OS. In a detailed analysis of local and metastatic events, tumor size was associated with an independent risk for developing metastases. No independent factor for suffering local recurrence could be identified. Discussion Omission of chemotherapy in a non-stratified way seems not justified. Size governs survival due to high linear association with risk of suffering metastatic recurrence in a granular classification.


2002 ◽  
Vol 30 (5) ◽  
pp. 689-692 ◽  
Author(s):  
Jyrki A. Kettunen ◽  
Martti Kvist ◽  
Erkki Alanen ◽  
Urho M. Kujala

Background: Little information is available on the long-term outcome of jumper's knee, a common problem among athletes. Purpose: Our aim was to determine the 15-year prognosis of jumper's knee. Study Design: Prospective case control. Methods: The prognosis for jumper's knee was studied using two groups: athletes with jumper's knee and nonsymptomatic control athletes. At baseline, all subjects participated in standardized clinical examinations and measurements, and 15 years later they were asked to respond to a questionnaire. Results: Twenty athletes with jumper's knee and 16 athlete control subjects responded (response rate 74% and 84%, respectively). The jumper's knee group reported significantly more knee symptoms according to their Kujala score and more knee pain after repeated squatting. Fifty-three percent of the subjects in the jumper's knee group (9 of 17) reported that they had quit their sports career because of their knee problem, compared with 7% of the control athletes (1 of 14). Patellar height was associated with knee symptoms at follow-up. Conclusion: Jumper's knee causes mild but long-lasting symptoms after an athletic career.


2016 ◽  
Vol 19 (1) ◽  
pp. 36-41 ◽  
Author(s):  
Richard L Meeson ◽  
Alex T Geddes

Objectives The aim of the study was to evaluate the management and long-term outcome of cats with pelvic fractures. Methods Cats with pelvic fractures had their records and radiographs reviewed. Radiographs were reviewed for fracture configuration, implants and pelvic canal narrowing. Owners were contacted for long-term follow-up. Results Forty-three cats met the criteria (mean follow-up 24 months [range 6–45 months]). The majority (93%) had more than one orthopaedic pelvic injury, with sacroiliac fracture luxations seen most commonly; 23% had presurgical neurological deficits. Most cats (74%) were managed surgically; 60% of sacroiliac fracture luxations, 82% of ilial fractures and 50% of acetabular fractures received surgery. The complication rate was 22%, most commonly sciatic neurapraxia (13%). Seventy-nine percent of all neurological deficits resolved and the remainder improved. Mean pelvic canal narrowing after trauma was −15% in surgical and −16% in conservatively managed cats. Canal width was improved postoperatively (–8%) but mildly narrowed further by follow-up (–12%); however, these changes were not significant. Nineteen percent of cats had constipation postsurgery; none developed megacolon. There was no clear correlation between the degree of narrowing of the pelvic canal up to −50%, or whether conservative treatment was opted for, and the development of constipation. Long-term mobility was not impaired in 86% of cats, and 84% did not have any lameness detectable. Conclusions and relevance The majority of cats were managed surgically, with a 22% complication rate; the most common being transient sciatic neurapraxia. Long-term outcome was generally excellent and most had a full recovery. Constipation/obstipation was very uncommon and no clear relationship with pelvic canal narrowing could be found when considering narrowing of up to −50% in both surgical and conservative groups. As no cats in this cohort had narrowing greater than −50%, the current recommendation of surgery to improve the canal width if narrowing is greater than −45% to −50% should remain.


VASA ◽  
2002 ◽  
Vol 31 (1) ◽  
pp. 36-42 ◽  
Author(s):  
. Bucek ◽  
Hudak ◽  
Schnürer ◽  
Ahmadi ◽  
Wolfram ◽  
...  

Background: We investigated the long-term clinical results of percutaneous transluminal angioplasty (PTA) in patients with peripheral arterial occlusive disease (PAOD) and the influence of different parameters on the primary success rate, the rate of complications and the long-term outcome. Patients and methods: We reviewed clinical and hemodynamic follow-up data of 166 consecutive patients treated with PTA in 1987 in our department. Results: PTA improved the clinical situation in 79.4% of patients with iliac lesions and in 88.3% of patients with femoro-popliteal lesions. The clinical stage and ankle brachial index (ABI) post-interventional could be improved significantly (each P < 0,001), the same results were observed at the end of follow-up (each P < 0,001). Major complications occurred in 11 patients (6.6%). The rate of primary clinical long-term success for suprainguinal lesions was 55% and 38% after 5 and 10 years (femoro-popliteal 44% and 33%), respectively, the corresponding data for secondary clinical long-term success were 63% and 56% (60% and 55%). Older age (P = 0,017) and lower ABI pre-interventional (P = 0,019) significantly deteriorated primary clinical long-term success for suprainguinal lesions, while no factor could be identified influencing the outcome of femoro-popliteal lesions significantly. Conclusion: Besides an acceptable success rate with a low rate of severe complications, our results demonstrate favourable long-term clinical results of PTA in patients with PAOD.


Crisis ◽  
1999 ◽  
Vol 20 (3) ◽  
pp. 115-120 ◽  
Author(s):  
Stephen Curran ◽  
Michael Fitzgerald ◽  
Vincent T Greene

There are few long-term follow-up studies of parasuicides incorporating face-to-face interviews. To date no study has evaluated the prevalence of psychiatric morbidity at long-term follow-up of parasuicides using diagnostic rating scales, nor has any study examined parental bonding issues in this population. We attempted a prospective follow-up of 85 parasuicide cases an average of 8½ years later. Psychiatric morbidity, social functioning, and recollections of the parenting style of their parents were assessed using the Clinical Interview Schedule, the Social Maladjustment Scale, and the Parental Bonding Instrument, respectively. Thirty-nine persons in total were interviewed, 19 of whom were well and 20 of whom had psychiatric morbidity. Five had died during the follow-up period, 3 by suicide. Migration, refusals, and untraceability were common. Parasuicide was associated with parental overprotection during childhood. Long-term outcome is poor, especially among those who engaged in repeated parasuicides.


2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.


Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Keskin ◽  
H.C Tokgoz ◽  
O.Y Akbal ◽  
A Hakgor ◽  
S Tanyeri ◽  
...  

Abstract Background and aims Although syncope (S) has been reported as one of the presenting findings in patients (pts) with acute pulmonary embolism (APE), its clinical and haemodynamic correlates and impacts on the long-term outcome in this setting remains to be determined. In this single-centre study we evaluated the clinical and haemodynamic significance of S in APE in initial asessment, and during short- and long-term follow-up period. Methods Our study was based on the retrospective and prospective analysis of the overall 641 pts (age 65 (51–74 IQR) yrs, 56.2% female) with diagnosis of documented APE who underwent anticoagulant (n=207), thrombolytic (n=164), utrasound-facilitated thrombolysis (UFT) (n=218) or rheolytic thrombectomy (RT) (n=52). The systematic work- up including multidetector computed tomography (MDCT), Echo, biomarkers, and PE severity indexes were performed in all pts, and Qanadli score (QS) was used as the measure of the thrombotic burden in the pulmonary arteries (PA). Results The S as the presenting symptom In 30.2% of pts with APE. At baseline assessment, S(+) vs S(−) APE subgroups had a significantly shorter symptom-diagnosis interval, a higher risk status according to the significant elevations in troponin T, D-dimer, the higher PE severity indexes, a more deteriorated right ventricle/left ventricle ratio (RV/LV r), right atrial/left atrial ratio (LA/RAr) and RV longitudinal function indexes including tricuspid annular planary excursion (TAPSE) and tissue velocity (St), a significantly higher PA obstructive burden as assessed by QS and PA pressures. Thrombolytic therapy (36.2% vs 21%, p&lt;0.001) and RT (11.9% vs 6.47%, p=0.037) were more frequently utilized S(+) as compared to S(−) group. However, all these differences between two subgroups were found to disappear after evidence-based APE treatments. In-hospital mortality (IHM) (12.95% vs 6%, p=0.007) and minor bleeding (10.36% vs 2.9%, p&lt;0.001) were significantly higher in S(+) pts as compared to those in S(−) subgroup. Binominal logistic regression analysis revealed that PESI score and RV/LVr independently associated with S while IHM was only predicted by age and heart rate. The COX proportional hazard method showed that RV/LVr at discharge and malignancy were independently associated with cumulative mortality during follow-up duration of 620 (200–1170 IQ) days. Conclusions The presence of S in pts with APE was found to be asociated with a higher PA obstructive burden, a more deteriorated RV function and haemodynamics and higher risk status which may need more agressive reperfusion treatments. However, in the presence of the optimal treatments, S did not predict neither in-hospital outcome, nor long-term mortality. Funding Acknowledgement Type of funding source: None


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