gms | German Medical Science

72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

06.06. - 09.06.2021

Patients with neurofibromatosis in Germany – prevalence of hospitalised patients and clinical characteristics between 2007 and 2017

Patienten mit Neurofibromatose in Deutschland – Prävalenz der in Krankenhäusern behandelten Patenten und klinische Merkmale zwischen 2007 und 2017

Meeting Abstract

  • presenting/speaker Aminaa Sanchin - Charité Universitätsmedizin, Klinik für Neurochirurgie, Berlin, Deutschland; Charité Universitätsmedizin, Outpatient neurofibromatosis clinic, Berlin, Deutschland
  • Dörte Huscher - Charité Universitätsmedizin, Biometrics and Clinical Epidemiology, Berlin, Deutschland
  • Wolfgang Böhmerle - Charité Universitätsmedizin, Klinik für Neurologie, Berlin, Deutschland; Charité Universitätsmedizin, Outpatient Neurofibromatosis Clinic, Berlin, Deutschland
  • Philipp Euskirchen - Charité Universitätsmedizin, Klinik für Neurologie, Berlin, Deutschland; Charité Universitätsmedizin, Outpatient Neurofibromatosis Clinic, Berlin, Deutschland
  • Peter Vajkoczy - Charité Universitätsmedizin, Klinik für Neurochirurgie, Berlin, Deutschland; Charité Universitätsmedizin, Outpatient Neurofibromatosis Clinic, Berlin, Deutschland
  • Nora Dengler - Charité Universitätsmedizin, Klinik für Neurochirurgie, Berlin, Deutschland; Charité Universitätsmedizin, Outpatient Neurofibromatosis Clinic, Berlin, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie. sine loco [digital], 06.-09.06.2021. Düsseldorf: German Medical Science GMS Publishing House; 2021. DocV265

doi: 10.3205/21dgnc251, urn:nbn:de:0183-21dgnc2513

Published: June 4, 2021

© 2021 Sanchin et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

Objective: Neurofibromatoses (NF) I-III are rare genetic diseases which affect the nervous system and in case of NF I and II other organ systems. Despite fundamental differences between the diseases, the German Disease Related Group (DRG) classification system summarizes the three NF entities with one DRG code (Q.85.0). So far, no nationwide evidence on clinical characteristics of NF in Germany as well as prevalence over time exists.

Methods: Data on all patients hospitalized in Germany between 2007 and 2017 with International Classification of Diseases (ICD) code Q85.0 were provided by the German Federal Statistical Office and included in the analyses. We examined the prevalence of NF as main and concomitant diagnosis, age groups, overall mortality as well as Operation and Procedure Classification (OPS) codes for NF main diagnosis. Ethical approval was granted by local authorities (EA1/275/20). Data is presented as means per year ± SD.

Results: Between 2007 and 2017, 43.453 patients with NF (7.736 main diagnosis, 35.717 concomitant diagnosis) were treated in German hospitals. There were no relevant changes in NF prevalence between 2007 (n=4.148, main diagnosis n=762) and 2017 (n=3845, main diagnosis n=691). Most frequent concomitant diagnosis include benign and malignant neoplasms, arterial hypertension, café au lait spots, facial paresis, and headache. The mean mortality rate was 0.32% (±0.31) for NF as main diagnosis and 0.02% (±0.004) for NF as concomitant diagnosis. In patients with NF as main diagnosis, the most prevalent age group treated in a hospital was age 5 to 10 (16.5 %±1.4). The mean duration of hospital stay for NF patients showed a slight decrease over time (2007: 5.5 days; 2017: 3.7 days). Between 2007 and 2017, the most frequent diagnostic in-hospital procedures per year were MRI (610 ± 32), EEG (123±21), computed tomography (72 ± 21), neurophysiological (61±9) and audiometric (19±11) examinations. Most frequent therapeutic procedures was excision of diseased tissue of the skin (radical: 244 ± 133, local: 97 ±27) as well as neurosurgical interventions including surgery for benign neoplasms (46 ± 6) and rare (n<10) other procedures such as craniotomy, orbitotomy, functional nerve decompression and nerve excision.

Conclusion: Between 2007 and 2017, the prevalence of NF remained unchanged. The duration of hospital stay showed a slight decrease over time. Most frequent therapeutic procedures included dermatological and neurosurgical interventions.