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Neuropediatrics 2017; 48(04): 315-322
DOI: 10.1055/s-0037-1603775
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Outcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis

Adela Della Marina
1   Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital 1, University of Duisburg-Essen, Essen, Germany
,
Heike Kölbel
1   Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital 1, University of Duisburg-Essen, Essen, Germany
,
Maximilian Müllers
1   Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital 1, University of Duisburg-Essen, Essen, Germany
,
Olaf Kaiser
1   Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital 1, University of Duisburg-Essen, Essen, Germany
,
Mahmoud Ismail
2   Department of General, Visceral, Vascular and Thoracic Surgery, Universitätsmedizin Berlin–Charité Campus Mitte, Berlin, Germany
,
Marc Swierzy
2   Department of General, Visceral, Vascular and Thoracic Surgery, Universitätsmedizin Berlin–Charité Campus Mitte, Berlin, Germany
,
Jens-Carsten Rueckert
2   Department of General, Visceral, Vascular and Thoracic Surgery, Universitätsmedizin Berlin–Charité Campus Mitte, Berlin, Germany
,
Ulrike Schara
1   Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital 1, University of Duisburg-Essen, Essen, Germany
› Author Affiliations
Further Information

Publication History

06 February 2017

03 May 2017

Publication Date:
19 June 2017 (online)

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Abstract

The aim of our study was to describe the long-term outcomes after robotic-assisted thymectomy in a cohort of acetylcholine receptor (AChR)-antibody (Ab)–positive, generalized juvenile myasthenia gravis (JMG). We retrospectively analyzed a cohort of 18 patients (15 females and 3 males) who underwent robotic-assisted thymectomy. At the time of diagnosis, 12/18 patients were prepubertal; the mean age was 9.8 years at the onset of the disease. All patients received therapy with pyridostigmine; additional immunotherapy included: corticosteroid therapy in 18/18, azathioprine in 14/18 patients, mycophenolate mofetil in 4/18, and cyclosporine in 1/18 patients. Eight patients received intravenous immunoglobulin and four plasma exchange. The mean age of patients at thymectomy was 11.7 years (range: 4.2–16 years). The mean duration of postoperative stay was 2.9 days. Thymectomy was followed by gradual clinical improvement (39% patients achieved clinical remission) and dose reduction in steroid therapy in all patients during the follow-up period (mean: 27.4 months). In children and adolescents with AChR-Ab–positive JMG, thymectomy has a beneficial effect on the weaning off immunosuppressive therapy in patients with generalized symptoms and should be considered as a part of multimodal therapy. Robotic-assisted thymectomy is a safe procedure with low morbidity and a comparable clinical outcome compared with the open sternal procedure.

Keywords

juvenile myasthenia gravis - acetylcholine receptor antibody - robotic-assisted thymectomy - outcome
 

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