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

Marina, Adela Della; Kölbel, Heike; Müllers, Maximilian; Kaiser, Olaf; Ismail, Mahmoud; Swierzy, Marc; Rueckert, Jens-Carsten; Schara, Ulrike

doi:10.1055/s-0037-1603775

Cited by 16 publications

(5 citation statements)

References 28 publications

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“…100 However, clinically relevant late effects on the immune system have not been observed, especially in thymectomized jMG patients. 101 Symptomatic therapy. In children and adolescents, the dose of pyridostigmine is calculated as 1-7 mg/kg bw/day, the single dose at the beginning as 0.5-1 mg/kg bw; a maximum daily dose of 450 mg should not be exceeded.…”

Section: Strong Agreementmentioning

confidence: 99%

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Guideline for the management of myasthenic syndromes

Wiendl,

Abicht,

Chan

et al. 2023

Ther Adv Neurol Disord

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Myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and congenital myasthenic syndromes (CMS) represent an etiologically heterogeneous group of (very) rare chronic diseases. MG and LEMS have an autoimmune-mediated etiology, while CMS are genetic disorders. A (strain dependent) muscle weakness due to neuromuscular transmission disorder is a common feature. Generalized MG requires increasingly differentiated therapeutic strategies that consider the enormous therapeutic developments of recent years. To include the newest therapy recommendations, a comprehensive update of the available German-language guideline ‘Diagnostics and therapy of myasthenic syndromes’ has been published by the German Neurological society with the aid of an interdisciplinary expert panel. This paper is an adapted translation of the updated and partly newly developed treatment guideline. It defines the rapid achievement of complete disease control in myasthenic patients as a central treatment goal. The use of standard therapies, as well as modern immunotherapeutics, is subject to a staged regimen that takes into account autoantibody status and disease activity. With the advent of modern, fast-acting immunomodulators, disease activity assessment has become pivotal and requires evaluation of the clinical course, including severity and required therapies. Applying MG-specific scores and classifications such as Myasthenia Gravis Activities of Daily Living, Quantitative Myasthenia Gravis, and Myasthenia Gravis Foundation of America allows differentiation between mild/moderate and (highly) active (including refractory) disease. Therapy decisions must consider age, thymic pathology, antibody status, and disease activity. Glucocorticosteroids and the classical immunosuppressants (primarily azathioprine) are the basic immunotherapeutics to treat mild/moderate to (highly) active generalized MG/young MG and ocular MG. Thymectomy is indicated as a treatment for thymoma-associated MG and generalized MG with acetylcholine receptor antibody (AChR-Ab)-positive status. In (highly) active generalized MG, complement inhibitors (currently eculizumab and ravulizumab) or neonatal Fc receptor modulators (currently efgartigimod) are recommended for AChR-Ab-positive status and rituximab for muscle-specific receptor tyrosine kinase (MuSK)-Ab-positive status. Specific treatment for myasthenic crises requires plasmapheresis, immunoadsorption, or IVIG. Specific aspects of ocular, juvenile, and congenital myasthenia are highlighted. The guideline will be further developed based on new study results for other immunomodulators and biomarkers that aid the accurate measurement of disease activity.

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Section: Strong Agreementmentioning

confidence: 99%

“… 100 However, clinically relevant late effects on the immune system have not been observed, especially in thymectomized jMG patients. 101 …”

Section: Essential Recommendations (2)mentioning

confidence: 99%

Guideline for the management of myasthenic syndromes

Wiendl,

Abicht,

Chan

et al. 2023

Ther Adv Neurol Disord

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“…In recent studies including 49 children, thoracoscopic thymectomy was also safe for children with juvenile myasthenia gravis (JMG) [143,144]. Two other studies with 9 and 18 children, reported the same results [145,146]. Robotic thymectomy is a safe procedure, complications were low, and without mortality.…”

Section: Thymectomymentioning

confidence: 93%

“…Robotic thymectomy is a safe procedure, complications were low, and without mortality. Thymectomy should be offered as a part of multimodal therapy for treating children and adolescents with acetylcholine receptor antibody-Positive JMG [146].…”

Section: Thymectomymentioning

confidence: 99%

Robotic-Assisted Minimally Invasive Surgery in Children

Navarrete-Arellano¹

2021

Latest Developments in Medical Robotics Systems

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Currently, minimally invasive surgery (MIS) includes conventional laparo-thoracoscopic surgery and robot-assisted surgery (RAS) or robotic surgery. Robotic surgery is performed with robotic devices, for example the Da Vinci system from Intuitive Surgical, which has a miniaturized camera capable of image magnification, a three-dimensional image of the surgical field, and the instruments are articulated with 7 degrees of freedom of movement, and the surgeon operates in a sitting position at a surgical console near the patient. Robotic surgery has gained an enormous surge in use on adults, but it has been slowly accepted for children, although it offers important advantages in complex surgeries. The areas of application of robotic surgery in the pediatric population include urological, general surgery, thoracic, oncological, and otorhinolaryngology, the largest application has been in urological surgery. There is evidence that robotic surgery in children is safe and it is important to offer its benefits. Intraoperative complications are rare, and the frequency of postoperative complications ranges from 0–15%. Recommendations for the implementation of a pediatric robotic surgery program are included. The future will be fascinating with upcoming advancements in robotic surgical systems, the use of artificial intelligence, and digital surgery.

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“…Similar to the adult-onset MG, AChR Ab are mostly detected in both ocular and generalized MG [ 55 , 58 ]. Patients with juvenile MG seem to benefit from thymectomy without relevant signs of long-term immunodeficiency [ 59 ]. Ocular MG affects only the external eye muscles, including the levator palpebrae muscle, and manifests with ptosis and diplopia [ 60 ].…”

Section: Clinical Spectrum Of Mgmentioning

confidence: 99%

Fc-Receptor Targeted Therapies for the Treatment of Myasthenia gravis

Keller

Pawlitzki

Wiendl

et al. 2021

IJMS

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Myasthenia gravis (MG) is an autoimmune disease in which immunoglobulin G (IgG) antibodies (Abs) bind to acetylcholine receptors (AChR) or to functionally related molecules in the postsynaptic membrane at the neuromuscular junction. IgG crystallizable fragment (Fc)-mediated effector functions, such as antibody-dependent complement deposition, contribute to disease development and progression. Despite progress in understanding Ab-mediated disease mechanisms, immunotherapy of MG remained rather unspecific with corticosteroids and maintenance with immunosuppressants as first choice drugs for most patients. More specific therapeutic IgG Fc-based platforms that reduce serum half-life or effector functions of pathogenic MG-related Abs are currently being developed, tested in clinical trials or have recently been successfully translated into the clinic. In this review, we illustrate mechanisms of action and clinical efficacies of emerging Fc-mediated therapeutics such as neonatal Fc receptor (FcRn)-targeting agents. Furthermore, we evaluate prospects of therapies targeting classical Fc receptors that have shown promising therapeutic efficacy in other antibody-mediated conditions. Increased availability of Fc- and Fc receptor-targeting biologics might foster the development of personalized immunotherapies with the potential to induce sustained disease remission in patients with MG.

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

Cited by 16 publications

References 28 publications

Guideline for the management of myasthenic syndromes

Guideline for the management of myasthenic syndromes

Robotic-Assisted Minimally Invasive Surgery in Children

Fc-Receptor Targeted Therapies for the Treatment of Myasthenia gravis

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