Challenges in Robotic thymectomy: stage IVa Myasthenia Gravis pediatric patient
Thymectomy is increasingly been used to treat both adult and paediatric patients with myasthenia gravis. However, paediatric cases might be challenging, especially due to their uncommon occurrence. We report the case of a 12 years old female patient diagnosed with thymic hyperplasia in myasthenia gravis. The patient presented diplopia, speech difficulty, limbs weakness and vertical nystagmus at time of admission (IVa MGFA and IIB Osserman scale). AChR and antiMUSK ab were positive. She was treated with 5 plasmaphereses and a starting cycle of IVIG (400mg/kg), then higher dosages followed in outpatient clinic. Chest CT scan and later RMN confirmed a thickening of the upper mediastinal tissue (35mmx11mm), thus she underwent a robot-assisted thoracic (RATS) thymectomy. The mediastinal mass was extracted through a radical robot-assisted thymectomy (left-sided approach) which resulted demanding due to the small thorax and narrow intercostal spaces. The perioperative management posed challenges, requiring expertise in both preoperative (IVIG and plasmapheresis) and intraoperative phases with a dedicated pediatric anesthesiologist. Frozen section was diagnostic for thymic hyperplasia. Postop ICU admission. Neurologic and paediatric consults established mestinon, prednisone and azathioprine dosages. Post-op clinical symptoms improved, leading to reduced doses of steroids. Chest drainage was removed on POD3. Patient was discharged on POD10. A MDT approach is imperative for optimal disease management and the integration of medical care and optimal surgical timing are essential steps in achieving favorable outcomes. However, these cases necessitate the resources and expertise available at large, specialized centers.
Authors
- Sara Ricciardi (ORCID: https://orcid.org/0000-0002-4638-8528)
- Marco Martino
- Delia Giovanniello (ORCID: https://orcid.org/0000-0002-3327-8039)
- Massimo Osvaldo Jaus (ORCID: https://orcid.org/0000-0002-0118-9672)
- M. Calvani
- Luigi Carbone (ORCID: https://orcid.org/0000-0002-6125-6830)
- Sara Mantovani (ORCID: https://orcid.org/0000-0001-9469-2738)
- Lorenzo Salvadori
- Stefano Treggiari
- Luigi Tritapepe (ORCID: https://orcid.org/0000-0003-0480-1705)
- Francesco Carleo (ORCID: https://orcid.org/0000-0002-1978-1586)
- Giuseppe Cardillo (ORCID: https://orcid.org/0000-0002-3750-7281)
- Maurizio Bitetto
- Silvia Rogante
- Monica Mucciarone
Institutions
- Carlo Forlanini Hospital (IT)
Publication Details
- Published
- 2024-09-14
- DOI
- https://doi.org/10.1183/13993003.congress-2024.pa2276
- Primary Topic
- Myasthenia Gravis and Thymoma
- Type
- article
- Field-Weighted Citation Impact
- 0.00