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Case report, case study

https://doi.org/10.21857/yk3jwhnx09

Perioperative anaesthetic management of patient with amelia and phocomelia - case report

Anja Mandarić ; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Tea Pašalić ; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Zrinka Orešković ; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Marijana Žura ; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Ivan Marić ; Clinical Hospital Dubrava, Department for Orthopaedics and Traumatology, Clinic for surgery
Ivan Šitum ; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Sandra Morović ; Arsano Medical Group


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Abstract

Phocomelia is a congenital condition involving malformations of the arm and leg, resulting in a finlike appendage. It is often caused by the mother’s use of the drug thalidomide during pregnancy, with various mechanisms being proposed. There is no specific treatment for phocomelia or amelia, but in certain cases surgical intervention may be recommended to improve limb function or other functional outcomes. This case report concerns a ten-year-old female patient with congenital amelia of the upper extremities and phocomelia of the lower extremities who was admitted to the hospital for elective thoracic scoliosis surgery It is a demanding and long-term operation with estimated major blood loss. In addition, the prone position of the patient makes access difficult in case of resuscitation, and therefore the role of the anesthesiologist in the care of such patients is crucial in the perioperative period. When caring for such patients, there are many technical difficulties, starting with the monitoring of arterial pressure values. Furthermore, difficulties may arise related to peripheral venous access, which can be a great challenge, and the placement of a central venous catheter is sometimes indicated. Also, intubation can be difficult due to limited mobility of the cervical spine. Early and adequate preoperative assessment of the patient is essential for the induction and maintenance of safe anesthesia and postoperative care. The current review of the literature does not reveal strict recommendations on the optimal treatment of these patients. Due to all the above-mentioned difficulties, preoperative preparation and
assessment are of key importance for the introduction and maintenance of safe anesthesia, as well as postoperative care.

Keywords

phocomelia; amelia; scoliosis; anaesthetic management; anaesthesia

Hrčak ID:

312736

URI

https://hrcak.srce.hr/312736

Publication date:

22.12.2023.

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