Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report
Abstract Background Pycnodysostosis is a rare inherited syndrome characterized by classical craniofacial defects and diffuse osteosclerosis disorder of bones with tendency for fractures after minor trauma. We describe the anaesthetic management of a parturient with pycnodysostosis with dual concerns...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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SpringerOpen
2022-10-01
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| Series: | Ain Shams Journal of Anesthesiology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s42077-022-00269-4 |
| _version_ | 1828106149002477568 |
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| author | Reena Ravindra Kadni Shyamasundar L G Pooja Rao Varghese K. Zachariah Priya Pushpavathi |
| author_facet | Reena Ravindra Kadni Shyamasundar L G Pooja Rao Varghese K. Zachariah Priya Pushpavathi |
| author_sort | Reena Ravindra Kadni |
| collection | DOAJ |
| description | Abstract Background Pycnodysostosis is a rare inherited syndrome characterized by classical craniofacial defects and diffuse osteosclerosis disorder of bones with tendency for fractures after minor trauma. We describe the anaesthetic management of a parturient with pycnodysostosis with dual concerns of precious pregnancy and a fractured femur. Case presentation A 33-year-old primigravida with 31 weeks of gestation presented with subtrochanteric fracture of left femur. Clinico-radiological features of diffuse osteosclerosis suggested a diagnosis of pycnodysostosis, which was confirmed by genetic testing. We encountered challenges for sequential surgeries of lower segment caesarean section, surgical fixation of the fractured femur and surgical wound debridement in this patient in view of a difficult airway, short stature, physiological changes of pregnancy, difficulties in positioning and possibility of increased blood loss. A combination of general anaesthesia with either neuraxial anaesthesia or regional block was performed, which helped in the safe management of this syndromic patient. Conclusions Multidisciplinary team dynamics, communication and expertise played a crucial role in the successful care of the patient. Planning anaesthesia contextually for each surgical setting was vital. Anaesthetic gadgets like fibre-optic bronchoscope and ultrasound were a boon to anaesthesiologist at every surgical procedure to aid the anaesthetic plan. |
| first_indexed | 2024-04-11T10:12:25Z |
| format | Article |
| id | doaj.art-05dae3c524294c40b3fa0ebca1a82441 |
| institution | Directory Open Access Journal |
| issn | 2090-925X |
| language | English |
| last_indexed | 2024-04-11T10:12:25Z |
| publishDate | 2022-10-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Ain Shams Journal of Anesthesiology |
| spelling | doaj.art-05dae3c524294c40b3fa0ebca1a824412022-12-22T04:30:03ZengSpringerOpenAin Shams Journal of Anesthesiology2090-925X2022-10-011411510.1186/s42077-022-00269-4Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case reportReena Ravindra Kadni0Shyamasundar L G1Pooja Rao2Varghese K. Zachariah3Priya Pushpavathi4Department of Anaesthesia, Bangalore Baptist HospitalDepartment of Orthopaedics, Bangalore Baptist HospitalDepartment of Anaesthesia, Bangalore Baptist HospitalDepartment of Anaesthesia, Bangalore Baptist HospitalDepartment of Anaesthesia, Bangalore Baptist HospitalAbstract Background Pycnodysostosis is a rare inherited syndrome characterized by classical craniofacial defects and diffuse osteosclerosis disorder of bones with tendency for fractures after minor trauma. We describe the anaesthetic management of a parturient with pycnodysostosis with dual concerns of precious pregnancy and a fractured femur. Case presentation A 33-year-old primigravida with 31 weeks of gestation presented with subtrochanteric fracture of left femur. Clinico-radiological features of diffuse osteosclerosis suggested a diagnosis of pycnodysostosis, which was confirmed by genetic testing. We encountered challenges for sequential surgeries of lower segment caesarean section, surgical fixation of the fractured femur and surgical wound debridement in this patient in view of a difficult airway, short stature, physiological changes of pregnancy, difficulties in positioning and possibility of increased blood loss. A combination of general anaesthesia with either neuraxial anaesthesia or regional block was performed, which helped in the safe management of this syndromic patient. Conclusions Multidisciplinary team dynamics, communication and expertise played a crucial role in the successful care of the patient. Planning anaesthesia contextually for each surgical setting was vital. Anaesthetic gadgets like fibre-optic bronchoscope and ultrasound were a boon to anaesthesiologist at every surgical procedure to aid the anaesthetic plan.https://doi.org/10.1186/s42077-022-00269-4Contextual anaesthesiaDifficult airwayFracture femurPycnodysostosisPregnancy |
| spellingShingle | Reena Ravindra Kadni Shyamasundar L G Pooja Rao Varghese K. Zachariah Priya Pushpavathi Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report Ain Shams Journal of Anesthesiology Contextual anaesthesia Difficult airway Fracture femur Pycnodysostosis Pregnancy |
| title | Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report |
| title_full | Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report |
| title_fullStr | Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report |
| title_full_unstemmed | Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report |
| title_short | Contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation: a case report |
| title_sort | contextual anaesthesia for a rare case of pycnodysostosis with myriad presentation a case report |
| topic | Contextual anaesthesia Difficult airway Fracture femur Pycnodysostosis Pregnancy |
| url | https://doi.org/10.1186/s42077-022-00269-4 |
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