Anaesthetic management of a child with Wolf–Hirschhorn syndrome
Wolf–Hirschhorn syndrome (WHS) is a malformation syndrome that is characterised by a partial deletion of the short arm of chromosome 4 and is known to occur in about one per 50,000 births. Ophthalmic manifestations are common in patients with WHS, who often require corrective surgery under general a...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | Journal of Clinical and Scientific Research |
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| Online Access: | https://journals.lww.com/10.4103/jcsr.jcsr_155_23 |
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| _version_ | 1841545830957318144 |
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| author | Priyanka Das Shalini Subramanian |
| author_facet | Priyanka Das Shalini Subramanian |
| author_sort | Priyanka Das |
| collection | DOAJ |
| description | Wolf–Hirschhorn syndrome (WHS) is a malformation syndrome that is characterised by a partial deletion of the short arm of chromosome 4 and is known to occur in about one per 50,000 births. Ophthalmic manifestations are common in patients with WHS, who often require corrective surgery under general anaesthesia. Managing the airway, titration of the anaesthetic medications while vigilant monitoring and controlling the haemodynamics during the surgery can be difficult. Further, presence of various anomalies, concurrent seizures, predisposition to cardiac failure and post-operative occurrence of malignant hyperthermia pose anaesthetic challenges in patients with WHS. We present the case of an 11-month-old child with WHS posted for multiple ophthalmological procedures and the challenges faced while administering anaesthesia for the same. |
| format | Article |
| id | doaj-art-675836027bc540888f42bdf75bcdcff2 |
| institution | Kabale University |
| issn | 2277-5706 2277-8357 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Clinical and Scientific Research |
| spelling | doaj-art-675836027bc540888f42bdf75bcdcff22025-01-11T09:46:24ZengWolters Kluwer Medknow PublicationsJournal of Clinical and Scientific Research2277-57062277-83572024-12-0113Suppl 1S47S5010.4103/jcsr.jcsr_155_23Anaesthetic management of a child with Wolf–Hirschhorn syndromePriyanka DasShalini SubramanianWolf–Hirschhorn syndrome (WHS) is a malformation syndrome that is characterised by a partial deletion of the short arm of chromosome 4 and is known to occur in about one per 50,000 births. Ophthalmic manifestations are common in patients with WHS, who often require corrective surgery under general anaesthesia. Managing the airway, titration of the anaesthetic medications while vigilant monitoring and controlling the haemodynamics during the surgery can be difficult. Further, presence of various anomalies, concurrent seizures, predisposition to cardiac failure and post-operative occurrence of malignant hyperthermia pose anaesthetic challenges in patients with WHS. We present the case of an 11-month-old child with WHS posted for multiple ophthalmological procedures and the challenges faced while administering anaesthesia for the same.https://journals.lww.com/10.4103/jcsr.jcsr_155_23anaesthetic managementbradycardiawolf–hirschhorn syndrome |
| spellingShingle | Priyanka Das Shalini Subramanian Anaesthetic management of a child with Wolf–Hirschhorn syndrome Journal of Clinical and Scientific Research anaesthetic management bradycardia wolf–hirschhorn syndrome |
| title | Anaesthetic management of a child with Wolf–Hirschhorn syndrome |
| title_full | Anaesthetic management of a child with Wolf–Hirschhorn syndrome |
| title_fullStr | Anaesthetic management of a child with Wolf–Hirschhorn syndrome |
| title_full_unstemmed | Anaesthetic management of a child with Wolf–Hirschhorn syndrome |
| title_short | Anaesthetic management of a child with Wolf–Hirschhorn syndrome |
| title_sort | anaesthetic management of a child with wolf hirschhorn syndrome |
| topic | anaesthetic management bradycardia wolf–hirschhorn syndrome |
| url | https://journals.lww.com/10.4103/jcsr.jcsr_155_23 |
| work_keys_str_mv | AT priyankadas anaestheticmanagementofachildwithwolfhirschhornsyndrome AT shalinisubramanian anaestheticmanagementofachildwithwolfhirschhornsyndrome |