BACKGROUND MURCS association,an acronym for Müllerian duct aplasia/hypoplasia,congenital renal agenesis/ectopia,and cervical somite dysplasia,presents unique anaesthetic challenges,particularly for airway control...BACKGROUND MURCS association,an acronym for Müllerian duct aplasia/hypoplasia,congenital renal agenesis/ectopia,and cervical somite dysplasia,presents unique anaesthetic challenges,particularly for airway control due to cervical spine anomalies.This case report adds to the limited literature by detailing airway management strategies in a paediatric patient with MURCS,emphasising the need for thorough preoperative evaluation and curated planning.CASE SUMMARY This report describes the successful anaesthetic and airway management of a 6-year-old girl with MURCS undergoing dental extraction.To address the complexities of the patient’s condition,a multidisciplinary approach involving comprehensive preoperative assessment,meticulous planning,and advanced airway management techniques was adopted.CONCLUSION This report emphasizes individualized anesthetic strategies and interprofessional collaboration for managing rare congenital syndromes.展开更多
The MURCS association which stands for Mullerian, Renal, Cervicothoracic Somite Abnormalities is a rare developmental anomaly seen in females. The clinical course of this disorder is not clearly defined as of yet-whic...The MURCS association which stands for Mullerian, Renal, Cervicothoracic Somite Abnormalities is a rare developmental anomaly seen in females. The clinical course of this disorder is not clearly defined as of yet-which may range from asymptomatic to severe disability. Here we present the case of a young 22-year-old female who was incidentally detected to have a generalized increase in bone density with an absent uterus. Further workup revealed a case of MURCS association with renal osteodystrophy. This is an as of yet unreported complication/association and must be kept in mind in the treatment of these patients.展开更多
MURCS association includes Mullerrian duct aplasia-hypoplasia (MU), renal malformations (R) and cervicothoracic somite dysplasia (CS). This rare disease (1/50 000 females) is sporadic and of unknown aetiology. The rep...MURCS association includes Mullerrian duct aplasia-hypoplasia (MU), renal malformations (R) and cervicothoracic somite dysplasia (CS). This rare disease (1/50 000 females) is sporadic and of unknown aetiology. The reported case is the first one with additional esophageal atresia and ovarianmature teratoma. Esophageal atresia first led to the diagnosis of VACTERL association, which is more frequent and well known, showing that the identification of such malformative association may be challenging. The presence of mullerrian abnormality has allowed the diagnosis of MURCS association, as there is no mullerrian hypoplasia in VACTERL association. Therefore the association of isolated or combined renal and cervical malformation with VACTERL features should lead to the search for mullerrian abnormalities.展开更多
基金Supported by Far-Eastern Memorial Hospital,No.FEMH-2025-C-016.
文摘BACKGROUND MURCS association,an acronym for Müllerian duct aplasia/hypoplasia,congenital renal agenesis/ectopia,and cervical somite dysplasia,presents unique anaesthetic challenges,particularly for airway control due to cervical spine anomalies.This case report adds to the limited literature by detailing airway management strategies in a paediatric patient with MURCS,emphasising the need for thorough preoperative evaluation and curated planning.CASE SUMMARY This report describes the successful anaesthetic and airway management of a 6-year-old girl with MURCS undergoing dental extraction.To address the complexities of the patient’s condition,a multidisciplinary approach involving comprehensive preoperative assessment,meticulous planning,and advanced airway management techniques was adopted.CONCLUSION This report emphasizes individualized anesthetic strategies and interprofessional collaboration for managing rare congenital syndromes.
文摘The MURCS association which stands for Mullerian, Renal, Cervicothoracic Somite Abnormalities is a rare developmental anomaly seen in females. The clinical course of this disorder is not clearly defined as of yet-which may range from asymptomatic to severe disability. Here we present the case of a young 22-year-old female who was incidentally detected to have a generalized increase in bone density with an absent uterus. Further workup revealed a case of MURCS association with renal osteodystrophy. This is an as of yet unreported complication/association and must be kept in mind in the treatment of these patients.
文摘MURCS association includes Mullerrian duct aplasia-hypoplasia (MU), renal malformations (R) and cervicothoracic somite dysplasia (CS). This rare disease (1/50 000 females) is sporadic and of unknown aetiology. The reported case is the first one with additional esophageal atresia and ovarianmature teratoma. Esophageal atresia first led to the diagnosis of VACTERL association, which is more frequent and well known, showing that the identification of such malformative association may be challenging. The presence of mullerrian abnormality has allowed the diagnosis of MURCS association, as there is no mullerrian hypoplasia in VACTERL association. Therefore the association of isolated or combined renal and cervical malformation with VACTERL features should lead to the search for mullerrian abnormalities.
