Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

Zafer Dogan; Huseyin Yildiz; Ismail Coskuner; Murat Uzel; Mesut Garipardic

doi:10.1016/j.bjane.2012.12.006

Clinical Information

Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

Anestesia em paciente com anemia de Fanconi e displasia do desenvolvimento do quadril: relato de caso

Zafer Dogan; Huseyin Yildiz; Ismail Coskuner; Murat Uzel; Mesut Garipardic

http://dx.doi.org/10.1016/j.bjane.2012.12.006 Braz J Anesthesiol, vol.64, n3, p.201-204, 2014

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Abstract

Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.

Keywords

Fanconi anemia, Caudal anesthesia, Developmental dislocation of the hip

Resumo

A anemia de Fanconi é uma síndrome hereditária autossômica recessiva rara, caracterizada por deficiência da medula óssea e anomalias congênitas e hematológicas. A literatura sobre o manejo anestésico dos pacientes é limitada. O manejo de uma displasia do desenvolvimento do quadril foi descrito em um paciente com anemia de Fanconi. Por causa da natureza heterogênea, um paciente com anemia de Fanconi deve ser submetido à avaliação pré-operatória para diagnosticar as características clínicas. Em conclusão, o bloqueio caudal foi a nossa escolha para esse paciente com anemia de Fanconi, sem trombocitopenia, para evitar o N2O, reduzir a quantidade de anestésico, a microcefalia existente, o hipotireoidismo e o aumento das enzimas hepáticas, proporcionar analgesia pós-operatória e reduzir a quantidade de analgésico usada no pós-operatório.

Palavras-chave

Anemia de Fanconi, Bloqueio caudal, Displasia do desenvolvimento do quadril

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Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

Anestesia em paciente com anemia de Fanconi e displasia do desenvolvimento do quadril: relato de caso

Zafer Dogan; Huseyin Yildiz; Ismail Coskuner; Murat Uzel; Mesut Garipardic

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