Anesthetic Management of a Patient With Tetra-amelia

Williams, AR; Mk, Bailey

doi:10.1097/00007611-199903000-00014

Cited by 6 publications

(4 citation statements)

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“…Femoral venlerin santral yol için uygun olup olmadığı yine ultrasonografi eşliğinde değerlendirilme-lidir. (9) Sirenomeli olgularının intraoperatif yö-netiminde böbrek ve akciğer işlevleri ile planlanan cerrahinin tipine (kolostomi, üriner stoma, ekstremitelerin ayrılma-sı) dikkat edilmelidir. Prenatal dönemde normal plasenta işlevlerinin varlığında bilateral renal agenezide bile elektrolit ve sıvı dengesinin normal olabileceği belirtilmektedir.…”

Section: Discussionunclassified

“…(1) Sirenomeli olgularında nöroaksiyal blokaj yöntemlerinin vertebra anomalileri nedeniyle olanaksız olduğu bu nedenle kaçınılması gerektiği bildirilmektedir. (5,9) Bösenberg (1) ise laparatomi planlanan sirenomeli olgusunda halotan ile anestezi indüksiyonundan sonra sakral hiatus kolaylıkla palpe edildiğinden % 0,25'lik bupivakain ile kaudal blokaj yaptığını bildirmiştir.…”

Section: Discussionunclassified

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Anesthetic Management of a Patient With Sirenomelia A Case Report

Yücel¹,

Begeç²,

Erdoğan³

et al. 2012

J Turk Anaesth Int Care

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ÖZETSirenomeli veya mermaid sendromu ender görülen kalıtsal bir anormallik olup, alt ekstremitelerin kısmen veya tamamen gelişmemesi veya füzyonu ile karakterizedir. Gastrointestinal ve genitoüriner kusurlar sıklıkla bu doğumsal bozukluğa eşlik eder. Periferik venlerin yerini belirlemedeki zorluk ve anormal ven drenajı nedeniyle intravenöz yol için ekstremite kullanımı sınırlı olabilir. Preoperatif dönemde eşlik eden anormallikler tespit edilmeli ve gereken önlemler alınmalıdır. Bu sunumuzda sirenomeli sendromu tanısı koyulan olguda anestezi uygulaması ve anestezi yönetimi tartışılmıştır.Anahtar kelimeler: Kaudal regresyon sendromu, sirenomeli, anestezi SUMMARY Anesthetic Management of a Patient with Sirenomelia: A Case ReportSirenomelia or mermaid syndrome is an extremely rare developmental malformation characterized by complete or partial hipotrophy or fusion of the lower limbs. Gastrointestinal and urogenital anomalies are often accompanies with this congenital syndrome. Difficulty in determining the location of the peripheral veins and abnormal venous drainage may limit the use of limb for intravenous access. Associated anomalies should be identified and measures should be taken during the preoperative period. In this report we discussed anesthetic management of a patient diagnosed as sirenomelia syndrome.

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Section: Discussionunclassified

Anesthetic Management of a Patient With Sirenomelia A Case Report

Yücel¹,

Begeç²,

Erdoğan³

et al. 2012

J Turk Anaesth Int Care

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“…Our patient's external airway anatomy was normal by exam. We did not pursue neck MRI or bronchoscopy to further evaluate her airway anatomy, as she had no known midline defects and previous case reports do not report difficulty with intubation in these patients [8].…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of a Parturient with Thalidomide Phocomelia

Blacker¹,

Angelo²

2012

OJAnes

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The teratogenicity of thalidomide has been known since the early 1960s [1]. Thalidomide is currently used world wide, including the United States, to treat erythema nodosum leprosum, multiple myeloma, refractory Crohn's disease, aphthous stomatitis and HIV wasting syndrome. New cases of thalidomide phocomelia are being reported as well. We report a case of the anesthetic challenges of a 23 year-old parturient with thalidomide phocomelia and review the important anesthetic challenges it presents. Spontaneous vaginal delivery under continuous lumbar epidural was achieved in this challenging patient. However, it required careful planning for reliable intravenous access and the use of magnetic resonance imaging (MRI) of her pelvis and lumbar spine.

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“…The published literature regarding tetra-amelia has mainly focused on post-mortem findings and its other associated birth defects [1,2]. Case reports on the provision of anaesthesia for adults with isolated tetra-amelia have described difficulties in blood pressure monitoring, suggesting the use of novel techniques and devices [3,4], or have used regional anaesthetic techniques and clinical observations to mitigate the absence of intra-operative blood pressure monitoring [5]. We are not aware of reports highlighting the other challenges encountered in the peri-operative care of patients with tetra-amelia, such as temperature and fluid management or titration and dosing of analgesic and anaesthetic drugs.…”

Section: Introductionmentioning

confidence: 99%

Peri‐operative management of an adult with tetra‐amelia undergoing major abdominal surgery

Loughnan

Milan

2020

Anaesthesia Reports

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Summary Tetra‐amelia is a rare birth defect characterised by the complete absence of all four limbs. Affected infants are often stillborn or die shortly after birth. There is therefore limited experience in the management of this condition in surviving adults, and published guidelines on peri‐operative and anaesthetic management do not exist. We present a 34‐year‐old woman with tetra‐amelia who underwent an elective distal pancreatectomy and splenectomy for a mucinous tumour of the body and tail of the pancreas. The anaesthetic challenges highlighted by this case include difficult intravenous access, unachievable blood pressure monitoring, management of fluid balance status peri‐operatively, effective drug dose titration and temperature management. In the absence of intra‐operative blood pressure monitoring, other modalities were used to monitor haemodynamic and volume status peri‐operatively. As drug dosing guidelines do not exist for this condition and drug pharmacodynamics may be unpredictable, we recommend careful dose titration and bespoke multimodal analgesia, which is best achieved in a high dependency setting.

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Anesthetic Management of a Patient With Tetra-amelia

Cited by 6 publications

References 0 publications

Anesthetic Management of a Patient With Sirenomelia A Case Report

Anesthetic Management of a Patient With Sirenomelia A Case Report

Anesthetic Management of a Parturient with Thalidomide Phocomelia

Peri‐operative management of an adult with tetra‐amelia undergoing major abdominal surgery

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