Literature DB >> 35813405

Peripartum management of tuberous sclerosis complex complicated by massive angiomyolipomas.

C Knapp1, M Ince1, A Zacharzewski2.   

Abstract

Tuberous sclerosis complex is a genetic disease that can affect multiple organs, commonly the renal, central nervous, cardiac and respiratory systems. Here, we present the peripartum management of a woman with a new diagnosis of tuberous sclerosis complex associated with massive renal angiomyolipomas. There was significant risk of catastrophic haemorrhage from the angiomyolipomas during pregnancy. This created a challenging scenario for the anaesthetists, obstetricians, urologists and interventional radiologists involved in their care, necessitating collaborative working, numerous investigations and honest patient-centred discussions. Evidence for the management of tuberous sclerosis and angiomyolipomas in pregnancy does not appear to have been previously reported. After much consideration, arterial embolisation of the most vascular area of the angiomyolipoma was completed at 28 weeks gestation under remifentanil sedation. An elective caesarean section under combined spinal epidural anaesthesia was completed at 37 + 3 weeks gestation. Central nervous system involvement must always be considered; benign tumours within the brain and spinal cord may contraindicate neuraxial techniques. Early multidisciplinary involvement is essential due to the diverse and complex nature of tuberous sclerosis. Individuals differ in their presentation and disease severity; therefore, they must be assessed on an individual basis with management tailored accordingly.
© 2022 Association of Anaesthetists.

Entities:  

Keywords:  pregnancy: haemodynamic effects; renal insufficiency: diagnosis; spinal anaesthesia: complications; surgery risk during pregnancy

Year:  2022        PMID: 35813405      PMCID: PMC9254078          DOI: 10.1002/anr3.12176

Source DB:  PubMed          Journal:  Anaesth Rep        ISSN: 2637-3726


  9 in total

1.  Rupture of renal angiomyolipoma during childbirth.

Authors:  Ann Kathrin Orywal; Martin Zeile; Roland Brüning; Andreas J Gross; Christopher Netsch
Journal:  Urology       Date:  2015-02-07       Impact factor: 2.649

2.  General anaesthesia for caesarean section in a woman with tuberous sclerosis.

Authors:  J Bowditch; R Russell; S McCready
Journal:  Int J Obstet Anesth       Date:  2017-05-05       Impact factor: 2.603

3.  Labor analgesia and cesarean section in women affected by tuberous sclerosis: report of two cases.

Authors:  A Causse-Mariscal; M Palot; H Visseaux; C Daigremont-Botmans; J M Malinovsky
Journal:  Int J Obstet Anesth       Date:  2007-03-06       Impact factor: 2.603

4.  Effects of pregnancy on the renal and pulmonary manifestations in women with tuberous sclerosis complex.

Authors:  Anna L Mitchell; Melissa A Parisi; Virginia P Sybert
Journal:  Genet Med       Date:  2003 May-Jun       Impact factor: 8.822

Review 5.  Anaesthesia and tuberous sclerosis.

Authors:  J J Lee; M Imrie; V Taylor
Journal:  Br J Anaesth       Date:  1994-09       Impact factor: 9.166

Review 6.  Tuberous sclerosis complex: perioperative considerations.

Authors:  Matthew J Rabito; Alan David Kaye
Journal:  Ochsner J       Date:  2014

7.  Renal angiomyolipoma during pregnancy: Case report and literature review.

Authors:  Cihan Çetin; Selim Büyükkurt; Cansun Demir; Cüneyt Evrüke
Journal:  Turk J Obstet Gynecol       Date:  2015-06-15

Review 8.  An update on recent developments in rupture of renal angiomyolipoma.

Authors:  Chenyang Wang; Xinyuan Li; Linglong Peng; Xin Gou; Jing Fan
Journal:  Medicine (Baltimore)       Date:  2018-04       Impact factor: 1.889

9.  Incidence of tuberous sclerosis and age at first diagnosis: new data and emerging trends from a national, prospective surveillance study.

Authors:  Daniel Ebrahimi-Fakhari; Lilian Lisa Mann; Martin Poryo; Norbert Graf; Rüdiger von Kries; Beate Heinrich; Darius Ebrahimi-Fakhari; Marina Flotats-Bastardas; Ludwig Gortner; Michael Zemlin; Sascha Meyer
Journal:  Orphanet J Rare Dis       Date:  2018-07-17       Impact factor: 4.123

  9 in total

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