///2014 Abstract Details
2014 Abstract Details2019-07-18T14:34:47-05:00

Anesthetic Management for Cesarean section in a patient with Von Hippel-Lindau Disease and Growth Hormone deficiency

Abstract Number: F-06
Abstract Type: Case Report/Case Series

Uma M Tharmaratnam FRCP(C)1 ; Timothy Van Haaften MD2; Mrinalini Balki MD3; Tharmaratnam Meera medical student4

Introduction: Von Hippel-Lindau Disease (vHL) is an autosomal dominant multisystem disorder often associated with craniospinal axial hamangiomas (60-80%) and pheochromocytomas (10-20%). Both of these manifestations have significant impact on pregnancy and management of labor and delivery. The anesthetic management of this condition is still controversial. Growth hormone deficiency is associated with short stature with difficult vaginal delivery, and need for adjustment of drugs and equipment used for anesthesia.

Case report: A 36 years old G2P0 presented to the Obstetric anesthesia clinic at 28 weeks gestation. She was diagnosed with growth hormone deficiency at 2 years of age and was treated with hormone replacement therapy. Despite this, she had a short stature (weight 33 kg, height 111 cm) but proportional body habitus. She had a strong family history of vHL disease. Her first pregnancy at the age of 24 years was terminated due to a new diagnosis of pheochromocytoma. A bilateral adrenalectomy was done with a small portion of adrenal gland left behind. Investigations also revealed asymptomatic haemangiomas in lower thoracic and upper lumbar spinal cord. A repeat brain MRI during her current pregnancy was normal, while the spine MRI, without gadolinium, showed lesions suggestive of hemangiomas, however, the study was suboptimal. She developed hypertension, tachycardia with increasing urinary catecholamine levels, but had normal ACTH level and hence was started on calcium channel blockers for adequate control of blood pressure. A multi-disciplinary patient care conference was held at 33 weeks of gestation and an elective cesarean section (CS) was planned at 37 weeks for cephalo-pelvic disproportion. However, at 36 weeks, the patient went in to premature labor and an urgent CS was required. A general anesthetic technique was carried out with close hemodynamic monitoring after the placement of arterial line and non invasive cardiac output monitor. A 5.5 mm endotracheal tube was easily inserted after induction. She received medications appropriate for her weight and had good intraoperative hemodynamic stability. The post-operative course was also uneventful.

Discussion: Both regional as well as general anesthetic techniques can be successfully used in patients with vHL disease (1, 2). The anesthetic management has to be tailored to the individual patient depending on the extent of their disease. We chose to provide general anesthesia mainly because of the concerns of possible high block and disruption of spinal hemangiomas due to her short stature from regional anesthesia. Our patient presented a unique challenge, however, multi-disciplinary management and close hemodynamic control led to a successful outcome.

References: Demiraran Y et al. Euro J Anesthesiol 2001,18;330-2; Razvi SAH et al. Int J Obstet Anesth 2009,18;294-5.

SOAP 2014