///2010 Abstract Details
2010 Abstract Details2018-05-01T17:52:49+00:00

Blonde Hair and Blue Eyes: A Contraindication to Regional Anesthesia?

Abstract Number: 199
Abstract Type: Case Report/Case Series

Ashley M Tonidandel MD, MS1 ; Laura Dean MD2

Introduction: Hermansky-Pudlak syndrome (HPS) is an autosomal recessive genetic disorder associated with albinism, visual impairment, and a platelet dysfunction with prolonged bleeding. Affected individuals can be found in virtually every nationality, with the highest prevalence in Puerto Rica (up to 1 in 1800). Normal standard bleeding tests and varied amounts of pigmentation, including dark hair and eyes, complicate the diagnostic picture. Parturients with HPS are at risk for postpartum hemorrhage and epidural hematoma given their bleeding diathesis. We present a case of a term parturient with HPS, including anesthetic considerations for labor and Cesarean section.

Case Report: A 23 yo, 57" 72kg, G1P0 with blonde hair and blue eyes presented to our antenatal clinic to discuss labor analgesia options given her known diagnosis of HPS. Her identical twin accompanied her and received supportive care for the spontaneous nose bleed that occurred during the interview. The patient described increased nose bleeds during pregnancy to 2-3 episodes per day. She also reported commonly associated manifestations of poor vision and easy bleeding but denied pulmonary, renal, or GI complications. Physical examination revealed frequent nystagmus but was otherwise unremarkable. Multidisciplinary conferences were scheduled with hematologists and obstetricians to develop a plan of care. The parturient presented in spontaneous labor weeks later. She required a fentanyl PCA for labor analgesia. She had a prolonged stage 2 but eventually delivered an 8lb baby by forceps with an episiotomy and 4th degree laceration. She received iv oxytocin, im methylergonovine, and a pheresis pack of platelets for postpartum hemorrhage. Her hemoglobin dropped from 11.4 g/dL to 6.9 the following day, but she did not require DDAVP or additional transfusions. A circumcision was not performed on the male infant until HPS could be ruled out.

Discussion:

HPS has been described primarily in the obstetric literature with varying degrees of severity ranging from uneventful to death from postpartum hemorrhage.1,2 The purpose of this case description is to promote awareness of the anesthetic considerations of HPS. The combination of albinism, nystagmus or poor vision, and bleeding history should arouse a high index of suspicion. HPS is diagnosed by electron microscopy confirming the absence of platelet dense bodies. Bleeding and coagulation studies may be normal. While successful epidural placement has been described without neurologic sequalae,3 regional anesthesia is probably best avoided. Anesthesia providers should also be aware of the potential for pulmonary fibrosis and be prepared for postpartum hemorrhage, including possible need for DDAVP and platelet transfusion. Optimal anesthetic management for HPS parturients can be achieved with advance preparation and multidisciplinary collaboration.

1 Am J ObstGyn 1985;153:564-5

2 Am J Perinat 2001;18:159-61

3 Br J Anaesth 2004;93:740-2

SOAP 2010