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Obstetric Management of a Rare Disease: Telangiectasia Macularis Eruptiva Perstans
Abstract Number: T-51
Abstract Type: Case Report/Case Series
Introduction: Mastocytosis is a heterogeneous group of diseases characterized by abnormal infiltration of mast cells in skin and other organs. Clinical presentation may include life threatening anaphylaxis, skin lesions, pruritis, nausea, vomiting, & diarrhea secondary to mast cell degranulation. We report the use of a bupivacaine fentanyl spinal for cesarean & successful transverse abdominis plane block(TAPB) in a patient with telengiectasia macularis eruptiva perstans(TMEP).
Case: A 28 y/o G1P0, with twins at 33 wks presented for 1° cesarean section. PMHx was significant for cutaneous mastocytosis (CM). Patient developed a maculopapular rash during her second trimester. Exam revealed blanchable spider hemangiomas distributed on chest, upper arms, & back. Skin biopsy revealed TMEP, a rare form of CM where systemic involvement & anaphylaxis may occur. Her clinical history was non-contributory for TMEP except for chronic diarrhea. Serum triptase levels were normal at baseline & remained so throughout her gestation. A multidisciplinary meeting was scheduled to plan the peripartum care of this patient.
The patient presented for cesarean section at 33 wks due to IUGR. She was pretreated with ranitidine & diphenhydramine. A spinal anesthetic was administered in the usual fashion using 11.25mg bupivacaine & 25mcg of fentanyl, MSO4 was withheld due to possible histamine release. The patient had an unremarkable intraoperative course. Post operatively a TAPB was performed with 20cc 0.25% bupivacaine bilaterally. The patient had an uneventful post operative course. Pain was well controlled without the need for additional narcotics within the first 24 hours.
Discussion: Mastocytosis is a rare disease with an annual incidence of 0.1-0.8% of dermatology patients. Urticaria pigmentosa is most common form of CM and is limited to skin. TMEP is a rare form of CM that can evolve into systemic mastocytosis (SM). With SM, organ involvement is important to peripartum management because it can lead to thrombocytopenia, anemia, & bleeding diathesis. Mast cell mediators include histamine, leukotrienes, prostaglandin, platelet activating factor, & others, which can cause hemodynamic collapse, peripartum bronchospasm, & coagulopathy. The major consideration in the management of the parturient is avoiding drugs that would trigger mast cell degranulation. (lidocaine, morphine, oxymorphone, codeine, d-tubocurarine, metocurine, acetylsalicylic acid, etomidate, thiopental, succinylcholine, enflurane, & isoflurane).
The present case illustrates the importance of a multidisciplinary approach to the management of this rare disease in pregnancy and highlights the importance of reviewing the differential diagnosis when presented with rash in the parturient.