Labor epidural analgesia in patients with neurofibromatosis type 1
Sherdiwala B, Subramanian H, Alsaden M. Labor epidural analgesia in patients with neurofibromatosis type 1. Regional Anesthesia and Pain Medicine 2017; 42(6).
Regional Anesthesia and Pain Medicine
Introduction Neurofibromatosis (NF) is an autosomal dominant genetic disorder affecting the nervous system. NF type 1 (NF1) is more common and accounts for 90% of all neurofibromatosis. NF1 has incidence of 1 in 3500 live births and affects about 75000 people in the US. NF1 involves multiple organ system and symptomatology varies from patient to patient. There has been limited research on the epidural analgesia during labor in patients with NF1. Experts recommend that patients with NF1 should be evaluated for increased intracranial pressure and for presence of spinal involvement by using CT or MRI before performing spinal or epidural. We present a case of pregnant female with NF1 whose labor was successfully managed with epidural analgesia. Results/Case report 27 year old G3P1102 and 38 weeks pregnant with history of NF1 with multiple cutaneous neurofibromas presented to the Labor and Delivery floor with rupture of membranes. She reported history of below knee amputation on right side due to bone involvement during her childhood. The patient had history of prior C-Section due to non-reassuring fetal heart tones under general anesthesia. During her second pregnancy, she had preterm spontaneous vaginal delivery with successful placement of epidural for labor analgesia. She had limited prenatal care in her current pregnancy and she presented in active labor with 10/10 contraction pain. The patient requested epidural analgesia for her labor pain. As patient presented in active labor, a plan was made to clinically evaluate patient to rule out spinal involvement. She denied any neurologic symptoms and thorough neurologic examination was normal. The patient was explained the risks of spinal involvement in NF1 and complications related to epidural placement in the event of spinal involvement. The patient had good understanding of the risks associated with the procedure and decided to go ahead with the epidural placement.The procedure was performed successfully by experienced anesthesiologist without any difficulty. She had adequate pain relief and had spontaneous vaginal delivery. The patient was discharged home after close observation for 2 days for any neurologic deficit. Discussion NF1 is a common genetic disorder with multi-system involvement posing challenges for anesthesiologists. There have been case reports describing successful placement of epidural in parturient for labor and c-section. It is recommended to perform such procedures after ruling out spinal involvement by CT or MRI. Many patients with NF1 may have airway pathology making them high risk for difficult intubation while getting general anesthesia. NF1 patients are also at high risks of difficult labor and cesarean delivery, in which case functioning epidural could be valuable. Moreover, many patients with NF1 have kyphosis or scoliosis, which could lead to potential technical difficulties. When CT or MRI is not possible due to urgency of pain or other reasons, as was the case with the patient, it is advisable that procedure be performed by experienced anesthesiologist. Hence, detailed clinical history and examination must be performed to rule out spinal involvement and patient should be explained in detail about the risks associated with the procedure.