J-LSMS 2017 | Annual Archive

JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY

increased risk of coagulopathy during times of acute stress, such as surgery.

These considerations suggest the outcome of the patient in this report may have been improved with additional prenatal planning and emphasis on prenatal care attendance. An aggressive search for underlying cardiac and hepatic disease may have allowed for additional preparation for vaginal delivery. Intense physical therapy and orthopedic consultation may have made vaginal delivery possible and resuscitative measures may have been further employed to facilitate this. If the patient was more aggressively resuscitated, it is possible that she may have been a candidate for operative vaginal delivery in the setting of non-reassuring fetal heart tones. If surgery is done, efforts to reduce the need for additional surgical procedures, such as abdomino-pelvic pressure packing that is removable through the vagina as treatment for diffuse bleeding, were prudent. Furthermore, the patient’s multiple pain crises suggested a need for more aggressive management including possible prenatal exchange transfusion, fluid and electrolyte replacement, and planning for massive intrapartum use of blood products. Cooperation with interventional radiology may have been employed to reduce bleeding risk perioperatively or even prophylactically with intermittent balloon occlusion of the hypogastric arteries or uterine artery embolization. It seems apparent in retrospect that the risk of a poor outcome in this patient was very high, even with prenatal preparation. Prenatal counseling should have focused on the risks and emphasized the importance of frequent prenatal care with this patient. Preconception counseling regarding the degree of pregnancy risk should ideally have been discussed, and sterilization considered in these situations.

REFERENCES

1. Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199:125.e1. 2. Malinowski AK, Shehata N, D'Souza R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta- analysis. Blood 2015;126:2424. 3. Niss O, Quinn CT, Lane A, et al. Cardiomyopathy with Restrictive Physiology in Sickle Cell Disease. JACC Cardiovasc Imaging 2016;9:243 4. Charlotte F, Bachir D, Nénert M, et al. Vascular lesions of the liver in sickle cell disease. A clinicopathological study in 26 living patients. Arch Pathol Lab Med 1995;119:46. 5. Hurtova M, Bachir D, Lee K, et al. Transplantation for liver failure in patients with sickle cell disease: challenging but feasible. Liver Transpl 2011;17:381. 6. Saltzman JR, Johnston DE. Sickle cell crisis and cocaine hepatotoxicity. Am J Gastroenterol 1992;87:1661. 7. ShahNL, Northup PG, Caldwell SH. A clinical survey of bleeding, thrombosis, and blood product use in decompensated cirrhosis patients. Ann Hepatol 2012;11:686.

William Robinson, MD, and Syem Barakzai are both affiliated with Tulane University School of Medicine, in New Orleans, Louisiana.

130 J La State Med Soc VOL 169 SEPTEMBER/OCTOBER 2017

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