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Hand-assisted Laparoscopic Splenectomy During Pregnancy for Hereditary Spherocytosis.

CASE REPORT

A 20-year-old woman G2P1001 at 12 + 1/7 weeks gestation presented to the emergency department with increasing shortness of breath, worsening fatigue, severe left upper quadrant (LUQ) abdominal pain, nausea, and vomiting. About 1.5 months prior to admission, she reported several episodes of severe nausea and vomiting, which she initially attributed to pregnancy. She also developed severe LUQ abdominal pain and scleral icterus. She had been hospitalized twice since then, requiring transfusion of four units of packed red blood cells for hemolytic anemia.

Her past medical history was significant for unspecified gallbladder disease, seizure disorder, and microcytic anemia. Although she had a history of anemia as an adolescent, she had not required blood transfusions prior to the start of her symptoms 1.5 months ago. Her family history is significant for splenectomies in her father at the age of five yea rs old for anemia and in several half siblings for unknown reasons. Family history in maternal family members was also significant for sickle cell disease and/or trait.

On arrival, her heart rate was 86 beats/minute, blood pressure 109/54mmHg, respiratory rate 18/minute,SpO2 100% on roomair, and she was afebrile. Physical exam revealed a pregnant female who was conversant, alert, and oriented. Head and neck exam was significant for a scleral icterus and pallor. Abdominal exam noted splenomegaly with moderate tenderness to palpation in the LUQ and no vaginal bleeding. Laboratory studies on arrival revealed normocytic anemia with hemoglobin of 7.2, elevated RDW of 26.1, decreased haptoglobin, hyperbilirubinemia of 4.2, and elevated LDH of 400. Blood smear revealed spherocytes, reticulocytes, and schistocytes. LUQ ultrasound revealed splenomegaly with the spleen measuring 12.6 cm in the craniocaudal dimension. She was evaluated for splenectomy for suspected hereditary spherocytosis. The obstetrics/gynecology service was consulted for assistance in the management of the patient, and the hematology/oncology service was consulted to confirm a diagnosis of hereditary spherocytosis and to rule out other hemoglobinopathies.

During admission, the patient's hemoglobin dropped to 6.9, and she required another unit of packed RBCs transfusion. Given her history of anemia, multiple transfusions, pregnancy, and suspected hereditary spherocytosis, the hematology/oncology team agreed with the surgical team to proceed with splenectomy to control her disease. She received preoperative vaccinations including Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis.

Patient underwent a hand-assisted laparoscopic splenectomy at 13 weeks gestation. She was positioned in the right lateral decubitus position. The spleen was 1117g and 25.5 * 13.0 * 5.5 cm and removed intact via the hand-assisted port. The patient had an uneventful postoperative course and was discharged home on the third post-operative day. Outpatient follow-up visits revealed improved symptoms, well-healing abdominal incisions, and reassuring fetal heart tones. The patient had an uneventful delivery.

DISCUSSION

Hereditary spherocytosis is the most common inherited chronic hemolytic disorder in northern Europe and North America affecting 1 in 2000 people. (1) It is caused by any of a number of defects in erythrocyte membrane proteins that result in abnormally shaped, infilexible red blood cells that are more prone to rupture. The disease is characterized by anemia, jaundice, and splenomegaly, but severe disease can manifest with significant hemolytic episodes and cholelithiasis. The severity of anemia in hereditary spherocytosis is variable, ranging from asymptomatic to life-threatening anemia. (1) Because the majority of hemolysis in spherocytosis occurs within the spleen, splenectomy is considered curative. However, splenectomy is usually reserved for individuals with severe hemolysis. Although our patient was reportedly anemic during adolescence, she had not required blood transfusions prior to her current pregnancy. Reports indicate that pregnancy may precipitate hemolytic crises and anemia in patients with hereditary spherocytosis. (2)

While maternal morbidity and fetal outcomes have been reported to improve following splenectomy to treat hereditary spherocytosis, during pregnancy, the anatomical and physiological changes in body habitus, coagulation status, the cardiovascular system, and nearly every other organ system can pose unique challenges for surgery. (2-4) Non-obstetric surgical procedures are estimated to occur in 1 of every 5000 pregnancies. (5) In a 2005 systematic review of 54 studies of surgeries during pregnancy, overall rates of miscarriage and major birth defects were similar to that of the general obstetrical population. (6) In a recent retrospective cohort study of 6.5 million pregnancies, Balinskaite et al. estimated that there was one additional stillbirth for every 287 surgeries during pregnancy and one additional preterm delivery for every 31 surgeries during pregnancy. (7) Although it was not possible to separate out whether these outcomes were due to surgery, anesthesia, or the underlying disease itself, the authors concluded that risks of non-obstetric surgery during pregnancy were relatively low. (7)

While fetal outcomes have been shown to be generally good, outcomes of non-obstetric surgery for maternal morbidity and mortality are still under debate. A retrospective cohort study of nearly 5600 pregnant women in Taiwan who underwent non-obstetric surgical procedures showed significantly increased adverse events including increased mortality, postoperative septicemia, and longer hospital stays. (8) However, a similarly designed retrospective cohort study matching over 2500 pregnant women in the United States to non-pregnant controls undergoing non-obstetric surgical procedures found no difference in overall morbidity or 30-day mortality rates. (9) Despite conflicting evidence, surgical delay in acute conditions such as appendicitis has been associated with worse outcomes for both the mother and fetus. (10) Non-obstetric surgery when indicated is generally recommended given their comparable fetal outcomes, encouraging maternal morbidity and mortality outcomes, and the potential for worse outcomes when surgery is postponed in acute conditions.

Pregnancy was previously a contraindication for laparoscopic surgery due to the potential of insufflation to decrease the blood flow to the placenta causing fetal hypoxia, in addition to the risk of perforating the gravid uterus during trocar insertion. However, the technological advances in laparoscopic surgery have improved outcomes of laparoscopic abdominal surgeries outcomes relative to laparotomy during pregnancy." Laparoscopic benefits include shorter operative time, which leads to reduced fetal exposure to anesthesia, decreased length of stay, and decreased wound complications."

Laparoscopic hand-assisted splenectomy has been shown to have favorable outcomes compared to an open approach including lower morbidity, lower transfusion rate, and shorter hospital stays. (12-14) Splenectomy during pregnancy poses a more difficult challenge given the spleen's high vascularity and its frequent enlargement in disease processes necessitating surgery. Some studies have shown that splenectomy during pregnancy is associated with increased risk of preterm delivery, higher transfusion rates, and longer hospital stays. (1516) Splenectomy during pregnancy is relatively rare, but several cases have been reported in patients with immune thrombocytopenia (ITP), splenic artery rupture, and hereditary spherocytosis. (17-20) Laparoscopic splenectomy has been shown to be successful and safe during pregnancy in several cases. (2021) A hand-assisted approach to laparoscopic splenectomy, as was performed in our case, may be used to provide better vascular control, improved visualization, and decreased operative time while still retaining benefits of laparoscopy such as faster healing time and decreased length of hospital stay. (22) Given the significant splenomegaly in our case, the hand-assisted port provided an improved surgical approach for splenectomy.

CONCLUSIONS

This case demonstrates a successful laparoscopic splenectomy in a pregnant patient with significant splenomegaly secondary to hereditary spherocytosis. Splenectomy during pregnancy was previously considered a high-risk procedure due to the increased risk of fetal loss and poor maternal outcomes. Planning to perform surgery during the early part of the second trimester when organogenesis of the first trimester is complete and the gravid uterus is minimal in size would be most advantageous to improve fetal and maternal outcomes. A hand-assisted port can provide improved vascular control during splenectomy while preserving advantages that laparoscopy offers. Advances in surgical and laparoscopic techniques have made laparoscopic splenectomy a feasible approach in pregnant patients.

REFERENCES

(1.) Perrotta, S., Gallagher, P. G. & Mohandas, N. Hereditary spherocytosis. Lancet Lond. Engl. 372, 1411-1426 (2008).

(2.) Pajor, A., Lehoczky, D. & Szakacs, Z. Pregnancy and hereditary spherocytosis. Report of 8 patients and a review. Arch. Gynecol. Obstet. 253, 37-42 (1993).

(3.) Brenner, B. Haemostatic changes in pregnancy. Thromb. Res. 114, 409-414 (2004).

(4.) Ouzounian, J. G. & Elkayam, U. Physiologic changes during normal pregnancy and delivery. Cardiol. Clin. 30, 317-329 (2012).

(5.) Kammerer, W. S. Nonobstetric surgery during pregnancy. Med. Clin. North Am. 63, 1157-1164 (1979).

(6.) Cohen-Kerem, R., Railton, C., Oren, D., Lishner, M. & Koren, G. Pregnancy outcome following non-obstetric surgical intervention. Am. J. Surg. 190, 467-473 (2005).

(7.) Balinskaite, V. et al. The Risk of Adverse Pregnancy Outcomes Following Nonobstetric Surgery During Pregnancy: Estimates From a Retrospective Cohort Study of 6.5 Million Pregnancies. Ann. Surg. 266, 260-266 (2017).

(8.) Huang, S.-Y. et al. Outcomes After Nonobstetric Surgery in Pregnant Patients: A Nationwide Study. Mayo Clin. Proc. 91, 1166-1172 (2016).

(9.) Moore, H. B. et al. Effect of Pregnancy on Adverse Outcomes After General Surgery. JAMA Surg. 150, 637-643 (2015).

(10.) Abbasi, N., Patenaude, V. & Abenhaim, H. A. Management and outcomes of acute appendicitis in pregnancy-population-based study of over 7000 cases. BJOG Int. J. Obstet. Gynaecol. 121, 1509-1514 (2014).

(11.) Cox, T. C. et al. Laparoscopic appendectomy and cholecystectomy versus open: a study in 1999 pregnant patients. Surg. Endosc. 30, 593-602 (2016).

(12.) Targarona, E. M. et al. Splenomegaly should not be considered a contraindication for laparoscopic splenectomy. Ann. Surg. 228, 35-39 (1998).

(13.) Targarona, E. M. et al. Effect of spleen size on splenectomy outcome: A comparison of open and laparoscopic surgery. Surg. Endosc. 13, 559-562 (1999).

(14.) Park, A. E., Birgisson, G., Mastrangelo, M. J., Marcaccio, M. J. & Witzke, D. B. Laparoscopic splenectomy: outcomes and lessons learned from over 200 cases. Surgery 128, 660-667 (2000).

(15.) Gershovitz, M. et al. Pregnancy outcome in women following splenectomy. J. Womens Health 2002 20, 1233-1237 (2011).

(16.) Bleau, N., Czuzoj-Shulman, N., Spence, A. R. & Abenhaim, H. A. Safety of splenectomy during pregnancy. J. Matern.-Fetal Neonatal Med. Off. J. Eur. Assoc. Perinat. Med. Fed. Asia Ocean. Perinat. Soc. Int. Soc. Perinat. Obstet. 1-5 (2016). doi:10.1080/14767058.2016.1222365

(17.) Gottlieb, P., Axelsson, O., Bakos, O. & Rastad, J. Splenectomy during pregnancy: an option in the treatment of autoimmune thrombocytopenic purpura. Br. J. Obstet. Gynaecol. 106, 373-375 (1999).

(18.) Mahey, R., Kaur, S. D., Chumber, S., Kriplani, A. & Bhatla, N. Splenectomy during pregnancy: treatment of refractory immune thrombocytopenic purpura. BMJ Case Rep. 2013, (2013).

(19.) Jacobson, J., Gorbatkin, C., Good, S. & Sullivan, S. Splenic artery aneurysm rupture in pregnancy. Am. J. Emerg. Med. (2016). doi:10.1016/j. ajem.2016.12.035

(20.) Allran, C. F., Weiss, C. A. & Park, A. E. Urgent laparoscopic splenectomy in a morbidly obese pregnant woman: case report and literature review. J. Laparoendosc. Adv. Surg. Tech. A 12, 445-447 (2002).

(21.) Majesky, I. et al. Laparoscopic splenectomy in pregnancy - from contraindication to golden standard. Bratisl. Lek. Listy 114, 484-487 (2013).

(22.) Kusminsky, R. E., Boland, J. P., Tiley, E. H. & Deluca, J. A. Hand-assisted laparoscopic splenectomy. Surg. Laparosc. Endosc. 5, 463-467 (1995).

Eric Wu, MS, Emad Kandil, MD

Eric L. Wu, MS, and Emad Kandil, MD, are in the Division of Endocrine and Oncological Surgery, Department of Surgery at Tulane University School of Medicine, New Orleans, Louisiana.
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Author:Wu, Eric; Kandil, Emad
Publication:The Journal of the Louisiana State Medical Society
Article Type:Clinical report
Geographic Code:1USA
Date:Nov 1, 2017
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