JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
Hand-assisted Laparoscopic Splenectomy During Pregnancy for Hereditary Spherocytosis
Eric Wu, MS, Emad Kandil, MD
Laparoscopic surgery has advanced significantly in recent years and has improved outcomes in abdominal surgeries including splenectomy. However, anatomical and physiological changes during pregnancy can complicate splenectomy and pose unique risks to a laparoscopic approach.We present a case of hand-assisted laparoscopic splenectomy in a pregnant patient with hemolysis and splenomegaly secondary to hereditary spherocytosis.
CASE REPORT
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 teamagreedwith the surgical teamtoproceedwith 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 x 13.0 x 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.
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 years 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, respiratoryrate18/minute,SpO2100%onroomair, 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
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, inflexible redblood cells that aremore 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
J La State Med Soc VOL 169 NOVEMBER/DECEMBER 2017 155
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