Pediatric Laparoscopic Splenectomy for Splenomegaly due to Hereditary Spherocytosis

Hereditary spherocytosis (HS) is a form of inherited hemolytic anemia seen in children. HS is characterized by anemia, jaundice, splenomegaly, and complications such as gallstone formation or growth delay. While mild cases may be managed conservatively, splenectomy remains the definitive treatment for patients with severe symptoms or complications. In this case, we present a 10-year-old male with HS who presented with anemia, fatigue, abdominal pain, and palpable splenomegaly. He was found to have splenomegaly with a splenic length of 19.6 cm. He ultimately underwent a laparoscopic total splenectomy after receiving appropriate preoperative vaccinations. The procedure was completed successfully without complications, and the patient was discharged on post-op day 3. At follow-up, he demonstrated improved hemoglobin levels, resolution of abdominal pain, and no early complications. This case highlights the role of laparoscopic total splenectomy as a safe and effective treatment for pediatric patients with hereditary spherocytosis and massive splenomegaly, offering durable hematologic improvement with the benefits of a minimally invasive approach.

Hereditary spherocytosis; pediatric splenectomy; laparoscopic surgery; hemolytic anemia; splenomegaly.

Hereditary Spherocytosis (HS) is an inherited hemolytic anemia caused by mutations in genes encoding red blood cell (RBC) membrane and cytoskeletal proteins. These defects result in spherical RBCs that are prone to being trapped and destroyed in the spleen, leading to anemia, jaundice, and splenomegaly. Patients can develop gallstone disease and growth delay as a consequence of this chronic hemolytic anemia.^1-3 While mild cases can be managed conservatively, children with moderate-to-severe symptoms often require splenectomy for definitive treatment. This is a case of a 10-year-old male who presented with abdominal pain and fatigue and was found to have splenomegaly. After testing confirmed he had HS, he was referred to our pediatric surgery clinic and subsequently underwent a laparoscopic splenectomy. The spleen was morcellated to maintain a minimally invasive approach with smaller incisions.

The patient is a 10-year-old boy with a history of Henoch-Schönlein purpura who initially came to the emergency department (ED) with one week of worsening abdominal pain, vomiting, fatigue, and poor appetite. Laboratory testing demonstrated anemia with a hemoglobin of 7.6, and abdominal CT revealed splenomegaly measuring approximately 17.5 cm in length, prompting transfer to a tertiary hospital. During admission, he was found to have hemolytic anemia, and genetic testing confirmed autosomal dominant spherocytosis. Family history was notable for his mother, who had previously undergone splenectomy for hereditary spherocytosis (HS), and a maternal grandmother with splenomegaly and concerns for hemolytic anemia. At follow-up clinic visits, management options, including observation and surgical management via splenectomy, were reviewed. Given his persistent anemia and family preference, he was scheduled for splenectomy following preoperative vaccinations.

Physical exam on initial presentation in the ED revealed an ill-appearing male with abdominal tenderness and scleral icterus. His vital signs showed that he was afebrile, tachycardic at 115, with a normal respiratory rate, normal blood pressure, and saturating well on room air. He reported severe abdominal pain (9/10), with tenderness to palpation along the left abdomen and right upper quadrant. Splenomegaly was palpable below the costal margin, extending to the level of the umbilicus. These findings are consistent with the triad of symptoms seen often in children with hereditary spherocytosis as a result of chronic hemolysis: anemia, jaundice, and splenomegaly.⁴ Additional findings in this population may include cholelithiasis and, in more severe cases, growth delay, short stature, or skeletal abnormalities, which arise from chronic hemolysis, persistent anemia, and increased metabolic demand.⁵

This patient underwent an abdominal ultrasound to evaluate spleen size and possible complications. He was found to have splenomegaly, measuring 19.6 cm in length, along with subcapsular wedge-shaped hypoechogenic areas and round soft tissue masses near the splenic hilum. The liver was mildly enlarged with normal shape and echotexture. The gallbladder was normal, with no cholelithiasis, wall thickening, or pericholecystic fluid. Ultrasound plays a key role in evaluating children with HS, particularly for assessing splenomegaly and detecting cholelithiasis. As mentioned, cholelithiasis can develop due to chronic hemolysis, and is recommended for screening in those with significant hemolysis starting around age 10, even if otherwise asymptomatic.⁵ Ultrasound also provides an objective measurement of spleen size, which can guide clinical decisions such as activity restrictions or planning for splenectomy. 

Options for treatment vary based on severity, patient age, symptoms, and family preference. They can vary from supportive care with folate supplementation and routine monitoring for mild cases to regular surveillance and transfusions for hemolytic or aplastic crises in more severe cases.⁵ A splenectomy is the definitive treatment, improving anemia, reducing hemolysis, and preventing long-term complications such as gallstones.

Splenectomy is indicated in children with HS who have significant anemia, transfusion needs, symptomatic splenomegaly, or complications from chronic hemolysis. A total splenectomy provides a more definitive hematologic resolution, eliminating transfusion needs. By eliminating the primary site of red blood cell destruction, total splenectomy has been shown to increase red cell lifespan, normalize hemoglobin levels, reduce reticulocyte counts and bilirubin, which are associated with improved growth parameters and reversal of skeletal changes over time.^6-9 However, it does carry a higher risk of overwhelming post-splenectomy infection (OPSI). Partial splenectomies offer hematologic improvement to a lesser degree, with preservation of immune function and lower infection risk. However, 25% of patients ultimately require complete splenectomy due to persistent hemolysis.^6,7,10-17 The choice between total and partial splenectomies remains individualized, taking disease severity, age, and family preference into consideration. If gallstones are present, concurrent cholecystectomy is recommended in HS patients.^18,19 

The patient tolerated the procedure well and was discharged on postoperative day 3 in stable condition. He was prescribed Penicillin V on discharge, and his drain, which produced only scant serosanguineous output, was removed the same day. At his 4-week follow-up visit, he demonstrated resolution of symptoms. Pathology confirmed an enlarged spleen with congested red pulp, consistent with hereditary spherocytosis, and no evidence of malignancy.

OPSI is most commonly caused by encapsulated organisms, such as Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae type b. It is more common in children 5 years of age or younger. Therefore, splenectomies for HS are not recommended for children until they are past the age of 6. Furthermore, appropriate vaccinations are essential when undergoing a splenectomy.²⁰ Vaccines are recommended to be administered at least 2 weeks prior to elective splenectomies or 2 weeks postoperatively if surgery is urgent, to optimize immune response and protection against encapsulated organisms. Postoperatively, antibiotic prophylaxis is recommended to further reduce the risk of OPSI. Oral penicillin V is the standard recommendation, with erythromycin as an alternative for patients allergic to penicillin.^5,20 The duration of prophylaxis remains controversial. The American Academy of Pediatrics (AAP) suggests discontinuation at age 5, The British Committee for Standards in Haematology recommends lifelong prophylaxis for all persons with asplenia. The literature does appear varied amongst hematologists, with some stating 1–3 years of prophylaxis and others advocating for lifelong prophylaxis.^5,20,21

In addition to infection prevention, technical considerations are equally critical to successful outcomes. Morcellation of the spleen is a widely used technique during laparoscopic splenectomy for HS, especially in cases with splenomegaly, and the specimen cannot be removed intact through a minimally invasive incision. In this case, we see that after the spleen is fully mobilized and vascular control is achieved, the spleen was placed in a specimen bag while in the abdominal cavity, then was morcellated using ring forceps, allowing for retrieval through the originally made 15-mm incision site. This approach minimizes incision size, allowing for decreased postoperative pain and improved cosmetic outcomes. It is imperative to note that the morcellation was done in a contained setting, such as in the specimen bag, as in this case. This is to avoid the spillage of splenic tissue, which could lead to splenosis or intra-abdominal infection.^22-25

Another key anatomic consideration is the close anatomic relationship between the splenic hilum and the tail of the pancreas. The tail courses within the splenorenal ligament, along with the splenic vessels. Because of this proximity, the pancreatic tail is at risk of injury during hilar dissection or splenic vessel ligation, particularly when the spleen is enlarged or when dissection planes are distorted. With meticulous surgical technique and careful control of the hilar vessels, clinically significant pancreatic injury in pediatric hereditary spherocytosis remains exceedingly rare.^25-27 Large pediatric series report no cases of pancreatic injury following laparoscopic splenectomy for HS specifically; however, broader pediatric and adult series report pancreatic injury in up to 9–16% in some studies, though most cases are mild and self-limited. Complications reported include asymptomatic hyperamylasemia, pancreatic collections, and pancreatic fistulae.²⁸ In this case, the tail of the pancreas was noted to be intimately involved with the splenic vein. This was carefully and meticulously taken off the splenic vein using sharp and blunt dissection. The tail of the pancreas appeared intact on inspection, but there were some close LigaSure lines. A 10-French round JP Blake drain was placed in the splenic bed as a precaution.

This case illustrates the importance of a comprehensive perioperative strategy, combining vaccination and prophylaxis to reduce infectious risk with meticulous surgical technique consisting of careful dissection and contained morcellation to ensure safe and effective splenectomy outcomes while maintaining a minimally invasive strategy for pediatric hereditary spherocytosis. Ultimately, surgical intervention not only mitigates the risks of complications such as gallstone disease and growth delay but also provides definitive resolution of the hemolysis underlying HS, restoring quality of life for affected children.

In addition to the standard pediatric laparoscopy tray, a specimen bag, an Endo GIA stapler, a Jackson Pratt (JP) Blake drain, and a handheld LigaSure were used. 

The authors have no conflicts of interest to disclose.

The patient’s parent referred to in this video article has given their informed consent for her son to be filmed and is aware that the information and images will be published online.