Temporal trends of splenectomy in pediatric hospitalizations with hereditary spherocytosis from 2000 to 2019: A national survey.

BACKGROUND: Total and partial splenectomy are used in pediatric patients with hereditary spherocytosis to resolve anemia and hemolytic complications. PROCEDURE: Data from the Healthcare Cost and Utilization Project's Kid's Inpatient Database was used to profile and describe temporal trends in pediatric (≤18 years) hospital admissions in the United States from 2000 to 2019 data release years. Survey sampling methods were used to produce national estimates. RESULTS: From 2000 to 2019, the use of splenectomy declined overall, from 427 to 206 weighted procedures (difference = 222, 95% confidence interval [CI]: 124-320; p < .0001); the risk of undergoing splenectomy during admission also declined from 56.7% to 38.7% (risk difference = 17.9 percentage points [p.p.], 95% CI: 9.7-26.1; p < .0001). Total splenectomy was mostly used. Age at time of splenectomy increased 10.2 years (difference = 1.6 years, 95% CI: 0.6-2.7; p = .0018). The risk of splenectomy increased with age until 10 years, then leveled off until 18 years. The proportion of children aged ≤5 years undergoing splenectomy decreased from 27.7% to 11.2% in 2019 (risk difference: 16.5 p.p., 95% CI: 7.3-25.7; p = .0004). The strongest clinical predictors of splenectomy, adjusting for patient- and hospital-level characteristics, were a co-diagnosis of symptomatic cholelithiasis (adjusted odds ratio [aOR] = 3.18, 95% CI: 1.92-5.28; p < .0001) and splenomegaly or hypersplenism (aOR = 2.52, 95% CI: 1.74-3.65; p < .0001). Risk of splenectomy with splenomegaly or hypersplenism increased over time. CONCLUSION: Splenectomy was delayed until age greater than 10 years. Older age, co-diagnosis with splenomegaly or hypersplenism, or symptomatic cholelithiasis were strongest clinical predictors of splenectomy. Conservative management of hereditary spherocytosis appears to be more common.

[Clinical and genotypic analysis of hereditary spherocytosis combined with cholestasis among pediatric patients].

Objective: To understand the clinical and genetic characteristics of hereditary spherocytosis (HS) combined with cholestasis among pediatric patients. Methods: 12 cases of HS children accompanied by cholestasis at Hunan Children's Hospital were selected as the research subjects between January 2013 and December 2022. Clinical data were collected. Whole-exome sequencing was performed by second-generation sequencing. Suspected pathogenic mutation sites were verified by Sanger sequencing. Results: All pediatric patients were admitted to the hospital due to their yellow skin tone. Eight cases (66.67%) had a positive family history. The clinical manifestations were jaundice, splenomegaly (12/12), abdominal pain, anemia (4/12), and hepatomegaly (5/12). All pediatric patients had decreased hemoglobin, an increased reticulocyte ratio, total bilirubin and direct bilirubin, a positive erythrocyte fragility test, and remarkable spherical erythrocytes in their peripheral blood. Seven cases had elevated aminotransferase; four cases had severely elevated aminotransferase and bilirubin; eight cases had biliary calculi; and two cases had a dilated biliary tract. Liver pathological examination showed mild damage to the liver cells (G1S1) in three pediatric cases. Five children had a total of six unreported mutations: SPTB gene c.2431_2450del, c.4974-2A > G, c.2575G > A, and exon 22-35 deletion; ANK1 gene: c.2379-2380delC; and c .6dupC. Children still had abnormal bilirubin levels following treatment. Two pediatric cases underwent splenectomy. Bilirubin and hemoglobin levels returned to normal after surgery. Conclusion: Children with HS may experience cholestasis, and those with poor treatment results may consider undergoing a splenectomy. Six new types of variants have expanded the HS gene mutation spectrum.

Long-term haematological response and maintained immunological function after laparoscopic subtotal splenectomy in patients with hereditary spherocytosis.

INTRODUCTION: Subtotal or total splenectomy are recommended in severe and should be considered in intermediate forms of hereditary spherocytosis (HS). Data on laparoscopic subtotal splenectomy (LSTS) in HS patients are sparse. METHODS: Thirty three patients with HS (median age 10.7 years (yrs), range 1.8-15.5) underwent LSTS. Baseline and follow-up investigation included haematological parameters, microscopic analysis of pitted erythrocytes (pitE), and B-cell subpopulations assessed by flow cytometry. Results were compared to those of non-splenectomised HS patients, HS patients after total splenectomy (TS), and healthy individuals. RESULTS: After LSTS, haemoglobin levels were normalised in all patients. During median long-term follow-up of 3.9 yrs (range 1.1-14.9), only four patients presented mild anaemia. Despite re-growing of the remnant spleen none of the patients required a second surgical intervention. As compared to TS, PitE in LSTS patients were significantly lower and indicated normal to only moderately decreased spleen function. Relative but not absolute IgM memory B-cell counts were reduced in both LSTS and TS patients. CONCLUSIONS: LSTS is effective for the treatment of patients with HS. A small remnant spleen is sufficient to provide adequate phagocytic function and to induce a pool of IgM memory B-cells.

Treatment of asymptomatic gallstones in children with hereditary spherocytosis requiring splenectomy.

BACKGROUND: Gallstones are common in hereditary spherocytosis (HS) and other chronic hemolytic diseases, with most affected patients being asymptomatic. Whether and how asymptomatic gallstones should be treated is controversial. METHODS: We conducted a retrospective cohort study of pediatric patients with HS and asymptomatic gallstones to compare the clinical outcomes between the observation group (followed up with gallstones in situ) and the intervention group (cholecystectomy or cholecystolithotomy). The primary outcome was the composite of adverse outcomes, including gallstone-associated, gallbladder surgery-associated, and splenectomy-associated events. Secondary outcomes included the changes in the size and number of gallstones and the recurrence of gallstones. RESULTS: Fifty-two patients were included (38 in the observation group and 14 in the intervention group), with a mean follow-up length of 5.2 years. Patients in the intervention group had a lower incidence of primary outcomes (7.1% vs. 42.1%) than patients in the observation group (OR, 0.12; 95%CI, 0.01-0.99). Patients with gallstones >5 mm in diameter had the highest risk of adverse events (47.4%). In the observation group, gallstone growth, disappearance, and stability were observed in 19.4%, 29.0%, and 51.6% of splenectomized HS patients, respectively. Small gallstones (diameter ≤5 mm) or sludge were more likely to be associated with disappearance (P = 0.04). CONCLUSIONS: Most asymptomatic gallstones grow or persist in splenectomized HS patients for an extended period. Surgical treatment of asymptomatic gallstones in HS patients requiring splenectomy is associated with a lower risk of adverse outcomes. LEVELS OF EVIDENCE: III (retrospective comparative study).

Hereditary spherocytosis before and after splenectomy and risk of hospitalization for infection.

BACKGROUND: The infectious burden in hereditary spherocytosis (HS) children before splenectomy has rarely been reported and the risk of severe postsplenectomy infection is controversial. METHODS: We conducted a retrospective study of pediatric patients with HS to evaluate the risk of infection presplenectomy and postsplenectomy. The primary outcome was any bacterial, Mycoplasma, or fungal infection that required hospitalization. The secondary outcomes were sepsis and septic shock. Appendectomized children were matched on age at surgery and enrolled as controls. RESULTS: In all, 232 patients were included. Before splenectomy, the primary outcome was identified in 51 (22.0%) patients, and the secondary outcome was identified in 1 (0.4%) patient. After splenectomy, the primary and secondary outcomes were detected in 8 (4.1%) and 1 (0.5%) patients, respectively. The risk of infection was higher presplenectomy than postsplenectomy (OR, 6.6; 95% CI, 3.0-14.2). HS patients had a higher risk of infection than the controls before surgery (OR, 3.7; 95% CI, 2.3-5.9) but not after surgery (OR, 1.4; 95% CI, 0.6-3.6). CONCLUSIONS: HS patients who require splenectomy later in life had a high incidence of hospitalization for infections. In contrast, postsplenectomy risk of hospitalization involving infection or severe infection was low. IMPACT: Patients with hereditary spherocytosis who require splenectomy later in life have a high risk of hospital admission for infections, especially those with severe hereditary spherocytosis. With vaccines or postoperative antibiotics, splenectomy does not increase the risk of infection or severe infections. Splenectomy may reduce the risk of hospitalization for infections by alleviating the complications of hereditary spherocytosis. With vaccines, prophylaxis, or advanced antibiotics, the benefits of splenectomy in children with hereditary spherocytosis and a heavy disease burden may outweigh the risks.

Case report: A follow up of a major pelvic splenosis.

The authors present a case of a young female with extensive pelvic splenosis, which was complicated by torsion of one of the splenosis nodules operated by laparoscopy. She has been followed during several years. The diagnosis was made on the basis of the history, imaging (ultrasound, CT scan, MRI, and Technetium 99m-labeled embrittled red blood cell scans), and blood workup. The diagnosis of splenosis can be made via complications such as torsion, infarction, hemorrhage, or most often incidentally. The treatment without symptoms is abstention.

The Successful Use of an Exchange Transfusion in a Child with Hereditary Spherocytosis Undergoing Congenital Cardiac Surgery.

Little is reported in the literature regarding hereditary spherocytosis (HS) and cardiopulmonary bypass (CPB). We present a case of a 19-month-old girl child who was referred for an atrioventricular septal defect (AVSD) and HS. The patient underwent surgical repair, and an exchange transfusion was performed at the initiation of CPB. No significant hemolysis or events attributed to HS were observed during or after CPB. The surgical repair of an AVSD in a pediatric patient with HS and total volume exchange transfusion is herein reported.

The Alexis® system for laparoscopic splenectomy in pediatric patients.

The laparoscopic splenectomy in pediatric patients is performed worldwide but often the disproportion between size of patients and size of organs requires an extra laparotomic access for spleen removal. The aim of the present study was to evaluate the safety and effectiveness of the Alexis® system to retrieve the spleen without additional laparotomic access. The charts of all patients who underwent splenectomy at our center during the last 5 years were retrieved. In all the cases the Alexis® system was placed in the umbilicus, thru which a 10 mm camera was inserted. Three additional 5 mm standard trocars were inserted. Seven patients, affected by spherocytosis (3), epidermoid cyst (2), idiopathic thrombocytopenic purpura (2) and thalassemia (1), underwent laparoscopic splenectomy at a median age of 10 years (range: 8-17). Median patients' weight was 32.5 kg (range: 25-71) and spleen size 15 cm (11-18). In all the cases, upon removal of the camera, the retrieval bag was inserted thru the umbilicus under direct view, the spleen retrieved, morcellated, and removed. No conversion nor enlargement of one of the ports nor an extra laparotomic access were required. The patients were discharged on the fifth post-operative day and the cosmetic results were excellent. Removal of the spleen can be safely performed without any additional laparotomy thru the Alexis® system placed in the umbilicus. This system is effective also in case of major patient/organ size disproportion and the final cosmetic aspect is excellent.

Laparoscopic Partial Splenectomy: A Critical Appraisal of an Emerging Technique. A Review of the First 457 Published Cases.

Background: Laparoscopic partial splenectomy (LPS) may allow avoiding total splenectomy (TS) complications and maximizing the advantages of mini-invasive approach. The objective of this review is to assess feasibility and safety of LPS, to compare this approach with alternative options. Materials and Methods: A literature review of articles reporting LPS is performed. Several parameters, including age, indication, surgical technique, devices used for splenic section/hemostasis, adverse outcomes, including morbidity/mortality, conversions to open surgery, conversions to TS, operative time (OT), and hospital stay (HS), are analyzed. Articles comparing LPS' results with those of open partial splenectomy and laparoscopic TS are also analyzed. Results: Fifty-nine articles reporting 457 LPS were included. Patients' characteristics varied widely, concerning age and indications, including hematological disease (hereditary spherocytosis, drepanocytosis), splenic focal masses, and trauma. Several technical options are reported. Mean OT and HS are 128 ± 43.7 minutes and 4.9 ± 3.8 days, respectively. No mortality and 5.7% morbidity are reported. Conversion rates to open surgery and to TS are 3.9% and 3.7%, respectively. Conclusions: In conclusion LPS is feasible and safe, with no mortality, low morbidity, and low conversion rates to laparotomy and to TS. LPS may be accomplished by various techniques and tools. Major complications are sporadically reported, thus potential risks should not be underestimated.

Partial splenectomy in children: Long-term reoperative outcomes☆.

PURPOSE: Partial, or subtotal, splenectomy (PS) has become an accepted alternative to total splenectomy (TS) for management of hematologic disorders in children, but little is known about its long-term outcomes. Here, we present our institutional experience with partial splenectomy, to determine rate of subsequent TS or cholecystectomy and identify if any factors affected this need. METHODS: All patients who underwent partial splenectomy at a single tertiary children's hospital were retrospectively reviewed from 2002 through 2019 after IRB approval. Primary outcome of interest was rate of reoperation to completion splenectomy (CS) and rate of cholecystectomy. Secondary outcome were positive predictor(s) for these subsequent procedures. RESULTS: Twenty-four patients underwent PS, at median age 6.0 years, with preoperative spleen size of 12.7 cm by ultrasound. At median follow up time of 8.0 years, 29% of all patients and 24% of hereditary spherocytosis (HS) patients underwent completion splenectomy at median 34 months and 45 months, respectively. Amongst HS patients who did not have a cholecystectomy with or prior to PS, 39% underwent a delayed cholecystectomy following PS. There were no significant differences in age at index procedure, preoperative splenic volume, weight of splenic specimen removed, transfusion requirements, preoperative or postoperative hematologic parameters (including hemoglobin, hematocrit, total bilirubin, and reticulocyte count) amongst patients of all diagnoses and HS only who underwent PS alone compared to those who went on to CS. There were no cases of OPSS or deaths. CONCLUSION: Partial splenectomy is a safe alternative to total splenectomy in children with hematologic disease with theoretical decreased susceptibility to OPSS. However, families should be counseled of a 29% chance of reoperation to completion splenectomy, and, in HS patients, a 39% chance of delayed cholecystectomy if not performed prior to or with PS. Further studies are needed to understand predictors of these outcomes.

Clinical Significance of Splenic Vessels and Anatomical Features in Laparoscopic Splenectomy.

Introduction: Laparoscopic splenectomy (LS) has become the standard treatment for benign hematological disorders and hypersplenism. However, serious complications such as pancreatic fistula and portal venous thrombosis (PVT) sometimes occur. We investigated the clinical significance of splenic vessels and anatomical features in LS. Methods: Patient data were collected from 32 patients who underwent LS. The indications for LS were hypersplenism due to liver cirrhosis, idiopathic thrombocytopenic purpura, hereditary spherocytosis, and others. Close contact of pancreatic tail with splenic hilum, spleen volume, and diameters of splenic vessels were evaluated on computed tomography images. Results: Close contact of pancreatic tail with splenic hilum was recognized in 15 of the patients. The close contact was significantly associated with operation time (P = .038), spleen volume (P = .021), and spleen volume/body surface area (BSA) ratio (P = .001). In multivariate analysis, spleen volume/BSA ratio was an independent factor for close contact (P = .022). PVT occurred in 3 cirrhosis patients, and the diameter of the splenic vein (SV) was significantly associated with PVT as a result of multivariate analysis (P = .027). Conclusion: Close contact of the pancreatic tail with the splenic hilum may cause a longer operation time at LS and be associated with spleen volume/BSA ratio. A larger SV diameter in cirrhosis patients may be related to PVT after LS.

Successful Splenectomy Management in a Patient With Moderate Factor VII Deficiency and Concomitant Severe Hereditary Spherocytosis.

By the advent of the effective therapies for many coagulation diseases and hereditary spherocytosis (HS), patient's survival has been improved significantly; however, if patients are diagnosed late or left untreated, both diseases could ominously be life threatening. Concurrent occurring of factor VII (FVII) deficiency and HS is extremely rare and there is no literature report that explain this condition, thus far. In this study, we confronted a 9-year-old female patient diagnosed with HS and enlarged spleen as a result of this blood disorder. Given to her sever signs and symptoms of splenomegaly, she was candidate for emergent splenectomy. However, assessment of coagulation tests revealed a prolonged prothrombin time, suggesting the moderate FVII deficiency. With a multidisciplinary consultation, we decided to performed total splenectomy with prophylaxis administration of totally 6 doses of active recombinant FVII, initiated 1 hour before surgery and followed until 30 hours postoperation. As a result of cautious undertaken in Mofid Children's Hospital, the patient did not experience any hemostatic defect. Patient is now 14-year-old, generally well-being under regular surveillance of FVII deficiency.

Open heart surgery in an infant with hereditary spherocytosis and a review of literature.

Hemolytic anemia often challenges congenital heart surgery. Hereditary spherocytosis is a rare familial hemolytic anemia. When associated with congenital heart disease, the safe performance of cardiopulmonary bypass becomes a priority. The increased risk of hemolysis during cardiopulmonary bypass could potentially lead to significant secondary organ damage. Till now, only very few reports of successful repair of a congenital heart defect in patients with hereditary spherocytosis have been reported. We report the only case of successful repair of a congenital heart defect in an infant with hereditary spherocytosis.

Conducción anestésica de un paciente pediátrico con micro esferocitosis hereditaria / Anesthetic approach on a pediatric patient with hereditary microspherocytosis

Introducción: La esferocitosis hereditaria (ESH) es una anemia hemolítica de observación frecuente, en la cual existen defectos cualitativos o cuantitativos de algunas proteínas de la membrana eritrocitaria que llevan a la formación de hematíes de forma esférica, osmóticamente frágiles, que son atrapados de formas selectiva y destruidos en el bazo, con incidencia variable y más frecuente en pacientes con descendencia europea. Objetivo: Describir la conducta clínica y anestesiológica de un paciente pediátrico con diagnóstico de micro esferocitosis hereditaria programado de forma electiva para procedimiento quirúrgico. Desarrollo: Se presenta un caso clínico de un paciente escolar con diagnóstico de micro esferocitosis hereditaria al cual se le realizó esplenectomía total electiva convencional. Con principal signo dolor a la palpación en hipocondrio izquierdo. Se condujo con anestesia total intravenosa con buenos resultados clínicos quirúrgicos, utilizando propofol a razón de 3 mcg/mL y ketamina a 0,2 mg/mL. La estrategia estuvo basada en cinco aspectos claves: evitar la hipoxia, la hipotermia, la acidosis, reducir la pérdida de sangre, así como un correcto control del dolor postoperatorio. Asociado a lo anterior es indispensable una estrecha vigilancia ya que estos pacientes pueden manifestar crisis hemolítica y aplásica. Conclusiones: El manejo perioperatorio del paciente con esferocitosis hereditaria depende de la severidad del cuadro clínico, de la anemia y su repercusión y del grado de hemólisis. La anestesia total intravenosa es una técnica segura para el tratamiento de pacientes con esferocitosis hereditaria(AU)

Introduction: Hereditary spherocytosis (HS) is a hemolytic anemia of frequent occurrence, in which there are qualitative or quantitative defects of some erythrocyte membrane proteins that lead to the formation of sphere-shaped red blood cells, which are osmotically fragile, and that are selectively trapped and destroyed in the spleen, with variable and more frequent incidence in patients with European descent. Objective: To describe the clinical and anesthesiological behavior of a pediatric patient with a diagnosis of hereditary microspherocytosis electively programmed for a surgical procedure. Development: A clinical case of a school-age patient with a diagnosis of hereditary microspherocytosis was presented. The patient underwent conventional elective total splenectomy. Pain was as the main sign on palpation to the left hypochondrium. The case was conducted with total intravenous anesthesia, with good surgical clinical results, using propofol at a rate of 3 mcg/mL and ketamine at 0.2 mg/mL. The strategy was based on five key aspects: avoid hypoxia, hypothermia, acidosis, reduce blood loss, as well as proper control of postoperative pain. Associated with the above-mentioned, close monitoring is essential, as these patients may manifest hemolytic and aplastic crisis. Conclusions: The perioperative management of the patient with hereditary spherocytosis depends on the severity of the clinical status, the anemia and its repercussion, and the degree of hemolysis. Total intravenous anesthesia is a safe technique for the treatment of patients with hereditary spherocytosis(AU)

Long-Term Evaluation of the Outcomes of Subtotal Laparoscopic and Robotic Splenectomy in Hereditary Spherocytosis.

BACKGROUND: Hereditary spherocytosis (HS) is a common inherited disease affecting the erythrocyte membrane. Total splenectomy (TS) is effective in reducing hemolysis and decreasing the need of transfusions, but total removal of the spleen represents a potential risk factor for infectious and non-infectious complications. On the other hand, subtotal splenectomy (STS) could be an alternative therapy for HS. The aim of this study is to establish which surgical approach has the best outcome in HS. METHODS: All patients (n = 63) receiving splenectomy for HS between 2002 and 2016 from one institution were retrospectively reviewed. Hemoglobin and reticulocytes levels during preoperative and postoperative follow-up periods were compared. Additionally, a meta-analysis was performed analyzing data regarding hemoglobin and reticulocytes levels from several available studies. RESULT: At 1-year follow-up, our clinical data showed that mean hemoglobin levels increased after TS from (mean ± SD) 9.77 ± 1.82 to 11.88 ± 2.08 g/dl, while after STS from 8.98 ± 1.66 to 11.87 ± 1.38 g/dl. At 3-year and 5-year follow-up after TS, we observed an increase from 9.77 ± 1.82 to 13.59 ± 2.03 and 13.46 ± 1.64 g/dl, respectively. At 3-year and 5-year follow-up after STS in our cohort, we observed an increase from 8.98 ± 1.66 to 13.21 ± 1.95 and 13.68 ± 1.65 g/dl, respectively. The meta-analysis (for a follow-up period of 1 year) showed that the hemoglobin levels increased with 2.61 g/dl (95% CI 2.15-3.08 g/dl; p < 0.001) after TS, and with 1.67 g/dl (95% CI 1.25-2.10 g/dl; p < 0.001) after STS. CONCLUSION: We conclude that subtotal and minimally invasive splenectomy could be considered as the first line of treatment in severe HS cases, especially in children.

Laparoscopic ligation of splenic vessels for the treatment of hereditary spherocytosis in children.

BACKGROUND: Total splenectomy is the most effective surgical treatment for hereditary spherocytosis (HS). Nevertheless, post-splenectomy sepsis and hypoimmunity can pose a great risk to children. Some alternative treatments have been proposed to avoid the post-splenectomy complications. In this study, we propose such a procedure (laparoscopic ligation of splenic vessels, L-LSV) for the treatment of HS in children and investigate its effectiveness and feasibility. MATERIALS AND METHODS: A total of 17 children with HS who underwent the L-LSV at our hospital between May 2015 and Apr 2018 were enrolled in the current study. All patients were followed-up for 3-38 months (mean 19.8 months). The volume of spleen was preoperatively and postoperatively measured using the AW VolumeShare5. The size of functional spleen and the condition of splenic infarction were evaluated using ultrasound and computed tomography (CT) after surgery. The routine blood, biochemistry and coagulation tests were carried out after surgery. RESULTS: The L-LSV was successfully performed in all patients. The average operative time was 115 min (range 60-180 min). No patients underwent blood transfusion during surgery, and the length of hospital stay varied 5-9 days after surgery (mean 7 days). Postoperatively, the red blood cells, platelet and hemoglobin were significantly increased (P < 0.05). The postoperative volume of functional spleen was significantly smaller than preoperative volume of spleen (307.393 ± 177.634 cm3 vs. 581.242 ± 270.260 cm3, P = 0.000). The recent volume of functional spleen was significantly bigger than the postoperative 1 month volume of functional spleen in ten children who were followed-up for more than 1 year (P = 0.004). The index of splenic infarction (the proportion of the postoperative 1-month volume of splenic infarction in the preoperative volume of spleen) was 0.31-0.99 (mean 0.53). There were no patients undergoing the blood transfusion after surgery. CONCLUSIONS: The L-LSV is an effective treatment for HS in children; however, future studies should re-evaluate the long-term prognosis.

Splenic function is not maintained long-term after partial splenectomy in children with sickle cell disease.

BACKGROUND: Partial splenectomy (PS) may allow preservation of splenic function in cases where splenectomy is indicated for hematologic diseases; however, the long-term outcomes are uncertain. We investigated the long-term outcomes of PS in patients with sickle cell disease (SCD). METHODS: A single-institution retrospective chart review was performed for children with SCD who underwent PS from 1997 to 2017. For comparison, we reviewed outcomes for patients who underwent PS for hereditary spherocytosis (HS). The primary endpoint was viability of the splenic remnant as inferred by the presence of remnant perfusion on ultrasound and/or liver spleen scan. RESULTS: Nine patients with SCD and 26 patients with HS underwent PS at a median age of 11 (IQR, 9-14) and 7.5 (IQR, 6-13) years, respectively. All underwent laparoscopic PS with three (7.9%) conversions to open. Two SCD patients were lost to long-term follow-up. The remaining seven SCD patients had initial postoperative splenic remnant perfusion demonstrated by ultrasonography. By 42 months postoperatively, however, none had a functioning splenic remnant. The median time to loss of splenic remnant was 12.6 (IQR 9.2-28.5) months. In contrast, all HS patients demonstrated robust splenic remnant blood flow with a median follow-up of 46 (IQR 37-82) months. CONCLUSION: No patient with SCD who underwent PS had viable splenic tissue for more than 42 months, likely due to continued autoinfarction typical of patients with this disease. Therefore, we believe that PS to preserve splenic function is not indicated in patients with SCD. LEVEL OF EVIDENCE: III.

Hereditary spherocytosis: a risk factor for thigh pressure myonecrosis in posterior spine surgery.

The objective of this study was to make surgeons aware of a potential pressure complication in posterior spine surgery for patients with hereditary spherocytosis (HS) and to present a plausible hypothesis for injury. Posterior spine surgery is common practice for adolescent idiopathic scoliosis (AIS). Common, less severe surgical risks include pressure ulcers; while rare, more severe pressure complications include rhabdomyolysis and compartment syndrome. In patients with HS, a familial hemolytic disorder with altered red cell deformability, it is unknown if their red cell disorder is an additional risk factor for pressure-related surgical injuries. Two patients with HS, an 18-year-old male and a 17-year-old female, were both post-splenectomy and underwent revision posterior spinal fusion and instrumentation for progressive AIS. Surgery lasted 9 hours and 7 hours respectively, with no intraoperative complications other than prolonged surgical time due to revision nature of the deformities. Thigh redness and swelling was noted in both patients directly deep to the thigh pads. Thigh myonecrosis was diagnosed with eventual recovery in both cases. Patients with HS may be at inherent more risk of pressure complications during posterior spine surgery. We propose that thigh myonecrosis occurs with decreased perfusion and hemolysis from HS erythrocytes' inherent fragility, decreased deformability within capillaries, and prolonged microvasculature compression from positioning, causing poor microvascular perfusion, tissue ischemia, and reperfusion injury. Level of veidence: IV.