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.

Obstetric and fetal short- and long-term outcomes of delayed-interval delivery in multiple pregnancies.

INTRODUCTION: Multiple pregnancies are at higher risk of preterm birth. However, both children do not necessarily have to be born and exposed to the morbidity of prematurity. One option is a delayed-interval delivery with reduction of morbidity and mortality for the second twin. The present case series demonstrates the feasibility and safety of this procedure including the long-term outcome of the surviving children compared to the overall outcome of premature born children at the same perinatal center. MATERIAL: Retrospective evaluation of all cases of delayed-interval deliveries in multiple pregnancies from 2003 to 2020 at the Women's and Children's Hospital of the University Hospital Ulm. RESULTS: In 17 cases, the delivery of the second twin could be delayed, on average by 36 days. Pregnancies with delivery of the first twin before 22 weeks of gestation had a longer prolongation than pregnancies with delivery of the first twin after 22 weeks (53 vs. 22 days). If a cerclage was placed after the delivery of the first twin a longer prolongation interval could be achieved (45 vs. 19 days). No severe maternal morbidity occurred, and the short- and long-term outcome of the second twin did not differ from age-matched controls. CONCLUSIONS: In multiple pregnancies with extremely preterm birth of the first twin, delivery of the second twin can in some cases be successfully postponed without serious maternal morbidity or additional fetal complications. Delivery of the first twin before 22 weeks of gestation and placement of a cerclage is associated with a longer prolongation interval.