Introduction. Extra-hepatic portal vein obstruction (EHPVO) is one of the most often causes of portal hypertension in children. Objective. Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism. Methods. Distal splenorenal shunt (DSRS) with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO. Indications for surgery were pain and abdominal discomfort caused by spleen enlargement, as well as symptomatic hypersplenism with leucopenia, thrombocytopenia and anemia. The partial spleen resection was performed by ligation of blood vessels to caudal two thirds of the spleen. After ischemic parenchymal demarcation transection with electrocautery LigaSure was performed with preservation of 20-30% of spleen tissue, and then Warren DSRS was created. Platelet and leucocytes counts and liver function tests were obtained before, one month and one year after surgery. Growth was assessed with SD scores (Z scores) for height, weight and body mass index at the time of surgery and one year later. Results. In all patients postoperative period was without significant complications. Platelets and leucocytes counts were normalized. Patency rate of shunts was 100%. Two significant shunts stenosis were observed and successfully treated with percutaneous angioplasty. During the follow-up period (1 to 9 years) all patients were asymptomatic, with improved quality of life and growth. Conclusion. Results of our study indicate that shunt surgery with a partial spleen resection is an effective and safe procedure for patients with enormous splenomegaly and severe hypersplenism caused by EHPVO.
Background: Surgical resection remains an important treatment of choice for a large number of liver tumors in children. Sometimes, if a tumor infiltrates a large part of the liver, after resection, the future liver remnant (FLR) is not enough for normal liver function. The size of the FLR is one of the determining factors for resectability as postoperative liver failure (PLF) is the most severe complication after partial hepatectomy. A new strategy for treating marginally resectable liver tumors in adult patients which were initially considered as unresectable was formally reported in 2011. This operative technique is a hepatectomy consisting of two stages with initial portal vein ligation and in situ splitting of the liver parenchyma. In 2012, the acronym “ALPPS” (associating liver partition and portal vein ligation for staged hepatectomy) was proposed for this novel technique. However, there is a small number of ALPPS procedures performed in pediatric patients published in the literature. Objectives: The aim of this paper is to present the first case of a pediatric patient with a marginally resectable rhabdoid tumor of the liver which was initially considered unresectable and who was treated with two-stage hepatectomy. We report a case of a 4-month-old girl with a large rhabdoid tumor of the liver who underwent this procedure. Conclusions: ALPPS can be a valuable technique to achieve complete resection of pediatric liver tumors although indications for ALPPS in children still need further research mainly focused on validation of the minimally needed FLR in children undergoing extended liver resections. To our knowledge, this is the youngest patient on whom ALPPS was performed, and the only one with a rhabdoid tumor.
Background and Objectives: The immunological function of the appendix, as part of the gut-associated lymphoid tissue, and its immunological importance in developing and maintaining the intestinal immune system are not yet clearly understood. Therefore, we sought some answers by analyzing the lymphocyte population in appendiceal specimens and inflammatory markers from peripheral blood. Materials and Methods: After histopathological examination of appendiceal specimens, immunohistochemical analysis of lymphocyte subtypes CD8, CD19, and CD20 in appendiceal specimens was performed, and a panel of commercially available mouse monoclonal antibodies was used to detect immunopositivity. hematological and biochemical parameters were analyzed from peripheral blood samples, and results were correlated with immunohistochemical findings. Results: The study included 100 patients: n = 20 in the control group, where the appendix was removed during some other abdominal surgical procedure, n = 35 patients with simple appendicitis in Group A, and n = 45 with complicated appendicitis in Group B. There were 65.0% male patients, and the mean age at surgery was 10.8 ± 4.0 years. CD19 B-lymphocytes were significantly more present in the appendiceal specimens of patients with complicated appendicitis (p = 0.014 and p = 0.007), and CD8 T-lymphocytes in patients with simple appendicitis (p < 0.001). Also, there were significantly fewer CD8 T-lymphocytes in patients with complications (p = 0.024). Conclusions: The number of CD8 T-lymphocytes is increased in simple appendicitis and is considerably lower in patients who develop postoperative complications. CD19 lymphocytes were more present in complicated appendicitis.
Introduction. Waugh syndrome (WS) is a rare condition characterized by association of intussusception and gut malrotation. Due to non-operativne management for intussusception in certain number of patients, it still remains undiagnosed. So far, only less than hundred casses have been reported in literature, most of them treated surgicaly, with good outcome and no complication if surgery is done timely after symptoms appeared. Case presentation. We present a five month old infant which was admited due to vomiting, blood and mucus in stool. Clinical findings revealed abdomen which was painful on palpation, without distension and abdominal mass. Blood and mucus were noted on rectal examination. After native X ray, abdominal ultrasound was done and confirmed intussusception. After unsuccessful attempt of hydrostatic reduction, ultrasound was repeated, the target sign persisted, this time accompanied by obvious “whirlpool sign” and inverse ratio of superior mesentery artery and vein. Infant underwent emergency surgery, and bowel resection with ileo-colic T-T anastomosis. Recovery was complete after two weeks. Conclusion. Waugh syndrome should be considered whenever hydrostatic reduction fails in a patient with previously diagnosed intussusception, and ultrasound verification of the superior mesenteric artery and vein relationship should be insisted upon to confirm the diagnosis off associated malrotation. In proven Waugh syndrome, timely surgical intervention is indicated to prevent bowel resection.