Pediatric Echinococcosis of the Liver in Austria: Clinical and Therapeutical Considerations.

Echinococcosis is considered a neglected disease in most European countries. However, migratory flows of populations, long-term stays in endemic areas, uninterrupted tourism (travel to Echinococcus-endemic countries), traveling dogs and dog translocations from endemic areas, and inappropriate hygiene practices are potential factors that alarm public health officials. Identifying a cyst-like mass in the liver or lung of an individual with a travel history of likely exposure to sheepdogs in an area where the parasite Echinococcus (E.) granulosus (sive cysticus) is endemic advocates for a prompt preliminary diagnosis of cystic echinococcosis (CE), no matter the age of the affected individuals. Routine imaging techniques, including ultrasonography, computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans, are used to detect cysts. After a cyst has been discovered, serologic investigations are used to confirm the diagnosis. Typically, alveolar echinococcosis (AE) is found in older individuals. Yet young people are also affected because frequent oral exploration of the environment is a regular behavior for infants and toddlers. In this review, therapeutic considerations for pediatric echinococcosis-drug-based benzimidazole therapy; AE: atypical liver resection, the resection of individual or multiple segments, a right or left hemi-hepatectomy, or an extended hemi-hepatectomy; CE: PAIR-technique, cyst excision, liver segment(s) resection (laparoscopically or conventionally)-are revised following experience in one of the most affected regions of Europe. In addition, we performed a systematic review using three databases (i.e., PubMed, EMBASE, and Scopus) to evaluate the quality of evidence in published studies on pediatric echinococcosis.

Congenital segmental dilatation of the intestine: an in-depth review.

OBJECTIVE: Congenital segmental dilatation of the intestine (CSDI) is a rare gastrointestinal condition. We conducted a scoping review through MEDLINE and Google Scholar, collecting data from 1959 through August 2020 to better understand this peculiar disease. METHODS: The clinical and pathological features of 150 patients were reviewed. RESULTS: The mean age was 25.9 days, and 61.3% of patients were male. An antenatal diagnosis was made in 15.3% of patients. Predominant symptoms included abdominal distension (83.9%) and vomiting (61.3%). Pallor and anemia were associated with ileal CSDI. The most common sites of the lesion were the ileum (56%) and colon (27.3%). Associated anomalies occurred in 57.3% of the patients, of which the most common included other abnormalities of the digestive system (69.8%), abdominal wall (19.8%), and cardiovascular system (11.6%). Resection and anastomosis was performed in 83.3% of patients. Postoperative complications occurred in 10%. Normal ganglion cells were commonly found (97.3%), while muscle layer hypertrophy and atrophy were found in 14.7% and 13.3% of the patients, respectively. Abnormal interstitial cells of Cajal were identified in four patients. Death occurred in 12.7% of patients. Demise was significantly associated with the duodenal location of CSDI (Mantel-Cox test, p = 0.002). CONCLUSION: CSDI remains poorly understood, and mortality is associated chiefly with its duodenal location. Further research is needed, and biorepositories should be promptly set up to study this disease in the future better.

What is currently known about the topic? CSDI is a sporadic condition, which can represent a challenge for neonatologists.What new information is contained in this article? Pallor and anemia are found explicitly in ileal CSD, and the duodenal location of CSDI is associated with the highest mortality rate.

Markers of childhood lupus nephritis indicating disease activity.

Current treatment regimens for childhood lupus nephritis (LN) are associated with significant side-effects and toxicity in vulnerable phases of growth and development. The paucity of biomarkers particularly in childhood impedes the appropriate clinical management and the development of new therapeutics. We analyzed markers of immune system (BAFF, RANTES), complement (Bb, C1q, C3d-CIC, C5a) and endothelial cell activation (sVCAM-1) in children with LN (n=22, mean age 14.8±4.7 years), nephrotic syndrome (n=13) and age-matched healthy controls (n=20) to define parameters that correlate with LN activity. Complement fragments of the alternative (Bb, p=0.0004) classical (C3d-CIC, p<0.0001) and common pathway (C5a, p<0.0001) and the levels of BAFF (p<0.0001), RANTES (p=0.0002) and sVCAM-1 (p=0.0004) were significantly higher in active compared to inactive LN. Activation of complement was associated with the occurrence of anti-C1q antibodies and reduced complement C1q. Complement-activation fragments highly correlated with the markers for immune system and endothelial cell activation. The ensemble of these parameters may be of great value in identifying early flares or remissions of childhood LN, and moreover may prove useful in the assessment of new treatments and in determining the optimization of their use.

Surgical aspects in the treatment of patients with unilateral wilms tumor: a report from the SIOP 93-01/German Society of Pediatric Oncology and Hematology.

OBJECTIVE: To assess surgical aspects in the treatment of children with unilateral Wilms tumor based on data from the Cooperative Tumor Study SIOP 93-01 of the German Society of Pediatric Hematology and Oncology. SUMMARY BACKGROUND DATA: Although multiple international study trials exist for the treatment of nephroblastoma, the impact of surgical details and the outcome of the patients have not yet been described comparing different approaches of the trials. METHODS: Treatment results of SIOP 93-01 of the German Society of Pediatric Hematology and Oncology were analyzed regarding frequencies of operations by surgeons and hospitals, surgical approaches, and operating subspecialties. Special attention was given to surgical complications, postoperative tumor stages and event-free survival. RESULTS: Data sets from 757 of 1020 registered patients were received for evaluation. A unilateral Wilms tumor was observed in 512 of 757 children.Median follow-up was 4.8 years (1.2-10.7). Event-free survival rates were comparable for frequencies of operation by surgeons and hospitals, surgical approaches, and surgical specialties. Intraoperative tumor rupture rates were 12% in primarily operated patients (protocol violations) versus 3.2% inpatients after preoperative chemotherapy. There were 7% intraoperative ruptures for hospitals and surgeons performing 1 operation per year, and 3%when more than 4 operations per year were carried out. Sampling of hilar lymph nodes was often incomplete for all surgical subspecialties. CONCLUSION: While the event-free survival for all groups is equal, there may be some long-term complications as a result of the more intensified therapy required for patients who suffer intraoperative ruptures. This will be defined only with longer term studies of late effects of the more intensified therapy. There is,however an increased rate of complications and ruptures associated with the use of midline laparotomy rather than a transverse or thoracoabdominal incision.

Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria.

Background and Aim Congenital segmental intestinal dilatation (CSID) is a neonatal condition with unclear etiology and pathogenesis. Typically, the newborn with CSID presents with a limited (circumscribed) bowel dilatation, an abrupt transition between normal and dilated segments, neither intrinsic nor extrinsic perilesional obstruction, and no aganglionosis or neuronal intestinal dysplasia. We aimed to review this disease and the long-term follow-up at the Children's Hospital of the Medical University of Innsbruck, Tyrol, Austria. Study Design Retrospective 25-year review of medical charts, electronic files, and histopathology of neonates with CSID. Results We identified four infants (three girls and one boy) with CSID. The affected areas included duodenum, ileum, ascending colon, and sigmoid colon. Noteworthy, all patients presented with a cardiovascular defect, of which two required multiple cardiac surgical interventions. Three out of the four patients recovered completely. To date, the three infants are alive. Conclusion This is the first report of patients with CSID and cardiovascular defects. The clinical and surgical intervention for CSID also requires a thorough cardiologic evaluation in these patients. CSID remains an enigmatic entity pointing to the need for joint forces in identifying common loci for genetic investigations.

Comparison of low-molecular-weight heparin and antithrombin versus antithrombin alone for the prevention of symptomatic venous thromboembolism in children with acute lymphoblastic leukemia.

BACKGROUND: Children with acute lymphoblastic leukemia (ALL) have a substantial risk for thromboembolism (TE) that is related to L-asparaginase-induced antithrombin (AT) deficiency and placement of central venous lines. Recent in vitro studies showed that the anticoagulant effects of low-molecular-weight heparin were profoundly affected by endogenous AT levels in children undergoing ALL therapy. METHODS: A total of 112 consecutively recruited children with newly diagnosed ALL treated according to BFM 95/2000 protocols were enrolled in this trial. This prospective cohort study was carried out to determine the influence of combined low molecular weight heparin-prophylaxis (enoxaparin 1 mg/kg/ per day) and AT supplementation versus AT alone (noncontemporaneous control group) on the incidence of symptomatic TE during a follow-up of 240 days. RESULTS: To maintain AT plasma levels above 50%, nearly 60% of all children needed at least one, most children two or three AT supplementations during induction therapy. 12.7% of the children that did receive only AT-prophylaxis (n = 71) (95% CI = 6.0-22.7) developed objectively confirmed symptomatic TE, as compared with no TE in children after combined prophylaxis (n = 41) (95% CI = 0.0-8.6, P < 0.05). Thromboses were located in the sinovenous system in the brain (n = 3), the lower deep veins (n = 3), the upper deep veins (n = 2) and in an upper deep vein combined with pulmonary embolism (n = 1). CONCLUSION: Prophylaxis with enoxaparin was safe and effective in preventing TE. Although our data are encouraging, the in vivo efficacy of combined enoxaparin and AT prophylaxis to prevent symptomatic venous TE in children with ALL should be evaluated in a prospective randomized clinical trial.

Congenital fibrosarcoma as cause for fetal anemia: prenatal diagnosis and in utero treatment.

OBJECTIVES: To discuss diagnosis and management of a case of a rare fetal tumor complicated by fetal anemia due to intratumoral hemorrhage. CASE REPORT: We report on a 29-week-old fetus with a tumor in the posterior left shoulder region. The morphologic aspect of the tumor, lack of fetal movements and an increased middle cerebral artery (MCA) peak systolic velocity (PSV) were indicative of fetal anemia caused by intratumoral bleeding. Following intravascular blood transfusion the pregnancy was safely prolonged for 15 days, during which lung maturity was induced. After delivery the neonate underwent surgical excision. Histological examination revealed an infantile congenital fibrosarcoma. CONCLUSION: Anemia must be ruled out in cases with fetal tumors. MCA PSV is useful in diagnosis and surveillance in these fetuses.

Pure esophageal atresia with normal outer appearance: case report.

Isolated esophageal atresia is characterized by a long segment between the 2 esophageal pouches. This article presents a case of pure esophageal atresia with a 1-cm-long segment at the midportion without discontinuity that resembled the subtype II3 according to the Kluth atlas. Resection of the atretic segment and primary anastomosis were performed successfully.

Management of neonates with large abdominal wall defects and undescended testis.

OBJECTIVES: To assess, in a retrospective study, the outcome of different treatment modalities in newborns with undescended testes secondary to large abdominal wall or diaphragmatic defects. Large abdominal and diaphragmatic defects are known to be associated with cryptorchidism, yet the reported incidence varies widely. METHODS: A total of 112 neonates with large abdominal wall or diaphragmatic defects were treated from 1981 to 2005. Of the 55 male patients in this series, 9 (16.4%) presented with abdominal testes and 4 had an extra-abdominal testis (7.3%). RESULTS: The 2 patients undergoing primary orchiopexy had testes of normal size and in the normal position at last follow-up. In one of these patients in whom the testis was brought down to the internal inguinal ring, spontaneous descent occurred and the testis on the affected side was normal. The other patient required additional surgery and had an atrophic testis at last follow-up. In 1 patient with severe concomitant malformations, primary orchiectomy was performed. The 4 patients who did not receive initial treatment all lost their testes owing to atrophy. CONCLUSIONS: The results of the present study have indicated that primary orchiopexy should be attempted in all cases of abdominal wall defects associated with abdominal cryptorchid testes because it yields better testicular salvage rates. In cases in which the spermatic cord is not long enough to place the testis into the scrotum, mobilization and fixation at the lowest site possible resulted in better outcomes than leaving the testis in the abdomen.

Insular-type component follicular thyroid carcinoma in a 10-year-old girl--case report.

Insular-type carcinoma of the thyroid is a rare form of undifferentiated thyroid cancer. The manifestation of disease occurs mainly in adults and is extremely rare in children. Prognosis of this type of thyroid carcinoma is unfavorable in childhood. Because of its rarity, it is not yet clear whether these tumors carry the same risk of progression. Therapy of choice is total thyroidectomy combined with a cervical lymph node dissection. In children, surgery is associated with a higher risk of recurrent nerve palsy and loss of parathyroid glands than in adults. Therefore, identification and protection of the recurrent laryngeal nerve using electrical neuromonitoring as well as exact preparation of parathyroid glands may reduce these risks. The history of a 10-year-old girl with insular-type thyroid carcinoma is presented. Surgical considerations such as total thyroidectomy vs less-than-total thyroidectomy with unilateral or bilateral cervical lymph node dissection are presented. Prognosis concerning morbidity, pointing out the aspect of electrical neuromonitoring and precaution of parathyroid glands, and survival rate of this extremely rare entity are discussed.

Retrospective evaluation of carcinoid tumors of the appendix in children.

Carcinoids of the appendix are rare in children and are usually diagnosed incidentally on histologic investigation following appendectomy for appendicitis. To investigate the significance of the diagnosis of appendiceal carcinoid in children, we conducted a retrospective study of the treatment and follow-up of 36 children with histologically confirmed carcinoid tumors of the appendix. Between 1970 and 2000 a total of 36 patients (25 girls, 11 boys) were diagnosed with appendiceal carcinoid. The median age of the patients at diagnosis was 12.3 years (range 6-16 years). The indication for appendectomy was acute lower right quadrant pain in 27 cases and chronic right lower quadrant pain in 9 patients. In 27 specimens the tumor was localized at the apex, in 7 at the midportion, and in 2 at the base of the appendix. The median tumor diameter was 6 mm (range 3-17 mm). Concomitant severe appendicitis was diagnosed in 14 patients 2 with a perforated appendicitis. In only one tumor were mucin-producing cells detectable. After a median follow-up of 10 years (range 2 months to 30 years) all patients were tumor-free. None of the patients had a synchronous or metachronous noncarcinoid malignant tumor. Appendiceal carcinoids are usually asymptomatic, and the indication for surgical intervention is acute or chronic abdominal pains in the right lower quadrant. For most patients the prognosis is excellent after appendectomy. As in adults, appendectomy is the appropriate treatment.