Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6-10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described. OBJECTIVE: To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses. MATERIALS AND METHODS: We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997-2015 and analysed the clinical, biological and imaging data. RESULTS: We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children. CONCLUSION: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.

Intraluminal Duplication of the Terminal Ileum with Ectopic Gastric Mucosa in an Infant: A Rare Cause of Intussusception.

Enteric duplication cysts are rare malformations mostly diagnosed before the age of two, with varied clinical presentations. Ectopic gastrointestinal epithelium can be present, and management involves surgical resection. A three-month-old girl presented with rectal bleeding due to an ileocolic intussusception. Abdominal ultrasound revealed a target sign in the right upper part of the abdomen. At hydrostatic contrast enema, an incomplete reduction of the intussusception was obtained: only a trickle of contrast material entered the terminal ileum. An exploratory laparotomy ensued with manual reduction of the intussusception. At the end of the maneuver, a soft intraluminal mass was palpated within the ileocecal valve. Thus, an ileocecal resection was performed. At histology, an intraluminal enteric duplication cyst was documented, containing ectopic gastric mucosa. Secondary intussusception should be suspected even in infants in case of abnormal findings at hydrostatic contrast enema. Intraluminal enteric duplication cysts may be a rare cause of intussusception.

Hyperechogenic Bowel: Etiologies, Management, and Outcome according to Gestational Age at Diagnosis in 279 Consecutive Cases in a Single Center.

Introduction The aim of the study was to investigate perinatal outcome of fetuses with hyperechogenic bowel (HB) in relation to gestational age at diagnosis. Materials and Methods This is a retrospective observational study of fetal HB cases from 2002 to 2012. Patients were divided into three groups according to trimester at diagnosis. For each group, data from fetal ultrasound examination, fetal medicine investigations, intrapartum cares, and neonatal outcome were obtained. Results A diagnosis of HB was made in 279 fetuses among them 17 (6%) during the first trimester, 186 (67%) during the second trimester, and 75 (27%) during the third trimester. A significant prevalence of maternal comorbidities was noticed in group 1 (12%: p = 0.02). A chromosomal defect was identified in 13% of the fetuses without difference among the three groups. HB was associated with prenatal infection in 11.5% (n = 32) of the cases, with an equal distribution between groups 2 and 3. Intrauterine growth retardation was noticed in 23% (n = 64) of the cases with a slightly high prevalence in groups 1 (35%). HB was the only ultrasonographic intestinal soft marker in 80% (n = 223) of the fetuses, two-third of them were detected during the first and the second trimesters (p = 0.001). However, HB was associated with bowel dilation in 33% of the cases diagnosed during the third trimester (p = 001). Ultrasonographic extraintestinal anomalies were identified in 30% of the fetuses with a higher prevalence in group 1 (59%). HB resolved spontaneously in 55 (19.7%) cases-without difference among the three groups. In group 1 we recorded a significant prevalence of intrauterine demise (23.5%, p = 0.004). Two hundred twenty-seven (81.3%) pregnancies resulted in live-born neonates; among them gastrointestinal anomalies were noticed in 12.5% with a significant prevalence in group 3 (36%) compared with 6 and 5.4% in groups 1 and 2, respectively (p = 0.001). Extraintestinal anomalies were confirmed in 27% of the cases, whereas postnatal mortality rate was of 7% without differences between the three groups. Conclusion Detection of HB during the first trimester is associated with an increased risk for maternal comorbidities, intrauterine growth retardation, and adverse pregnancy outcome. Moreover, if HB is detected during the second trimester, it is associated with a favorable prognosis. Otherwise, HB detected during the third trimester is associated with a significant risk of gastrointestinal anomaly.

Vacuum assisted closure for the treatment of complex wounds and enterocutaneous fistulas in full term and premature neonates: a case report.

BACKGROUND: The Vacuum Assisted Closure (VAC) system has become an effective treatment for acute and chronic wound defects. Although its use has been reported in wound care of children and premature infants, the management of the device in this population has not been well established. CASE PRESENTATION: We report the satisfactory results in two neonates (one full-term and one preterm) with complex wounds secondary to major abdominal surgery. In the premature baby an enterocutaneous fistula was also present. Complete epithelialization of the wounds was achieved in both patients within a few weeks thus avoiding any further surgical procedure. CASE PRESENTATION: The use of VAC system in neonates is safe and effective in the management of complex wounds and should be considered as a first line treatment in the event of a major dehiscence.

Incidental Diagnosis of Giant Cell Tumor After Urachal Remnant Removal in a Thalassemic Child.

Urachal anomalies are relatively uncommon; they usually present in childhood as urachal cysts, urachal sinuses, diverticula, and patent urachus. The malignant involvement of the urachus, although rare in children, is commonly found in adulthood when adenocarcinoma accounts for the majority of the cases, representing up to 20-30% of all adenocarcinomas of the bladder. Urachal tumors of variable histology have been reported in children and adolescents as single case report. We present the first case of a 4-year-old thalassemic boy, with an incidental finding of giant cell tumor arising in an urachal remnant.

Gastrostomy Placement in Children: Percutaneous Endoscopic Gastrostomy or Laparoscopic Gastrostomy?

The aim of this study is to compare the outcomes and the complications between the 2 most adopted procedures for gastrostomy placement: percutaneous endoscopic gastrostomy (PEG) and laparoscopic gastrostomy (LG) in children. We present our study on 69 patients (male: 46/female: 23): group 1 (37 patients, 54%) undergoing PEG, group 2 (32 patients, 46%) undergoing LG. A total of 5 major complications were observed all in the PEG group (13.5%), no major complication was observed in the LG group (P-value<0.05). A total of 12 minor complications were observed: 4 occurred in the PEG group (10.8%) and 8 (25%) in the laparoscopic gastrostmoy group, not statistically relevant. We suggest that the LG should be considered the preferred technique for gastrostomy placement in pediatric patients, particularly in newborns, children with significant skeletal malformations, and patients who underwent previous abdominal surgery.

Pitfalls in the surgical management of undifferentiated sarcoma of the liver and benefits of preoperative chemotherapy.

AIM: Undifferentiated sarcoma of the liver (USL) is the third commonest malignant liver tumor in children. The aim of our study is to evaluate the outcome of this rare entity according to the quality of the surgical resection and the compliance to the European pediatric soft tissue sarcoma group guidelines. PATIENTS AND METHODS: We conducted a monocentric review of patients referred to our department with a definitive pathologic diagnosis of USL between 1997 and 2013. We looked at the diagnosis and management pitfalls, surgical technique, and outcomes. Results are expressed as median (range). RESULTS: There were 13 patients (M/F=7:6=1.1). Age at presentation was 8 years (range, 11 months-16 years). Of the 13 patients, 10 patients (69%) presented with abdominal pain, 5 (38%) with tumoral bleeding, and 2 (15%) with peritoneal rupture. All lesions were unique, nonmetastatic, and heterogeneous with cystic components measuring 14 (6-19) cm. Six (46%) patients had an upfront surgery: five because of wrong clinical diagnosis (three query mesenchymal hamartoma, one spontaneous peritoneal bleeding, and one cystic lymphangioma), and one because of rapid enlargement of the mass. Seven (54%) patients (including one with tumoral bleeding) received neoadjuvant chemotherapy, and had their tumor diameter decreasing by 40% (range, 0-60%). Final surgery consisted of seven right hepatectomies; one right extended hepatectomy; three mesohepatectomies; two left hepatectomies. There were three incomplete resection in the upfront surgery group versus none in the neoadjuvant chemotherapy group. The degree of tumor necrosis after chemotherapy ranged from 95 to 100%. Surgical complications included the following: liver transplantation (LT) following a Budd-Chiari syndrome after a mesohepatectomy, one biliary ducts injury treated by Roux-en-Y loop. All patients received the postoperative chemotherapy according to the European protocol. One of seven patients (14%) with neoadjuvant chemotherapy underwent radiotherapy for rupture at diagnosis versus three of six patients (50%) with upfront surgery: one for rupture at diagnosis and two for rupture during upfront surgery. One patient (17%) with upfront surgery had local recurrence at 2 years after initial surgery, and is in second complete remission 1 year after a redo surgery. All patients are alive at a median 34 months (range, 5-134) follow-up. CONCLUSION: USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.

Um estudo sobre a representação parental de casais homoafetivos masculinos / A study on the parental representation of homoaffective male couples / Un estudio sobre la representacion parental de parejas homoafetivas masculinas

Da família tradicional-patriarcal à família contemporânea, mudanças relevantes ocorreram, gerando uma pluralidade de modelos. O papel fundamental da família na constituição psíquica do indivíduo, frente ao crescente aparecimento de novos arranjos familiares, torna essencial construir conhecimento acerca das novas formas de se relacionar e vivenciar a parentalidade. Este trabalho objetiva apresentar os resultados de uma pesquisa que investigou as representações parentais de casais homossexuais masculinos, por meio das imagos parentais e legados geracionais. Com objetivo de compreender como se dão tais representações, bem como, suas possíveis ligações e/ou influências na relação do casal homoafetivo e, possivelmente, na família homoparental, foram entrevistados cinco casais homossexuais masculinos sem filhos e aplicado o Desenhos de Família com Estórias (DF-E) adaptado. O método utilizado foi o clínico-qualitativo e a análise feita com base no referencial psicanalítico vincular. Os resultados apontaram casais ainda muito ligados à suas famílias de origem; com questões conflitivas associadas ao processo de construção de suas identidades homossexuais; presos ao modelo de conjugalidade heteronormativa; cujos relacionamentos caracterizaram-se pela brevidade com que se tornaram compromissados, com a finalidade principal de apoio mútuo à assunção da identidade homossexual. Concluiu-se que o despreparo para deixar o lugar de filho é fator influente para não se pensar acerca da parentalidade; e está relacionado aos conflitos de cerne familiar, especialmente à falta de aceitação. Enfatizamos a necessidade de maior número de pesquisas na área, visando uma abordagem científica que propicie o estabelecimento de uma ética pautada no vínculo filiativo.

From the traditional patriarchal family, relevant changes have occurred, generating a plurality of models. The fundamental role of family in psychic constitution, ahead of the growing emergence of new family arrangements, it is considered essential the study of the new functions and forms of relating in these groups. Aiming to comprehend how these representations and its possible relations and/or influences in the homoaffective couple relation and, possibly, in the homoparental family, five homosexual couples without children have been interviewed in their own houses. The method was the clinical-qualitative and the analyses were made based on the psychoanalytical referential. The results point couples still very linked to their families of origin; with conflictive issues associated to the process of construction of their homosexual identities; stuck to the model of heterosexual couples, whose relationships are characterized for the brevity of engagement, with the main goal to be the mutual support to the homosexual identity assumption of each of them. This way, we conclude that the unpreparedness to leave the place of "son" is an influent factor to not think about parenthood; and both are related to conflicts centered in the family, specially the lack of acceptance of homosexuality. Emphasizing the need of a greater number of researches in the area of homoparenthood, aiming a scientific approach of it, and providing the establishment of an ethic punctuated in the bond.

Desde la familia tradicional-patriarcal hasta la familia contemporanea, mudanzas relevantes ocurrieron, generando una pluralidade de modelos. El papel fundamental de la familia en la constituicion psíquica del individuo, frente al creciente aparecimiento de nuevos arreglos familiares, se considera fundamental el estudio de las nuevas funciones y formas de se relacionar en esos grupos. Con objetivo de comprender como ocurren esas representaciones y sus posibles relaciones y/o influencias en la relación de la pareja homoafectiva y, posiblemente, en la familia homoparental, fueron entrevistados cinco parejas homosexuales masculinas sin hijos, y aplicado el Dibujos de Familia con Historias (DF-E) adaptado. El metodo fue el clinico-cualitativo y los analisis hechos com base em el referencial psicoanalitico vincular. Los resultados apuntan a parejas todavía muy ligados a sus familias de origen; con cuestiones conflictivas asociadas al proceso de construcción de sus identidades homosexuales; atados al modelo de conjugabilidad heteronormativa; cuyos relacionamientos se caracterizan por la brevedad con que se tornaron comprometidos, con la finalidad principal de apoyo mutuo a la aceptación de la identidad homosexual. Se concluye que el despreparo para dejar su sitio de "hijo" es factor influyente para no se pensar en la parentalidad; que está relacionado con conflictos de cerne familiar, especialmente la falta de aceptación de la homosexualidad. Enfatizamos la necesidad de un mayor numero de investigaciones en el área de la homoparentalidad, apuntando un abordage cientifico de este y así propiciar el establecimiento de una ética puntuada en el vinculo filiativo.