Idiopathic pulmonary haemosiderosis in a child with Down's syndrome: case report and review of the literature.

We report the case of a female child with Down's syndrome affected by idiopathic pulmonary haemosiderosis (IPH), who was successfully treated with hydroxychloroquine. First-line conventional treatment of IPH is traditionally based on systemic corticosteroids; however, many steroid-sparing agents are being increasingly used as adjuncts to corticosteroids in children with recurrent or refractory bleeding. The use of these drugs is particularly promising for maintenance treatment, because it tends to avoid the adverse effects of long-term corticosteroids.

Evolution of the surgical management of bowel atresia in newborn: laparoscopically assisted treatment.

PURPOSE: The jejunal-ileal atresias are the most common cause of intestinal occlusion in neonatal period and the duodenal atresia is the most common cause of high bowel obstruction in newborn. The treatment classically used is performed by a transverse sovraumbilical laparotomy. The first "minimal-invasive" approach was performed at the end of the years '70. Nowadays three clusters of thought are delineated about of mini-invasive treatment of small bowel atresias: the intestinal continuity can be established with a circumbilical incision, a totally laparoscopic technique or a laparoscopically assisted approach. It's presented our initial experience of bowel atresia in newborn treated with laparoscopically assisted approach. MATERIALS AND METHODS: Three newborns with prenatal diagnosis of suspected bowel obstruction and one with suspected duodenal atresia, confirmed the diagnosis after birth, underwent to laparoscopically-assisted surgery to correct these congenital anomalies. We have treated with this procedure a duodenal atresia, two jejunal atresias and an ileal form. Surgical management was consisted of an explorative laparoscopy that allowed to recognize and isolate the pathological portion. The second step was traditional open surgery: after the exteriorization of the pathological tract through the umbilical wound, we performed the intestinal anastomosis. RESULTS: In all cases the operation was completed successfully, no conversion was necessary. Intraoperatively no complications occurred. The post-operative course was uneventful. CONCLUSION: The video-assisted technique for the correction of small bowel atresia adds the advantages of the classic laparotomic procedure to the laparoscopic ones.

[Video-assisted treatment for biliary atresia]. / Trattamento videoassistito dell'atresia delle vie biliari.

BACKGROUND: The surgical treatment of biliary atresia is still a great challenge for pediatric surgeons. Kasai's operation usually needs a wide, painful, muscle-cutting laparotomies that quite often are followed by pain and peritoneal adhesion. These possible complications may disturb the post-operative course and humper liver transplantation. Advancements in minimally invasive surgery have allowed even the most complex procedures to be approached using these techniques. METHODS: The authors present a case of successful Roux-en-Y laparoscopic portoenterostomy for the treatment of biliary atresia. We report a case of a 3-month-old patient with biliary atresia who weighted Kg 5,300 at the operation. The patient was placed in supine position. The procedure was performed with 4 trocars of 3 mm and 1 of 10 mm. The umbilical site was used for extracorporeal Roux-en-Y enteroenterostomy. CO2 was insufflated at a pressure of 8 mmHg and a flow of 0.5 L/min. A drain was placed through the lower trocar site with the tip near the anastomosis. RESULTS: The procedure was free of neither intraoperative nor post-operative complications. Feeding by nasogastric tube was started after 2 days. Total oral feeding was possible after 8 days. CONCLUSION: Laparoscopic approach to perform Kasai's operation is technically feasible and thanks to a magnified vision, it allows to abtain a good visualization of the portal structures with an adequate retraction of the liver. This procedure can avoid or decrease the post-operative complications such as pain, breathing difficulty, adhesions and resulting in very small scars. Anyway laparoscopic Kasaiportoenterostomy should be done by a surgeon with a good experience in laparoscopic hand-suturing and neonatal experience and with the support of an experienced in neonatal and infantile videosurgery anaesthesiologist.

Pleuropulmonary blastoma in congenital cystic adenomatoid malformation: report of a case.

Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic lesion. The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made. Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.

[Urethro-vesical-vas deferens reflux as a cause of acute scrotum]. / Il reflusso uretro-vescicolo-deferenziale come causa di "scroto acuto".

The Authors reviewed 159 children who underwent surgical therapy for "acute scrotum", and they consider that the torsion of the appendix testis is the most common cause of the syndrome in children. On the other hand the orchi-epididymitis is very rare in paediatric age. About the incidence and etiopathogenesis of orchi-epididymitis is reported the revision of the literature; in particular is described the condition of the urethro-vesical-deferential reflux causing a chemical or infective orchi-epididymitis.

[The diagnosis of gastroesophageal reflux in childhood: a comparison between echography and pH measurement]. / Diagnosi di reflusso gastroesofageo nell'infanzia: confronto tra ecografia e pH-metria.

Gastroesophageal reflux (GER) is a very common event in childhood. Therefore we must use an exact and nontraumatizing test to differentiate between physiologic and pathologic conditions. Sonography appears to be a methodology endowed with these particularities. The aim of our study was to evaluate the diagnostic efficacy of Ultrasound (US) in comparison with a very precise test: the pH monitoring. We studied, by both methodologies, 76 children with suspected GER. Compared to pH-metry data, sonography showed a diagnostic sensitivity of 88,46% and a specificity of 58,33%. Therefore US appears to be an efficacious and innocuous test both in the screening and in the follow-up of patients with GER.

Benign mediastinal lipoblastoma in a 14-months-old infant.

Lipoblastomas are rare benign tumors arised from the fetal-embryonal fat that occur almost exclusively in children. About 70% of them arise in the limbs but several other sites have been reported. To date there are only 4 cases of mediastinal lipoblastoma in children, described in the international literature. The radiologic examination, including the chest X-ray, sonography and CT scan, can be used to detect the mass and its relationship to surrounding structures, and to define the typical features of the fat-like tissue. A case of a mediastinal lipoblastoma in a 14-months-old infant is reported, with a respiratory stridor and occasional dyspnoea, appearing only in the supine position. The radiologic findings, treatment and follow-up are also described.

Cystic mesenchymal hamartoma of the liver.

Two cases of multicystic mesenchymal hamartoma of the liver in children are described. In both cases noninvasive imaging techniques (ultrasound, CT or MR, radionuclide scan) showed a bulky cystic mass suggesting a correct preoperative diagnosis, while selective arteriography defined the operability of the tumor.

[Diagnosis of gastro-esophageal reflux in children. Ultrasonography versus pH monitoring]. / Diagnosi del reflusso gastro-esofageo nell'età pediatrica. Ecografia versus pH-metria.

The reliability of ultrasonography (US) was investigated in the diagnosis of gastroesophageal reflux in the pediatric patient. Therefore, specificity and sensitivity of US were compared with those of a 24-hour pH monitoring. A hundred and twenty-nine patients were examined; their age ranged 38 days to 14 years (56 were under and 73 over 2 years old). Since these patients presented with gastroesophageal symptoms, they underwent both pH monitoring and US. The results of the two methods were in agreement in 79.8% of the cases. Notably, in the age group ranging 0 to 2 years, US exhibited greater sensitivity (87% vs. 78.8%) and slightly lower specificity (76.5% vs. 81.1%) than in the age group ranging 2 to 14 years. The greater sensitivity in the 0-2 age group can be explained by physiological factors (which are typical of the first months of life) as well as by technical factors. Therefore, in infants, US should be combined with pH monitoring to identify the cases which are unquestionably pathological. The high specificity in the children over 2 years of age could justify the use of US alone in this group of patients for both diagnosis and follow-up, while pH monitoring might be used only in the most severe and/or complicated cases. The results suggest that, in the two age groups considered, abdominal US can be used not only as the diagnostic method of choice in the study of gastroesophageal reflux, but also as an extremely useful tool during follow-up when therapeutic monitoring is also needed.

Wilms' tumor involving the inferior vena cava: preoperative evaluation and management.

Neoplastic invasion of the inferior vena cava due to renal tumors (especially Wilms' tumor) is uncommon in children. The tumor thrombus, according to the aggressiveness of the original neoplasm, can extend in diverse ways, obliterate the vascular lumen completely, and even reach the right atrium. The luminal thrombus might be accompanied by the involvement of the caval wall, which requires wide vascular resection. The purpose of this paper is to present our experience with 7 children, aged 18 months and 6 years, affected by caval invasion due to Wilms' tumor. Furthermore, the diagnostic techniques and the surgical treatment in simple caval thrombosis and in associated invasion of the caval wall are described.

Fetus in fetu: report of an additional, well-developed case.

An 8-month-old boy presenting with a fast-growing abdominal mass was operated upon to remove the tumor, which was confirmed to be a well-formed fetus in fetu. The authors describe the clinical, radiologic, and surgical findings and review the literature.