Abdominal asymmetry in a 17 year old girl.

The pseudopapillary pancreatic solid tumor (TPSP) is a rare malignancy typical of young adult women (only 12 pediatric cases from 2000 to 2009), it can recur and metastasize. The prognosis is usually good after radical surgical removal. We emphasize the importance of TPSP in differential diagnosis of retrogastric, peripancreatic masses especially in puberal females. We describe the case of an adolescent girl with an abdominal mass revealed as a rare pancreatic neoplasia.

Medium-term outcome after stented and un-stented distal urethroplasty: A retrospective analysis on redo-urethroplasty need and cosmetic results. / Resultados a medio plazo de la uretroplastia distal con colocación de sonda y sin ella. Análisis retrospectivo sobre la necesidad de reintervención y los resultados estéticos.

INTRODUCTION: Long-term complication rates after distal hypospadias repair can be close to 20%. There are no available guidelines regarding the need of a catheter in distal urethroplasty. We report a retrospective analysis on medium-term redo-urethroplasty rate and cosmetic results after a two-year pilot study on stented/un-stented distal urethroplasties. MATERIALS AND METHODS: A total of 11 stented (Group A) and 17 un-stented (Group B) Snodgrass-procedures were performed by one pediatric surgeon at our Institution (2011-2013). The median age at surgery was 2.1 years (range 1-8.5). Inclusion criteria were primitive distal defect, same surgeon in both interventions, catheter-free discharge. The median follow-up was 6.4 years (range 1.5-8.1). All patients received at least one post-operative clinical-cosmetic examination (HOSE). The aim of our study was to compare medium-term complications and redo-urethroplasty rates before starting a randomized study. A retrospective analysis was performed. We used Fisher's exact-test (P<0.05) for statistical analysis. RESULTS: Of 28 complications, 5 required redo-surgery: 2/11 stented-cases, 3/17 un-stented. Cosmetic results were satisfactory in both groups. These results were not statistically significant (P=1.000). CONCLUSION: Long-term follow-up is mandatory to know redo-urethroplasty rate and cosmetic outcome after distal stented/un-stented repair. Further studies are needed to evaluate the role of catheter placement and the definitive outcome in distal urethroplasty.

Medium-term outcome after stented and un-stented distal urethroplasty: A retrospective analysis on redo-urethroplasty need and cosmetic results.

INTRODUCTION: Long-term complication rates after distal hypospadias repair can be close to 20%. There are no available guidelines regarding the need for a catheter in distal urethroplasty. We report a retrospective analysis on medium-term redo-urethroplasty rate and cosmetic results after a two-years pilot study on stented/un-stented distal urethroplasties. MATERIALS AND METHODS: A total of 11 stented (Group A) and 17 un-stented (Group B) Snodgrass-procedures were performed by the same pediatric surgeon at our Institution (2011-2013). The median age at surgery was 2.1 years (range 1-8.5). Inclusion criteria were primitive distal defect, same surgeon in both interventions, catheter-free discharge. The median follow-up was 6.4 years (range 1.5-8.1). All patients received at least one post-operative clinical-cosmetic examination (HOSE). The aim of our study was to compare medium-term complications and redo-urethroplasty rates before starting a randomized study. A retrospective analysis was performed. We used Fisher's exact-test (P < 0.05) for statistical analysis. RESULTS: Of 28 complications, 5 required redo-surgery: 2/11 stented-cases, 3/17 un-stented. Cosmetic results were satisfactory in both groups. These results were not statistically significant (P = 1.000). CONCLUSION: Long-term follow-up is mandatory to know redo-urethroplasty rate and cosmetic outcome after distal stented/un-stented repair. Further studies are needed to evaluate the role of catheter placement and the definitive outcome in distal urethroplasty.

Lymphangiomas of the head and neck in children.

Lymphangiomas are rare benign congenital tumours, involving both the head and the neck and causing obstructing symptoms in the upper airways as well as aesthetic anomalies. In recent years, sclerosing therapy with OK-432 has become the treatment of choice in the management of these lymphatic malformations. Nonetheless, surgery still seems to be the therapy advocated for resolution of symptoms. Herein, three cases of lymphangiomas involving the head and the neck are described and a review of the English scientific literature is outlined.

Laparoscopic versus Open Surgery in Complicated Appendicitis in Children Less Than 5 Years Old: A Six-Year Single-Centre Experience.

Introduction. Acute appendicitis is the most common surgical emergency in the pediatric population. The peak incidence occurs in the first decade of life, while it is uncommon to face appendicitis in children younger than 5 years of age. Laparoscopy is now demonstrated to be the optimal approach also to treat complicated appendicitis, but in very young children this standardized operation is not always easy to perform. Material and Methods. From January 2009 to December 2015 we operated on 525 acute appendicitis, with 120 patients less than 5 years of age. Results. 90 children had a complicated appendicitis (localized or diffuse peritonitis): 43 (48%) were operated on by open approach and 47 (52%) by laparoscopy. The overall incidence of postoperative complications was greater in the open appendectomy group (63% versus 26%) and all severe complications requiring reintervention (6% of cases: 3 postoperative abscesses resolved with ultrasound guided percutaneous abscess drainage; 1 tubal surgery for salpingitis; 1 adhesion-related ileus requiring relaparotomy) were mostly associated with open surgery. Conclusions. Laparoscopic surgery resulted as the best approach for treating complicated appendicitis also in younger children, with minor and less severe postoperative complications compared to open surgery.

Massive Retro-Pneumoperitoneum and Lower Limb Subcutaneous Emphysema After Pediatric Heart Transplantation: A Case Report.

An emphysema in a lower limb is usually a clinical sign of a severe and life-threatening infection. We report a rare case of subcutaneous emphysema of the left lower limb associated with a massive retro-pneumoperitoneum and pneumatosis intestinalis after cardiac transplantation in a 4-year-old girl. The child was nearly asymptomatic beside an abdominal distension. A benign pneumoperitoneum associated with an extensive pneumatosis intestinalis is a rare complication after organ transplantation and should be treated conservatively. The association with an emphysema in a lower limb in a child has not been previously reported to our knowledge in the literature.

A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Ventriculopleural shunt: thoracoscopic placement of the distal catheter.

Ventriculopleural shunting is usually reserved for patients with limited options for shunt revisions. We report the case of a 16-year-old boy with posthemorrhagic hydrocephalus who required numerous shunt procedures. At the age of 6 years, a ventriculopleural shunt was inserted by an intercostal thoracotomy, and 4 years later replacement of the distal catheter was necessary. Recently, he presented again with a shunt malfunction due to migration of the pleural catheter. We describe a technique for performing the placement of the distal catheter under direct thoracoscopic vision by a peel-off needle into the unscarred thoracic cavity despite two previous pleural procedures. The postoperative course was uneventful. Thoracoscopic assistance in ventriculopleural shunt placement appears to be a safe and effective technique, offering several advantages over the open procedure: it is less invasive, allows a precise positioning of the thoracic catheter under visual control, and confirms appropriate function.

Metastatic retroperitoneal paraganglioma in a 16-year-old girl. Case report, molecular pathological and cytogenetic findings.

Retroperitoneal paraganglioma is a rare tumor, especially occurring in childhood and adolescence, with a marked tendency to become biologically malignant. It has not been possible to predict the clinical outcome of paraganglioma patients by conventional histology, hence malignancy can only be demonstrated by the occurrence of metastatic lesions. Currently, only limited information on the genetics of this tumor is available. We report on a 16-year-old girl with a large retroperitoneal paraganglioma and an osseous metastasis to the first lumbar vertebra. In addition to morphological and immunohistochemical examinations, a molecular cytogenetic analysis was performed. Comparative genomic hybridization (CGH) revealed imbalanced chromosomal aberrations with a loss of chromosome 1p and a gain of 1q, indicating isochromosome 1q. A loss of chromosome 3 as well as low-level gains of chromosomes 4, 5, 6q, 11q and 13q were detected. A PCR-based microsatellite analysis of 1p confirmed the loss of heterozygosity, including NB1 and NB2 putative tumor-suppressor gene regions. Telomerase activity, which is found in the majority of malignant tumors, could not be detected. The case presented here is the first more comprehensive molecular genetic analysis of a sporadic malignant paraganglioma.

A case of severe cerebral trauma in a patient under chronic treatment with cyclosporine A.

A case of severe cerebral head injury in a child, chronically treated with cyclosporine A after orthotopic liver transplantation, is presented. The initial Glasgow Coma Scale score after the motor vehicle accident was 3, and computed tomography showed multiple sites of intracerebral bleeding, an epidural hematoma, and signs of perifocal edema. Although these lesions are normally associated with a poor outcome, the child recovered unexpectedly well. In brain injury, a lucid interval can be followed by secondary neurologic deterioration due to a loss of high-energy metabolites, a release of neurotransmitters, and an increase in intracellular Ca2+. These events finally led to cell damage in the penumbra of an ischemic infarction. Among other drugs, immunosuppressants such as cyclosporine A have been shown to exhibit neuroprotective properties in experimental models if given during this time interval of secondary neurologic deterioration. Although human data on these effects are still lacking, we conclude that neuroprotective actions of cyclosporine A may have been involved in the favorable outcome in this 14-year-old boy.

Technical aspects of laparoscopic splenectomy in children.

Different authors reported about their experiences in laparoscopic splenectomy. The preparation of vessels and ligaments are feasable without major problems. Nevertheless the handling of the organ during the operation and during splenic extraction can be very difficult, especially in larger organs. The authors report experience with the technique of laparoscopic splenectomy and describe a new technical aspect in handling the spleen during this procedure. A loop of an umbilical band is used for retracting the organ during the preparation and placing the spleen into a sterile bag for the removal of the organ. This device helps prevent damages to the capsule caused by forceps. Large organs can be placed into the pelvis, and the bag is placed and opened in the upper abdomen. The authors used this technique successfully in seven patients. The results are compared with previous experiences.

Primary non-Hodgkin lymphoma of the stomach--role of surgery.

Thirty-one patients with Non-Hodgkin lymphoma of the stomach were retrospectively investigated. The prognostic factors regarding survival after treatment were determined in an univariate analysis. Twenty-seven of the patients revealed a stage I or II disease (according to the Ann Arbor classification) at the time of operation. The analysis showed no significance for grading, staging, lymph node-, serosa- or splenic involvement of our patients. Significant prognostic factors for survival of gastric Non-Hodgkin lymphomas were age (< 60 years) and resection margins R0 or R1/R2 (p < 0.05). The post-treatment evaluations for prognostic differentation between radical resections, patients with complete response after palliative resection including additional chemotherapy and patients without therapy response showed a significant difference in survival (p < 0.01).

Congenital "transhaesio intestini tenuis supragastrica" in a 14-year-old girl - a rare case of internal hernia.

Intestinal obstruction caused by internal hernia is a rare condition. A 14-year-old girl who suffered from acute abdominal pain two days after appendectomy is presented. Abdominal sonography and plain abdominal x-ray showed dilated small bowel loops and air-fluid levels indicating mechanical intestinal obstruction. Exploratory laparotomy revealed small intestine loops herniated through the lesser omentum. The anatomical aspects are reviewed and discussed.

[Endoscopic hernia repair]. / Endoskopischer Hernienverschluss.

A laparoscopic procedure for surgical hernia repair is reported. In comparison to other methods we do not only remove the peritoneal sac, but close although the inguinal canal with a nonresorbable marlex mesh. Up to now we practiced this technique in 35 patients. First post-operative results are encouraging but nothing can be said about longterm results, especially the recurrence rate.

Circumcision in paediatric surgery using an ultrasound dissection scalpel.

In contrast to electrocautery, the ultrasound dissection scalpel allows tissue dissection and haemostasis to be performed without the danger of thermal tissue damage or burning caused by uncontrolled active current. In a clinical application study performed on 24 boys with an average age of six years, we examined whether the use of this instrument is also practical in a routine operation such as circumcision. It was possible to perform all operations with the ultrasound dissection scalpel (Ultracision, manufactured by Ethicon) by the classic technique without the additional use of electrocautery for haemostasis. Although dissecting speed is slower than with conventional instruments, the fact that simultaneous haemostasis is possible meant that operations could be carried out quickly and with minimal bleeding. There were no perioperative complications such as haemorrhaging or burning. All children were examined postoperatively over an average of 3 weeks (range 1-8 weeks). Wound healing was completely without complications for 22 patients (92%). One patient (4%) suffered a candidal infection in the first postoperative week. One further patient (4%), who was already suffering preoperatively from an extended chronic inflammation of the prepuce, developed pronounced swelling postoperatively. Heavy scarring occurred subsequently in both cases. Our results show that the advantages of the ultrasound dissection scalpel, e.g. gentle tissue dissection with simultaneous haemostasis, can also be used to advantage for a technically simple operation such as circumcision without having to fear the risks of electrocautery.

The scope of minimally invasive pediatric surgery (MIPS) and its influence on reduced length of hospitalization.

Minimally invasive pediatric surgery (MIPS) is a new dimension in pediatric surgery. The scope of methods replacing large operations in children by smaller endo- or laparoscopic interventions is presented.Electromagnetic double-segmental elongation has achieved auto anastomosis in 16 out of 22 children with long gap esophageal atresias since 1975. A new radial laser-applicator with entirely lateral radiation has led to a method for endoscopic occlusion of esophagotracheal and other fistulas with a 100% success rate in a minipig-model. Our experience with 54 laparoscopic interventions including large bowel resections, hiatus hernia, and a new technique for pyloromyotomy documents that laparoscopy halves hospitalisation-periods and may well become the most rapidly growing branch of pediatric surgery.

Rectal atresia, choanal atresia and congenital heart disease: a rare association.

Rectal atresia is an extremely rare anorectal malformation. A unique case of rectal atresia presenting as an imperforate membrane associated with congenital cardiac malformations and unilateral choanal atresia in a newborn is reported. Ultrasound examination in the newborn infant located the presence of the rectal membrane which was surgically treated successfully.

Laparoscopic and thoracoscopic surgery in infancy and childhood, the Münster/Gent experience.

Laparoscopic and thoracoscopic surgery have had a tremendous impact on adult surgery, but are still rarely used in children. In the past 3 years 168 children have been treated endoscopically in the three cooperating institutions. The course of all patients was documented prospectively in order to determine the value and prove the safety of endoscopic surgery in children. The operations performed endoscopically comprised appendectomy (n = 39), diagnostic procedures (24), adhesiolysis (n = 22), cholecystectomy (n = 13), bowel resection (n = 8), pyloromyotomy (n = 6), ovarial resection (n = 6), hiatus hernia repair (n = 22), splenectomy (n = 6), first stage of Fowler-Stephens operation for kryptorchidism (n = 3) and interruption of persistent botallic ducts (n = 6) in preterm infants. Apart from four wound infections (2.4%) following perforated appendicitis, one of which required laparotomy for perityphlitic abscess there were no serious complications. No patient died, and overall morbidity related to the procedure was 1.8% (i.e. incidence of uneventful minor intraoperative complications). Furthermore endoscopic surgery provides a better diagnostic survey of the abdomen or thorax so that missing of secondary pathology is unlikely. Pain and reflectory impairment of bowel-function or respiration were diminished, the aesthetic results were excellent and hospitalization could be reduced to a minimum. It is concluded that laparoscopic and thoracoscopic surgery are safe and reliable procedures for children and yield very encouraging results.

[Laparoscopic staging in abdominal tumors in children: an alternative to staging laparotomy]. / Laparoscopia diagnóstica en tumores abdominales infantiles: una alternativa a la laparotomía.

INTRODUCTION: In abdominal tumors in childhood, staging laparotomy is usually an essential diagnostic tool to obtain information about the histology and the extension of the tumor, when less invasive methods do not reach to clarify the process. PATIENTS: In fourteen children between 3 months and 17 years a laparoscopical procedure was performed. MATERIAL AND METHOD: Only in patients without a clear diagnosis after noninvasive exploration were submitted to an explorative laparoscopy in order to define the histology, the extension of the illness or to locate it. The laparoscopical instrumentarium used was from Dufner, adapted to children. RESULTS: The patients tolerated well this procedure, the posoperative period was short (mean 2.07 days), no complications after the operation are reported. Only one case of hemorrhage lead to a conversion after biopsy of a neuroblastoma. In all cases the diagnosis and staging was achieved. CONCLUSIONS: When non invasive diagnostic methods are not conclusive an explorative laparoscopy should be performed because it offers a lot of advantages over a laparotomy.

[Congenital sternal cleft. Diagnosis and treatment]. / Esternón bífido congénito. Diagnóstico y tratamiento.

The first known case of sternal cleft was described by Torres in 1740. Since then, many publications have appeared concerning sternal cleft, which have led to introduce a classification and some therapeutical procedures. This disease is a developmental anomaly of the sternum that usually shows vascular and cardiac malformations as well as diaphragmatic, abdominal wall, pericard and middle line organs anomalies. Two cases of sternal cleft are presented who have been treated in the neonatal period with different associated anomalies. Diagnostic procedures and therapeutical aspects are discussed.