Long-term follow-up after esophageal replacement in children: 45-Year single-center experience.

PURPOSE: We evaluated the results of esophagocoloplasty (ECP) in children by performing a 45-year retrospective cohort study in a single center. MATERIALS AND METHODS: We collected all of the medical charts of patients who underwent ECP at our hospital from January 1969 to January 2014. We reviewed the medical history for each patient and then contacted the patients by phone to obtain responses to a questionnaire. The questionnaire evaluated the following four areas: overall general state, gastrointestinal function, dependence on the Health Sanitary System, and their aesthetic satisfaction. RESULTS: We contacted 96 patients, and 72 completed our questionnaire. There were 45 males and 27 female respondents, with a mean age of 34.5 years. The mean Karnofsky performance status index was 96.4%. There were excellent scores obtained from 55 patients and fairly good scores from 16 patients. There was 1 patient with a low score, and there were no scores reported below 70%. Most patients (58/72) reported regular bowel habits, and no patients required drugs or other measures to facilitate defecation. Gastroesophageal reflux was present in 51 patients, and 62.8% have bothersome symptoms. Twenty-five patients used pharmacological therapies. Forty-eight patients (66.6%) were satisfied with the aesthetic result of surgery (mean score in a scale from 0 to 5, is 3). CONCLUSIONS: ECP can be used in children who require esophageal substitution. The resulting long-term QoL is acceptable. However, the aesthetic outcomes remain a problem.

Trajectories of internalizing, externalizing, and grades for children who have and have not experienced their parents' divorce or separation.

This study examined whether the occurrence and timing of parental separation or divorce was related to trajectories of academic grades and mother- and teacher-reported internalizing and externalizing problems. The authors used hierarchical linear models to estimate trajectories for children who did and did not experience their parents' divorce or separation in kindergarten through 10th grade (N = 194). A novel approach to analyzing the timing of divorce/separation was adopted, and trajectories were estimated from 1 year prior to the divorce/separation to 3 years after the event. Results suggest that early parental divorce/separation is more negatively related to trajectories of internalizing and externalizing problems than is later divorce/separation, whereas later divorce/separation is more negatively related to grades. One implication of these findings is that children may benefit most from interventions focused on preventing internalizing and externalizing problems, whereas adolescents may benefit most from interventions focused on promoting academic achievement.

Parent academic involvement as related to school behavior, achievement, and aspirations: demographic variations across adolescence.

A longitudinal model of parent academic involvement, behavioral problems, achievement, and aspirations was examined for 463 adolescents, followed from 7th (approximately 12 years old) through 11th (approximately 16 years old) grades. Parent academic involvement in 7th grade was negatively related to 8th-grade behavioral problems and positively related to 11th-grade aspirations. There were variations across parental education levels and ethnicity: Among the higher parental education group, parent academic involvement was related to fewer behavioral problems, which were related to achievement and then aspirations. For the lower parental education group, parent academic involvement was related to aspirations but not to behavior or achievement. Parent academic involvement was positively related to achievement for African Americans but not for European Americans. Parent academic involvement may be interpreted differently and serve different purposes across sociodemographic backgrounds.

Divorce and Child Behavior Problems: Applying Latent Change Score Models to Life Event Data.

Effects of parents' divorce on children's adjustment have been studied extensively. This article applies new advances in trajectory modeling to the problem of disentangling the effects of divorce on children's adjustment from related factors such as the child's age at the time of divorce and the child's gender. Latent change score models were used to examine trajectories of externalizing behavior problems in relation to children's experience of their parents' divorce. Participants included 356 boys and girls whose biological parents were married at kindergarten entry. The children were assessed annually through Grade 9. Mothers reported whether they had divorced or separated in each 12-month period, and teachers reported children's externalizing behavior problems each year. Girls' externalizing behavior problem trajectories were not affected by experiencing their parents' divorce, regardless of the timing of the divorce. In contrast, boys who were in elementary school when their parents divorced showed an increase in externalizing behavior problems in the year of the divorce. This increase persisted in the years following the divorce. Boys who were in middle school when their parents divorced showed an increase in externalizing behavior problems in the year of the divorce followed by a decrease to below baseline levels in the year after the divorce. This decrease persisted in the following years.

Multiple intestinal atresia with apple peel syndrome: successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent.

The association of apple peel bowel with multiple intestinal atresias is a rare event. The authors report a case of a 2.200-g newborn affected by this uncommon condition. The interruptions of small bowel continuity were treated successfully with 5 end-to-end anastomoses and with the construction of 2 ostomies. A gastroduodenal silicone tube (Vygon S.A., Ecouen, France) was used as an intraluminal stent.

Contemporary developmental theory and adolescence: developmental systems and applied developmental science.

PURPOSE: To discuss developmental systems models of human development and explain how they offer a productive frame for research, policies, and programs aimed at understanding adolescents' development and enhancing their health and positive development. Contemporary developmental theory stresses that the multiple levels of organization involved in human life (ranging from biology through culture, the natural and designed ecology, and history) are systemically integrated across ontogeny. Relations within this developmental system are the focus of developmental analysis and application. METHODS: The key features of developmental systems theories are reviewed, and their use for framing scholarship about and applications for improving adolescent development are assessed. RESULTS: We demonstrate the potential of contemporary developmental theory for understanding the character and dynamics of adolescent development and for using this knowledge for the design of effective policies and programs that promote positive youth development. CONCLUSIONS: An adequate and sufficient science of adolescent development, and one that is able to help in the development of successful policies and programs for youth, must integratively study the relations between individuals and contexts in an integrated, systemic, and temporal manner.

[Malignant primary tumors of the liver in childhood]. / I tumori maligni primitivi del fegato in età pediatrica. Esperienza di un singolo centro.

Twenty-one children (16 males, 5 females) with malignant primary hepatic tumors were admitted to the Pediatric Clinic of the University of Bologna between June 1973 and July 2001. The diagnosis was hepatoblastoma (HBL) in 16 cases; hepatocellular carcinoma (HCA) in 3 cases; undifferentiated sarcoma in 1, malignant rhabdoid tumour of the liver in 1. Median age at diagnosis was 1.8 year (1 mounth-13 years). As to intrahepatic tumor's extension, patients were classified in groups (from I to IV) according to International Society of Pediatric Oncology staging. 2 patients were ascribed to group I; 9 to group II; 9 to group III and I to group IV. At diagnosis 3 pts presented lung metastases. Seventeen patients (81%) were treated with surgery, in 11 cases as first approach to the tumor. In 10 patients, initially with unresesectable tumor, chemotherapy was started first. Drugs used were mostly Cisplatinum or Carboplatinum with Doxorubicin. Sussequently 6 patients were submitted to surgery. At a median follow up of 12.5 years, 52.3% of patients is alive without disease. This percentage rises to 58% taking into consideration only HBL and HCA cases (alive 11/19). We conclude that excluding metastases at diagnosis (3 deaths), the main prognostic factor is resectability and radical surgery: in our experience 4 patients with unresectable tumor died, as 2 patients with microscopical residual after surgery.

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.

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.

Laparoscopic surgery of diaphragmatic diseases in children: our experience with five cases.

AIMS: In paediatric surgery the laparoscopic approach can be used to repair diaphragmatic anomalies originating from the abdomen or containing abdominal viscera. Candidates for laparoscopic correction are children with mild symptoms and good respiratory and haemodynamic conditions. The authors present their experience with 5 patients treated successfully for different types of diaphragmatic lesions. PATIENTS AND METHODS: Five children were treated laparoscopically since 1998. Two true Morgagni-Larrey hernias, one recurrent left Bochdalek hernia, one diaphragmatic dysontogenetic cyst and one huge congenital sliding and rolling hiatal hernia. All the herniated viscera were repositioned in the abdomen and the defects--including the diaphragmatic hole at the level of the dysontogenetic cyst--were directly sutured without the use of a mesh. RESULTS: All patients are healthy without signs of recurrence observed at chest X-ray after a follow-up of 3 months to 1 year. DISCUSSION: Under specific conditions the laparoscopic approach can be an effective and more advantageous alternative to laparotomy for diaphragmatic congenital diseases in a paediatric population.

Intestinal bypass of the oesophagus: 117 patients in 28 years.

This study includes 117 patients operated upon in the period from 1970 to 1999. Indications, surgical techniques, complications, and results are reviewed. Indications included: long-gap oesophageal atresia with or without fistula in 81 patients; peptic stenosis in 19; caustic stenosis in 12; oesophageal varices in 2; and 1 case each of oesophageal epidermolysis bullosa, total oesophageal leiomyomatosis, and a non-functioning antiperistaltic retrosternal colic graft operated upon in another hospital. A retrosternal bypass was performed 106 times: 98 first operations and 8 redos; the intrathoracic technique was used 19 times. The left transverse colon was used in 107 cases (85.6%), the right transverse colon in 8 (6.4%), and the ileocecum in 10 (8%). All the intestinal bypasses were placed in the isoperistaltic direction. There were 5 deaths in the first 11 years of our experience; no patient died from 1982 on. Ten complications were treated conservatively (8%): 2 wound infections healed with medical treatment, and 8 leaks of the cervical anastomosis closed spontaneously. The major surgical complications were 8 gangrenous bypasses (6.4%), removed and reoperated about 1 year later utilizing an ileocolic retrosternal graft. Three cases of peptic disease of the colic bypass (2.4%) were successfully treated with the author's technique. Nine patients had minor surgical complications (7.2%): 3 strictures of the oesophagocolic anastomosis in a retrosternal bypass (resected and reoperated) and 6 cases of adhesive occlusion. In our opinion, the best substitute of the oesophagus is the colon, particularly the left transverse segment, which may be placed behind the sternum or in the oesophageal bed, always in the isoperistaltic direction. The low mortality (4%), restricted to the early period of our experience, and few major surgical complications (6.4%) are acceptable considering the importance of the operation, and the long-term results may be considered very satisfactory.

Laparoscopic repair of Morgagni-Larrey hernia in a child.

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.

Recurrent posttraumatic urethrovaginal fistula: a new application for ASTRA.

The authors describe the results of an application of the surgical technique called ASTRA (anterior sagittal transrectal approach) in the case of a 16-year-old girl with a posttraumatic urethrovaginal fistula. The young girl with a posttraumatic urethrovaginal fistula had recurrence after 4 surgical attempts at closure performed by the vaginal approach before it was definitively corrected with the ASTRA. Two years after the ASTRA operation the patient is well with a complete restitution of function confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. The successful recovery confirmed that ASTRA may be an excellent method of treating the developmental or acquired anomalies of the perineal region.

Recurrent posttraumatic urethro-vaginal fistula: a new application for ASTRA.

The authors describe the results of an application of the surgical technique called ASTRA (anterior sagittal transrectal approach) in a 16-year-old girl with recurrent urethro-vaginal fistula. The young girl had a posttraumatic urethro-vaginal fistula. It recurred after 4 operations by a direct vaginal approach before definitive correction with the ASTRA. Three years after the operation the patient has remained well with complete healing and no fistula recurrence confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. This report suggests that ASTRA is a useful method of treating acquired or developmental anomalies of the perineal region.

Anterior sagittal transanorectal approach to the posterior urethra in the pediatric age group.

PURPOSE: Surgical access to the posterior urethra is often difficult and several surgical solutions have been proposed. We suggest an anterior sagittal transanorectal approach based on splitting the anterior rectal wall only. This alternative technique provides excellent exposure to the retrourethral region, permitting simple and safe surgery. MATERIALS AND METHODS: Between 1994 and 1996 we performed surgery via the anterior sagittal transanorectal approach in 8 patients with a mean age of 9.06 years. Patients included 1 girl with a posttraumatic urethrovaginal fistula, 3 with intersex disorders (2 with mixed gonadal dysgenesis raised as boys and 1 with male dysgenetic pseudohermaphroditism with an enlarged urtricle) and 4 boys (1 with penile agenesis raised as girl, 2 with urethral duplication and 1 with prostatic rhabdomyosarcoma). The patient was placed in a knee-chest position. A midline sagittal incision was made through the anterior anorectal wall only and deepened through the perineal body to expose the posterior urethra and retrovesical space. After the pathological condition was corrected the anterior rectal wall and perineal body were reconstructed. The operation was completed with protective colostomy. In our final patient with prostatic rhabdomyosarcoma the anterior sagittal transanorectal approach was used without colostomy. Anorectal manometry was done 6 months postoperatively. RESULTS: All patients were completely continent of stool and urine. Convalescence was unremarkable in all cases. Postoperative manometry in 7 patients revealed no differences from preoperative measurements. CONCLUSIONS: This procedure should be considered a useful alternative to other techniques for various congenital and acquired pelvic disorders.

Multicystic kidney joined to the homolateral testis by an ectopic vas deferens: embryological hypothesis for a new description.

Ectopic location of the vas deferens is a rare congenital anomaly with multiple variations. The frequent association with ano-rectal abnormalities and hypospadias is well known. We report two cases of a newborn and a 3-year-old child with ectopic vas deferens coupled with a left multicystic kidney without any ureteral structure. Furthermore, the latter had a contralateral vesicoureteral reflux and anal agenesis with recto-bulbar fistula. Our attempt with this report is to explain the development of such anomalous deferential outlet into the multicystic kidney, according to the most qualified theory of the Wolffian duct embryology.

Esophageal leiomyomatosis in children: report of a case and review of the literature.

The authors describe a case of a 5-years-old girl affected by diffuse oesophageal leiomyomatosis presenting with progressive dysphagia, cyanosis, recurrent pneumonia and retrosternal pain. She also suffered from occasional constipation due to perineal involvement by the disease and had a past history of recurrent microscopic hematuria, suggesting an association with an Alport-like syndrome. Only 24 cases of esophageal leiomyomatosis could be found in the medical literature in children aged less than 14 years, confirming the rarity of the disease. A clinical analysis of all cases reviewed from the literature is made, stressing the importance of an accurate preoperative diagnosis for the choice of proper surgical treatment to avoid recurrence.

Microvascular autotransplantation of the testis: the "refluo" technique.

We think that the microvascular orchidopexy is the best technique for the treatment of the high intraabdominal testis. To prevent the problems related to the performing of the anastomosis between the spermatic and the inferior epigastric arteries we changed the traditional way for microvascular orchidopexy performing the venous anastomosis only, relying on the collateral deferential circle for the arterial supply; we called this technique the "refluo testicular autotransplantation". We supported our idea with an experimental investigation in rats and rabbits, with which we confirmed the efficacy of our proposal. This original technique, performed in 41 cases, allowed us to gain three important ends: a) reduction of the age at operation (under two years); b) reduction of the operating time (2 hours); c) improved success rate compared with the Fowler-Stephens technique or staged orchidopexy.