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.

The role of endoscopy in the treatment of IV type laryngotracheal cleft complications after surgical repair.

Laryngotracheal cleft (LTC) is a rare congenital anomaly. Severe forms of LTC are usually treated surgically using extracorporeal circulation. Despite tremendous improvement of surgical techniques, postoperative complications are still frequent. We report a comprehensive description of an endoscopic approach to diagnosis, surgical repair and endoscopic treatment of complications after correction in a type IV LTC.

Neonatal adrenal hemorrhage mimicking an acute scrotum.

Twenty-two cases of scrotal hematoma caused by neonatal adrenal hemorrhage are reported in the literature and unnecessary surgical exploration was performed in nine (41%), suspecting testicular torsion. In this paper, we present a newborn male with right adrenal gland hemorrhage causing right scrotal swelling and discoloration of groin managed conservatively.

Initial experience with laparoscopic adrenal surgery in children: is endoscopic surgery recommended and safe for the treatment of adrenocortical neoplasms?

Through a review of 83 cases reported in literature, including our experience of two successful right laparoscopic adrenalectomies performed in a 3-year-old girl for androgen-secreting adenoma and in a 9-year-old male for pheochromocitoma, we have anaIyzed the indications, the techniques and the results of laparoscopic or retroperitoneoscopic adrenalectomy in children. Nineteen pheochromocytomas, 18 neuroblastomas, 15 adenomas, 12 ganglioneuromas, 9 hyperplasias, 2 carcinomas, 1 teratoma and 1 adrenogenital syndrome have been treated with excellent results (follow-up 1 month to 4 years) with no evidence of recurrence. The age range is from 1 month to 16 years with an equal female/male ratio. The tumor size range from 10 mm to 80 mm with approximately a mean of 40 mm. Sixty-three laparoscopies of which 54 lateral and 9 anterior have been performed versus 14 posterior retroperitoneoscopies. Regarding right adrenalectomy, 29 were laparoscopic and only 2 retroperitoneoscopic. With regards to left adrenalectomy, 23 were laparoscopic and 9 retroperitoneoscopic. Looking at bilateral lesions, these tumors were approached laparoscopically in 6 cases alternating both sides and 1 retroperitoneoscopically (this case was already operated on the other side). The operative time ranged from 25 minutes in newborn to 320 minutes in bilateral cases with an average of 120 minutes. Conversion rate is higher in retroperitoneoscopy (30%) (4 cases out of 13), than laparoscopy (6%) (4 cases out of 63). Specifically, for right retroperitoneoscopic adrenalectomy conversion rate was 100% (2 cases out 2), for left retroperitoneoscopic adrenalectomy 22.2% (2 cases out 9), for right laparoscopic adrenalectomy 6.8% (2 cases out of 29) and for left laparoscopic adrenalectomy 8.6% (2 cases out of 23). Only in 1 case were blood transfusions requested. Hospital stays ranged from 35 hours to 17 days with an average of 4 days. In conclusion the indications of endoscopic adrenalectomy in children are not different from those of traditional surgery and the well-known advantages of laparoscopic adrenal surgery should be applied to pediatric patients. It seems that there is no age and tumor size limits for a well-trained surgical team. Lateral transperitoneal approach is the most utilized with the child positioned in 90-degree flank decubitus. Laparoscopy is undoubtly preferred for right adrenalectomy (93% of cases), while for left adrenalectomy retroperitoneoscopy has been used in 39% of the cases. Considering the conversion rate and on the basis of our experience with adults, we recommend laparoscopic adrenalectomy for both right and left adrenal lesions, but we think that the pediatric surgeon should feel free to choose the approach in which he/she is more skilled. However, the best surgical result will be achieved if the pediatric and adult surgeon collaborate with their different experiences. Lastly, we suggest the use of new technological devices such as Ultracision Harmonic Scalpel which was a critical factor in our two successful right adrenalectomies.

[Esophageal atresia with distal tracheo-esophageal fistula. Evolution of the treatment in the period of 1955-2000 at the Anna Meyer Children's Hospital of Florence]. / Atresia dell'esofago con fistola esofago-tracheale distale. Evoluzione del trattamento nel periodo 1955-2000 nell'Ospedale Pediatrico Anna Meyer di Firenze.

BACKGROUND: Over the past decades the esophageal atresia (EA) has represented the greatest challenging malformation encountered by the pediatric surgeon. Since then, there have been considerable advancements in the treatment of EA. In this paper the experience at the "Anna Meyer Children's Hospital of Florence" in regards to the surgical treatment of the EA from 1955 to present day is reported, so that the analysis of the various medical and surgical choices followed by the authors and their predecessors in this long period, can be an important learning tool for the EA management. METHODS: From 1955 to 2000, 223 newborns affected by EA with tracheoesophageal fistula (TEF) have been operated on out of a total of 250 cases of EA. Our experience has been subdivided into periods on the basis of homogeneous medical and surgical treatment adopted in that determined time. We have analyzed particularly the data of the last period 1995-2000, where there has been a well standardized protocol of treatment from the medical, surgical and intensive care points of view. RESULTS: The mortality rate has decreased from 44.8 to 3.4% with a significant reduction (p<0.001) between the years 1979-1983 and 1984-2000, due to the introduction of a perioperative treatment in the newborn intensive care unit. Moreover, a significant correlation (p<0.05) has been shown between low birthweight and associated malformations, two risk factors that however do not negatively influence the results of the treatment in the last period 1995-2000. CONCLUSIONS: A full integration between the surgeon and neonatologist is necessary in order to guarantee a good result. The risk connected to EA is not as much the surgical procedure as the presence or absence of associate malformations that are undetected in the prenatal diagnosis. It is suggested that, in order to further reduce the mortality and morbidity rate after EA correction, the number of prenatal diagnoses should be increased.

Laparoscopic decapsulation of a large epidermoid splenic cyst in a child using the UltraCision LaparoSonic Coagulating Shears.

Splenic cysts are rare in pediatric surgery. Congenital epidermoid cysts are exceptional representing only 2.5% of all splenic cysts in childhood. Nowadays, considering the short- and long-term complications of splenectomy in children, the management of epidermoid cyst consists of partial splenectomy or decapsulation of the cystic wall. To our knowledge, the case reported in this article describes the first successful laparoscopic decapsulation of an epidermoid splenic cyst in an 10-year-old child using the UltraCision LaparoSonic Coagulating Shears (LCS). Follow-up at six months confirms no recurrence. Laparoscopic splenic decapsulation provides minimal access and small surgical trauma for treating the cyst while preserving splenic function. The use of UltraCision LCS makes the laparoscopy safely, expeditiously, with minimal blood loss and short hospital stay.

[Use of a Gore-Tex patch in the primary repair of congenital defects of the anterior abdominal wall]. / Impiego di patch di Gore-Tex nella riparazione primaria dei difetti congeniti della parete addominale anteriore.

The surgical treatments for large omphaloceles and gastroschisis have to avoid a dangerous primary fascial closure when it's not feasible, so the pediatric surgeon can use staged surgical procedures that achieve a gradual increase in size of the abdominal cavity. Therefore, a staged approach is mandatory to avoid a complicated reduction and it's the best treatment for large congenital abdominal wall defects. Gore-Tex soft tissue is easy to handle, pliable, soft and well tolerated, so Gore-Tex patch closure is a safe and good alternative to staged repair in large omphaloceles and gastroschisis. The Authors report about five newborns with large congenital abdominal wall defects, in whom primary fascial closure could not be accomplished, so they were successfully treated by primary repair with Gore-Tex patch. The Authors also describe the surgical technique and they reconsider the last surgical techniques for large omphaloceles and gastroschisis.

[Primary acute acalculous cholecystitis in childhood: a report of 2 clinical cases]. / La colecistite acuta alitiasica primitiva nell'infanzia: descrizione di due casi clinici.

Acute acalculous cholecystitis (AAC) is not frequently encountered in adults and children whether in association with other conditions or above all in primitive form. AAC in infancy, although rare, is well recognized, but its possible presentation is not always kept in mind in considering the differential diagnosis of the acute abdomen. On the other hand, AAC has significant clinical signs and abdominal u.s. scanning usually provides evidence of diagnosis. Only early diagnosis has been shown to limit high morbidity and mortality rates for AAC, since cholecystectomy is the simple procedure of choice for treatment of AAC. We report two cases of primitive AAC one in a six and one in two half years old girls. So we consider the most important features about etiologic factors, pathogenesis, clinical signs and therapy through review of the literature and our personal experience.

[Ectopia cordis and Cantrell's pentalogy: personal experience and considerations on the surgical treatment]. / Ectopia Cordis e Pentalogia di Cantrell: esperienza personale e considerazioni sul trattamento chirurgico.

Clefts of the sternum have always attracted attention whether for pathological and physiological features or for research of surgical correction. Two cases of sternal cleft, one with partial ectopia cordis, the other with Cantrell's pentalogy, are presented. Embryology, strategies and several surgical techniques are discussed on the grounds of personal experience. The pediatric surgeon can make a choice among a lot of surgical techniques, because the ectopia cordis and Cantrell's pentalogy are very uncommon and the surgical treatment has a difficult codification. The knowledge of several methods of surgical correction is necessary to reduce high mortality of ectopia cordis and Cantrell's pentalogy. Primary repair in the neonatal period is the best type of management for these rare conditions, because simple closure of the sternal defect during the first month of life avoids the more complex reconstruction necessary in older children.

[Genetic aspects of hypospadias: considerations apropos of 362 cases]. / Aspetti genetici dell'ipospadia: considerazioni a proposito di 362 osservazioni.

The Authors present 362 cases of hypospadias, during 10 years, considering genetical aspects of the malformation. The mean frequency rate of hypospadias is 0.32%. In 60 of the examined patients (17.1%) there was another subject with hypospadias and in 12 cases (4.3%) there was a third subject with the malformation. In 32 cases (9.1%) the relationship was between brothers and in 15 cases (4.2%) the fathers were concerned. Thus, each group had a clean increasing risk in comparison with the mean frequency rate. From the analysis of the results appears a not mendelian inheritance and not even sex linked or single locus linked, in the transmission of the characters. The Authors emphasize the role of some hereditary predisposition in concomitance of environmental conditions, to date not well known. The hypospadias, thus, to fit in the "mid-line pathology", a recent chapter of genetics, characterized by a lack of coalescence, just in the mid-line of embryonal development, during organogenesis.

[Gastroesophageal reflux and respiratory pathology]. / Reflusso gastroesofageo e patologia respiratoria.

The prevalence of gastroesophageal reflux (GER) in 86 children with respiratory disease (recurrent pneumonia, chronic cough, bronchial asthma) has been evaluated by mean of prolonged (22-24 hours) esophageal pH-monitoring. The following parameters were evaluated: the total percentage of time pH < 4 and the percent time the esophageal pH was < 4 while sleeping. None of the children had gastrointestinal symptoms suggesting GER and no neurological disorder was noted in any of the studied patients. The mean age was 68.98 +/- 46.46 months (range 14-189); 53 (61.6%) males and 33 (38.4%) females were considered in the study. Atopy was evidenced in 42/86 (48.8%) children (total IgE > 2SD in 42/86 and prick tests positiveness in 32/86. A pH-metry indicating pathological GER was present in 52/86 (60.5%) children: 39/62 (62.9%) patients with bronchial asthma, 5/10 (50%) subjects with chronic cough and 8/14 (57.2%) children with recurrent pneumonia. No significant difference in the diagnosis of GER was recorded between atopic or non-atopic patients. The children with abnormal pH-metric recording were also evaluated by upper gastrointestinal series and/or endoscopy. A conventional barium radiology was performed in 44/52 patients and confirmed GER in 19/44 (43.2%). Esophagitis was evidenced in 21/46 (45.7%) studied patients. The presence of esophagitis was significantly (p = 0.032) related to the total percentage of time pH < 4, but the most significant (p = 0.002) association was with the percent time the esophageal pH was < 4 during sleep.(ABSTRACT TRUNCATED AT 250 WORDS)

[Sensitivity and specificity of antigliadine antibodies in the diagnosis of celiac disease in a population of children in Toscana]. / Sensibilità e specificità degli anticorpi antigliadina nella diagnosi di celiachia in una popolazione toscana in età pediatrica.

The aim of this study was to determine the value of IgA and IgG antigliadin antibody test (AGA) for screening, diagnosis and follow-up of coeliac disease. A rapid, simple, sensitive and accurate immunosorbent assay (ELISA) was used to determine AGA IgA and IgG in the sera of patients with untreated coeliac disease (I stage), coeliac patients in gluten withdrawal, healthy controls, children with gastroenterological disorders other than coeliac disease and children with constitutional short stature. In the I stage of coeliac disease the AGA IgA gave a sensitivity of 90.9% and a specificity of 97.9%, the IgG assay resulted in a sensitivity of 100% and a specificity of 82.3%. AGA IgG resulted positive in 17.7% of control disease group, but it's interesting to remark that they had a mean level significantly lower than in coeliac patients. On gluten free diet the titres of AGA IgA returned to normal value in three months, while the AGA IgG showed a slower decrease.