[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.

Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

[Intracranial arachnoid cyst in pediatric age]. / Le cisti aracnoidali intracraniche in età pediatrica.

Arachnoid cysts are a pathological condition in which an arachnoid-lined cavity is filled with a CSF-like fluid, often creating a disturbance in intracranial dynamics, due to shift and displacement of surrounding structures, and intracranial hypertension. Primary or congenital arachnoid cysts are maldevelopmental anomalies and contain clear CSF-like fluid. Acquired cysts may result from a variety of aethiologies such as traumas and infections. In these cases the intracystic fluid may be emosiderin-stained or may contain inflammatory cells. A general survey of this peculiar pathological finding in the pediatric age group is reported by the Authors in this paper.

[Hydrocephalus in the pediatric age group: considerations on a series of 245 shunted patients]. / L'idrocefalo in età pediatrica: considerazioni su una casistica di 245 pazienti operati di derivazione liquorale.

A series of 245 hydrocephalic patients in the pediatric age group who underwent 544 surgical shunting procedures is reviewed in this paper. The subsequent average of 1.5 operations per patient appears therefore significantly low, when compared with the Literature data. The authors emphasize that this result may be due mainly to the fact that all the surgical procedures were performed by the same surgeons, even if two other concepts appear extremely important in this regard: the great care in prevention of shunt infections and the use of one-piece shunting devices. A pattern for the "clinical surveillance" of hydrocephalic children is proposed and the neuropsychological follow-up of the series is described in the paper. Authors conclude that serious damage to cerebral parenchyma due to intracranial hypertension related to the hydrocephalic condition may be avoided by an accurate selection of patients to be operated on, a correct surgical timing and a careful postoperative control.

[Congenital anomalies of the penis in children. A few consideration about 92 cases]. / Malformazioni congenite del pene in età pediatrica. Alcune considerazioni a proposito di 92 casi.

The authors present their experience of 92 cases of anomalies of the penis, hypospadias excepted, occurring in children between 12 months and 10 years old, during the last 5 years, in their Pediatric Surgical Department. In the literature, there are reported only many single cases of these anomalies, for the most part associated in serious multiple malformations, or with hypospadias. The children included in this report present the anomalies of the penis as a sole malformations. The classification of these anomalies was made by embryological criteria and by the frequency of occurrence. In 41 cases there was congenital curvature of the penis, in 9 cases there was a webbed penis, in 6 cases a concealed penis. Torsion of the penis occurred in 6 cases (in 5 anticlockwise) the microphallus in 5 cases. The "hypospadias without hypospadias" occurred in 5 cases, in the others there was an association among these anomalies. The authors emphasize the importance of these malformations for the children and their families, also in order to prevent, also serious, psychological effects.

[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.

[Xanthogranulomatous pyelonephritis in childhood: considerations on 2 new cases]. / La pielonefrite xantogranulomatosa in età pediatrica: considerazioni a proposito di 2 nuovi casi.

The Authors report their experience about two cases of xanthogranulomatous pyelonephritis in childhood. The clinical history, diagnostic procedures and surgical treatment are described. The xanthogranulomatous pyelonephritis is a rare form of chronic inflammatory disease of the kidney, in which pre-operative differential diagnosis with renal cancer or with extra renal neoplastic and inflammatory diseases is very difficult. The signs and symptoms are not characteristic, such as renal tumour, recurrent UTI, abdominal pain, fever. Also non characteristic are the findings, performed with diagnostic ultra sound and computed tomography. The surgical treatment is always effective in the xanthogranulomatous pyelonephritis. About 500 cases are described in adults and about 80 cases in children, from the first description in 1963. The first of our cases, occurring in a boy 6 years old, was treated with nephrectomy, because of the pre-operatory aspect was like tumour. In the second of the cases described, occurring in a girl 11 years old, the nephrectomy was performed because the chronic purulent inflammatory process had involved all the kidney. In the post-operative, in the first case was performed a relaparotomy for intestinal occlusion in 12th day, in the second case the post-operative was uneventful. The Authors emphasize the importance of the xanthogranulomatous pyelonephritis in the differential diagnosis in children with recurrent UTI, renal masses, fever, in the clinical history.

[Acute scrotum in childhood: considerations on 457 cases]. / Lo scroto acuto in età pediatrica: considerazioni a proposito di 457 osservazioni.

The Authors present their experience about 457 consecutive cases of acute scrotum, occurring from 1980 to 1990, at their Pediatric Surgery Unit. The condition is caused by torsion of the appendage of the testis (78.1%), torsion of the funicle (9.6%), acute idiopathic oedema of the scrotum (2.87%), and also tumours (1.17%). Also in their experience, the surgical approach is the correct treatment for the acute scrotum in order to prevent the irreversible damage of the testis. The surgical treatment present no problem and all the post-operatives were uneventful. In children under 2 years, the interventions were performed by inguinal incision, above 2 years by scrotal incision. The contralateral orchiopexy, in case of torsion of the funicle, was performed in the same session, or performed later, because of the anesthetic consideration.

Skin substitutes in the treatment of deep partial skin thickness burns in children: clinical experience and long-term results.

This report describes the use of skin substitutes in the treatment of deep partial skin thickness burns in childhood. These are lesions that, if treated inadequately, can result in severe scarring. However, if treated appropriately, they can heal without any sequelae, which is obviously crucial for aesthetic and psychological reasons. This review contains children admitted to the authors' Burn Unit over a 5-year period (1984-88) with deep partial skin thickness lesions which were treated with synthetic and/or biosynthetic skin substitutes and without surgical procedures. This group of children has been compared with another group hospitalized for burns of the same depth and treated with conventional closed wound management. First, short-term results are presented, highlighting healing time, followed by the long-term results from an aesthetic and functional viewpoint.

Spermatic and peripheral venous plasma concentrations of testosterone, 17-hydroxyprogesterone, androstenedione, dehydroepiandrosterone, delta 5-androstene-3 beta,17 beta-diol, dihydrotestosterone, 5 alpha-androstane-3 alpha,17 beta-diol, 5 alpha-androstane-3 beta,17 beta-diol, and estradiol in boys with idiopathic varicocele in different stages of puberty.

Serum testosterone (T), 17-hydroxyprogesterone (17P), androstenedione (delta 4-dione), dehydroepiandrosterone (DHA), delta 5-androstene-3 beta, 17 beta-diol (delta 5-diol), estradiol (E2), dihydrotestosterone (DHT),5 alpha-androstane-3 alpha,17 beta-diol (3 alpha diol), and 5 alpha-androstane-3 beta,17 beta-diol (3 beta diol) were measured in the peripheral and spermatic venous blood of 21 boys undergoing surgery for idiopathic left varicocele. The boys were divided into 3 groups according to their pubertal development: prepubertal (group 1 or P1; n = 8), pubertal stage 2 (group II or P2; n = 6), and pubertal stages 3-4 (group III or P3-4; n = 7). The testes of the prepubertal boys secreted T, 17P, DHA, delta 5-diol, DHT, and 3 alpha diol, but not delta 4-dione, E2, and 3 beta diol. In pubertal stage P2, the mean spermatic-peripheral secretory gradients of T, 17P, DHA, delta 5-diol, DHT, and 3 alpha diol were significantly higher than those in the prepubertal stage, and there was testicular secretion of delta 4-dione, E2, and 3 beta diol. In pubertal stage P3-4, the mean spermatic-peripheral secretory gradients of most of these steroids, even if increased, were not significantly different from those in stage P2 (with the exception of 17P, delta 5-diol, and DHA). We suggest that after the important modifications of testicular secretion occurring in pubertal stage P2, the testicular secretory pattern of the pubertal testis is similar to the pattern of the adult testis. We suggest also that these results, obtained in boys with idiopathic varicocele, can probably be extended to the secretory activity of the testes of normal pubertal boys.

[Cystic renal disease in childhood]. / La malattia cistica renale nell'infanzia.

In the last five years, in the Surgery Pediatric Department and in the Pediatric Nephrology and Dialysis Service of the Florence University, have been observed 13 patients with cystic renal diseases. In every single case, for the diagnosis we have considered the age of the patients and the examinations made to them. We payed attention mostly to the echography considered the main diagnostic examination, even in prenatal age.

[Intestinal infections in the course of continuous elemental enteral feeding by way of the jejunum in the infant]. / Infezioni intestinali in corso di alimentazione enterale elementare continua per via digiunale nel lattante.

The authors take an interest in the study of the intestinal infections during continuous enteral elemental feeding by jejunum in infants. They discuss the pathogenesis and indicate the measures in order to prevent that complications. The Authors report 9 cases of infants on whom an enteral (jejunal) elemental feeding was made up: on the basis of their experiences they consider that the occurrence of intestinal infections does not compromise the employing of the enteral feeding in front of other methods of artificial nutrition.

[Rare case of double cyst of the omentum]. / Su una rara osservazione di duplice cisti dell'omento.

In Pediatric Surgery "A. Meyer's" Hospital of Florence in the period 1973-1982 a people of 140.000 children is recovered. The AA report a case of double omental cyst, that is arrived in Hospital only for an increase of the abdomen. The omental cyst are uncommon in pediatric age and presents etiopathogenetic aspects still controverted.

[Opportunistic infections in burned children]. / Infezioni opportunistiche in bambini ustionati.

The burn injury is a typical pathology in which there is a possibility of opportunistic infections because the patients have a severe derangement in their defense. The authors examine etiology, pathogenesis, simptomatology, diagnosis, prophylaxis and therapy of these infections. The authors also report their own experience about these infections based on study of a group of burned children hospitalized in the pediatric surgery unit of A. Meyer hospital in Florence.

Injection therapy with triamcinolone hexacetonide in the treatment of burn scars in infancy: results of 44 cases.

In many cases deep second degree and third degree burns cause severe scarring. The authors have reported here their experience of the treatment of hypertrophic scarring from burns carried out on 44 children with intralesional injections of a long-acting cortico-steroid (Triamcinolone hexacetonide) using the jet spray technique.