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.

Massive ovarian edema in a prepubertal girl without signs of virilization or early puberty.

Massive ovarian edema is a rare cause of ovarian enlargement in young women. This article describes the case in a nine-year-old girl without signs of virilization or early puberty. The patient underwent laparotomy and a right oophorectomy was performed. The final pathology was massive ovarian edema. Awareness of this rare and benign lesion in young women and pediatric patients may allow a conservative management avoiding oophorectomy in some cases.

MODIS and OMI satellite observations supporting air quality monitoring.

Within the framework of air quality monitoring, measurements by Earth-observing satellite sensors are combined here with regional meteorological and chemical transport models. Two satellite-derived products developed within the QUITSAT project, regarding significant pollutants including PM(2.5) and NO(2), are presented. Estimates of PM(2.5) concentrations at ground level were obtained using moderate resolution imaging spectroradiometer (Terra-Aqua/NASA) aerosol optical properties. The semi-empirical approach adopted takes into account PM(2.5) sampling and meteorological descriptions of the area studied, as simulated by MM5, to infer aerosol optical properties to PM projection coefficients. Daily maps of satellite-based PM(2.5) concentrations over northern Italy are derived. Monthly average values were compared with in situ PM(2.5) samplings showing good agreement. Ozone monitoring instrument (OMI) (Aura/NASA) NO(2) tropospheric contents are merged using the GAMES chemical model simulations. The method employs a weighted rescaling of the model column in the troposphere according to the OMI observations. The weightings take into account measurement errors and model column variances within the satellite ground pixel. The obtained ground-level concentrations of NO(2) show good agreement with the environmental agencies' in situ.

Left pneumonectomy and expandable prosthesis placement for pleuropulmonary blastoma in a infant: one year follow-up.

Pneumonectomy is a rare procedure in pediatric age. In contrast to adults, children that undergo this intervention are prone to postoperative mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. The authors present the case of a 9-month-old infant affected with a pleuropulmonary blastoma who underwent left pneumonectomy and prosthesis placement in the same operation.

Cytosolic calcium concentration is reduced by photolysis of a nitrosyl ruthenium complex in vascular smooth muscle cells.

The effect of the NO donors cis-[RuCl(bpy)(2)(NO)](PF(6)) (RUNOCL) and sodium nitroprusside (SNP) on the cytosolic Ca(2+) concentration ([Ca(2+)](c)) was studied in cells isolated from the rat aorta smooth muscle of cells isolated from the rat aorta smooth muscle. SNP is a metal nitrosyl complex made up of iron, cyanide groups, and a nitro moiety; the RUNOCL complex is made up of ruthenium and bipyridine ligands, with chloride and nitrosyl groups in the ruthenium axial positions. Rat aorta smooth muscle cells were loaded with fluo-3 acetoxymethyl ester (Fluo-3 AM) and imaged by a confocal scanning laser microscope excited with the 488 nm line of the argon ion laser. Fluorescence emission was measured at 510 nm. One of the NO donors, RUNOCL (100 micromol/L) or SNP (100 micromol/L), was then added to the cell chamber and the fluorescent intensity percentage (%IF) was measured after 240 s. RUNOCL reduced the %IF to 60.0+/-10.0% of the initial value. After treatment with the soluble guanylyl cyclase inhibitor 1H-[1,2,4]oxadiazole[4,3-a]quinoxalin-1-one (ODQ) (10 micromol/L), the measurement of %IF was 81.0+/-5.0% (n=4). In the presence of tetraethylammonium (TEA) (1 mmol/L) the %IF was 79.0+/-6.4% (n=4). A combination of ODQ and TEA increased the %IF to 97.0+/-3.5% (n=4). As for SNP, it reduced the %IF to 81.4+/-4.7% (n=4), but this effect was inhibited by ODQ (%IF 94.0+/-3.6%; n=4) and TEA (%IF 88.0+/-2.1%; n=4). The combination of ODQ and TEA increased (%IF 92.0+/-2.8%; n=4). Taken together, these results indicate that both the new NO donor RUNOCL and SNP reduce [Ca(2+)](c). Our data also give evidence that soluble guanylyl cyclase and K(+) channels sensitive to TEA are involved in the mechanisms responsible for the reduction in [Ca(2+)](c) of the rat aorta smooth muscle cells.

Ileocaecal valve atresia: our surgical approach.

BACKGROUND: Atresia of ileocaecal valve is the rarest type of intestinal atresia; there are very few reports on it in the literature and all cases described to date were treated with ileocolic resection. METHODS: We present a rare case of ileocaecal valve atresia, the third in the literature, in which a "different" technical approach was employed as an alternative to the usual surgical technique (ileocolic resection). The described case came to our attention in a patient presenting with complicated intestinal perforation in whom we performed an ileal stoma after an unsuccessful conservative approach. Approximately four months later we operated her to close the stoma: during this procedure we discovered the colic valve atresia and treated it by creating a "new ileocaecal valve" and carrying out an appendectomy. After a 7-year follow-up no complications have occurred and the girl enjoys very good health. CONCLUSIONS: We conclude that performing a plasty of Bauhin's valve is a valid alternative to intestinal resection in such cases of atresia even if, according to some authors, the resection of the ileocaecal valve was not associated with increased morbidity and mortality (3).

High-frequency oscillatory ventilation versus conventional mechanical ventilation in congenital diaphragmatic hernia.

AIMS: Newborns affected by congenital diaphragmatic hernia (CDH) are high-risk patients: today the mortality is still elevated and is essentially due to severe pulmonary hypoplasia, pulmonary hypertension and the absence of surfactant. High-Frequency Oscillatory Ventilation (HFOV) seems to be a good pre- and postoperative technique in cases of CDH. PATIENTS AND METHODS: We report our experience in the treatment of CDH; since 1987 we have followed 44 patients. We divided them into two different groups in accordance with the ventilation technique used: Group I (1987-1994): 25 patients treated with conventional mechanical ventilation (CMV); Group II (1994-1997): 19 patients treated with HFOV used since the first day of life until clinical stabilisation and also prolonged during surgery and in the postoperative period. RESULTS: 37 patients (84%) underwent surgery with closure of the diaphragmatic defect. We had a very good improvement in survival, rising from 67% (CMV) to 94% (HFOV) of the patients operated on, reaching an overall survival of 56% vs 79%. CONCLUSIONS: The use of HFOV for the treatment of CDH has proved to be a valuable technique for pre-operative stabilisation and for intra- and postoperative respiratory treatment, above all for newborns with CDH.

Rhabdomyoma of a rare type in a child: case report and literature review.

We describe a 9-year-old boy who presented with dyspnea and with a neck mass which was initially described as a thyroid nodule. At the end of the diagnostic-therapeutic research this mass was finally diagnosed as an adult rhabdomyoma originating from the mediastinum. This is an extremely rare tumor which is usually found in the adult population.

Prenatal diagnosis of bladder exstrophy: what counseling?

PURPOSE: Bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases. MATERIALS AND METHODS: At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy. RESULTS: We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria. CONCLUSIONS: We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy.

A series of 17 cases of congenital cystic adenomatoid malformation of the lung: management and outcome.

During a 5-year-period, 17 cases of congenital adenomatoid malformation of the lung were observed in the Department of Pediatric Surgery of Bologna University-S. Orsola-Malpighi Hospital; 16 cases were diagnosed in utero by ultra-sound. 13 patients had prenatal diagnosis between 21 and 23 weeks of gestation; 2 cases were diagnosed at 25 weeks and another one at 26 weeks. The other case came from another hospital, and the diagnosis was accidentally detected postnatally. In 3 cases the parents decided to terminate the pregnancy 3 cases had spontaneous improvement during the last weeks of gestation, and were perfectly normal at birth. One case was lost to follow-up; all remaining patients underwent resection. Among these patients only one was a nonsurvivor, while 9 have completely recovered. However, one patient developed persistent pulmonary hypertension, which could successfully be treated with drugs, another underwent laser treatment for laryngeal stenosis due to intubation. A special epidemiological information: 13 out 17 cases (76.5%) were female.

[Relationship between prenatal and postnatal echographic diagnosis of uropathy: is mass screening useful?]. / Correlazione tra diagnosi ecografica prenatale e postnatale delle uropatie: ha valore uno screening di massa?

Urinary tract malformations have a 1-2% incidence. An early diagnosis of these defects allows to realize as fast as possible the best medical and/or surgical treatment, preventing or at least slowing down the evolution toward chronic renal failure. Urinary tract malformations are particularly suitable for a "prevention" program due to their elevated incidence, to the "silent period" preceding complications and to the therapeutic possibilities strictly related to an early diagnosis. Ultrasound screening can easily identify congenital urinary tract abnormalities, especially obstructive ones, but unfortunately almost half of the cases escape even the most expert "eye". It is necessary a complete check-up right after birth. We tried to compare the method and the results of prenatal screening with the postnatal one, already used in many hospitals. We believe, even following our experience, that the screening for urinary tract malformations of all the neonatal population is a goal to pursue and achieve as soon as possible for its high sensitivity and specificity.

[Risk factors and protective factors in a population a risk for newborn necrotizing enterocolitis]. / Fattori di rischio e fattori protettivi in una popolazione esposta a enterocolite necrotizzante neonatale.

NEC represents the most common gastrointestinal disorder in newborn. Its range varies from 1% to 7.7% and is frequently associated with factors such as intestinal ischaemia, prematurity, gastrointestinal infection and early and rapid enteral feeding. Between 15/1/1990 and 15/6/1995, 129 critically ill newborns were admitted in NICU of Policlinico S. Orsola-Bologna. We examined only 93 patients, hospitalized for over 48 hours, presenting one or more risk factors for the development of NEC, such as birthweight < 2000 gm, respiratory distress, gastrointestinal bacterial colonization, sepsis, PDA and use of umbilical catheters. The aim of the study was to evaluate NEC incidence in newborns exposed to this complication and the analysis of risk factors associated with the elements of prevention and protection. No cases of NEC were observed despite the high incidence of risk factors. The newborns studied were divided in six different groups with increasing risk factors. Among the prevention elements of NEC, every patient was treated by nutrition, at first exclusively by TPN followed by careful enteral feeding (< 20 ml/kg/die) and the improvement of mesenteric blood flow by dopamine (2-3 mcg/kg/min); other preventive treatments were given according to clinical condition: dobutamine (5-10 mcg/kg/min in 51 ps.) to improve the cardiovascular function, gastrointestinal decontamination (8 ps.), antibiotic therapy (81 ps.), in cases of diagnosed infection and intravenous immunoglobulin (25 ps.) after discovering low ematic values. Analyzing the treatments and their day numbers in the 6 groups of patients no statistically significant differences were evident. On the contrary, dividing the patients into 3 groups according to GA (< 30 w, 30-35 w, > 35 w) an extension in treatment time is more evident in the group of GA < 30 weeks. Our therapeutic behaviour, based on respect of gastrointestinal blood flow, careful and gradual enteral feeding and prevention, constant monitoring and infection treatment, has been useful to stop the NEC incidence.

Lumbar hernia as congenital aplasia of lumbo-dorsal muscle.

A newborn with congenital aplasia of the dorso-lumbar muscles is described. The lesion seemed to be a congenital lumbar hernia and is a very uncommon feature. The child underwent surgery and although it was a very large bilateral defect, it was repaired without using prosthetic patch. The surgical technique and the results are discussed. The function of the region was conserved as demonstrated at the check-up of the child at 6 and 12 months of age.

Prenatal diagnosis and management of fetal ovarian cysts.

Congenital ovarian cysts are a pathological condition which can be diagnosed in utero by ultrasound. We report 14 consecutive diagnoses of fetal ovarian cysts, obtained in the second and third trimesters of pregnancy. Congenital ovarian cysts have almost invariably a good prognosis, and no change in the standard obstetrical management is required. However, the evolution of the disease in utero is extremely variable. The cyst may increase in size, decrease or even disappear, or undergo complications such as torsion and rupture, which may carry some risks to the fetus. When a fetal ovarian cyst is detected, serial ultrasound examinations should be performed. If one of the above complications is suspected, the option of prompt caesarean section should be considered.

Prenatal diagnosis and obstetrical management of multicystic dysplastic kidney disease.

Multicystic dysplastic kidney disease (MDKD) is one of the most common congenital renal anomalies. We report 16 consecutive cases of MDKD recognized in the antenatal period by sonography. Diagnosis is usually easy as MDKD has in the vast majority of cases a striking ultrasound appearance including enlargement of the kidney and multiple renal cysts. However, differentiation from obstructive uropathy may be difficult, and we made a total of five erroneous diagnoses. Unilateral MDKD has almost invariably a good prognosis. However, severe life-threatening associated anomalies were found in six cases. Therefore, a detailed survey of fetal anatomy and determination of karyotype are strongly recommended.

Abdominal wall defects: neonatal surgical problems.

The advantages accruing from the prenatal diagnosis of gastroschisis and omphalocele are outlined. The surgical management of each defect is discussed, including details of the various surgical options available at the time of the repair. Seventy-five cases with abdominal wall defects born between 1970 and 1984 without an antenatal diagnosis having been made are compared with 8 recent cases in whom an antenatal diagnosis was made. The early results suggest that antenatal diagnosis improves survival.

Gastrointestinal tract anomalies: neonatal surgical problems.

This report deals only with anomalies, treated after prenatal diagnosis, with which we have experience. Other malformations have been intentionally excluded. The neonatal management of duodenal atresia and jejunoileal atresia is briefly outlined. Our early results in 7 cases treated after antenatal diagnosis are compared with 81 cases treated without the benefit of antenatal diagnosis. Antenatal diagnosis appears to improve the outcome in these patients.

Genitourinary tract anomalies: neonatal surgical problems.

This paper is based on our experience in managing 79 cases of genitourinary tract anomalies diagnosed in utero and treated after birth. These babies seldom present as emergencies, and the antenatal diagnosis can usually be confirmed in the first few days of life. The surgical correction of these babies requires the services of a surgeon skilled and experienced in neonatal surgery. Surgeons experienced only in adult surgery may find these procedures technically difficult. Magnification is recommended. Postoperatively careful attention to fluid and electrolyte balance and correct functioning of drainage tubes is stressed. Antibiotic prophylaxis is vital, as is long-term follow-up.

Abdominoscrotal hydrocele in childhood. Case report and review of the literature.

Abdominoscrotal hydrocele in infancy and childhood is a very rare entity. A review of the literature reveals only five cases at this stage. Symptoms are often poor and non-specific and the mass must be differentiated from other abdominal ones; to achieve this, I.V.P., cystography and sonography play an important role. Total excision is necessary to obtain permanent cure.

[Neonatal necrotizing enterocolitis (NEC). Observations of 21 cases studied from 1977 to 1980]. / Enterocolite necrotizzante del neonato (NEC). Rilievi su 21 casi osservati negli anni 1977-1980.

We have carried out a study together with neonatologists, paediatricians, paediatric surgeons and paediatric radiologist on the etiopathogenic, clinical-statistical, therapeutical and evolutionary aspects of NEC during the period from 1977 to 1980 in the Neonatology Clinic and in the Paediatric Surgery Department University of Bologna. The most important data were as follows: a high percentage of full-term newborns, the apparently unfavourable action of artificial feeding, a little amount of infectious etiology, an absence of predisposing factors in more than half the cases, an uniform symptomatology in a lot of patients with adverse evolution in the cases of secondary colic stenosis. The mortality was 33 percent, specially among the small for gestational age infants. The surgical therapy during four years period was modified technically, i.e. there was an orientation towards conservative surgery. The final surgical operation has recently been made right after the fourth month from the acute crisis as it has recently been demonstrated that secondary stenosis always occurs during this period of time. Long - term follow - ups showed: completely satisfactory progress in patients treated only with medical therapy, particularly unfavourable evolution in patients after a more demolishing - type of operation (resectioning of the last ansa of ileum and ileum - caecum valve), considerable improvement in auxological and nutritional parameters also in the latter cases after the final surgical operation.