[Cystic lymphangioma of the abdominal wall in childhood: case report]. / Linfangioma cistico della parete addominale in età pediatrica. Caso clinico.

Intra-abdominal lymphangiomas are rare benign tumours that can cause various symptoms, mainly during childhood. They are diagnosed by ultrasonography, CT scanning or at laparotomy; ultrasonographic examination often shows a voluminous tumoral cystic formation with septa. The location of the cyst may be determined either by ultrasonography alone or by CT scanning. The definitive histological diagnosis is confirmed by immunohistochemical staining techniques. Rarely intra-abdominal lymphangiomas can occur in the abdominal wall. Complete resection is the treatment of choice. The case of a 2-year-old-boy with a large lymphangioma involving the lower half of the abdominal wall is reported. The patient underwent the complete removal of lymphangioma with good cosmetic result.

[The Currarino syndrome: two case reports]. / La sindrome di Currarino: due casi clinici.

The association of congenital anal stenosis, or other anal and rectal malformation, sacral defect and a presacral mass is known as the Currarino syndrome described for the first time in 1981. Currarino et al. proposed that abnormal endoectodermal adhesions and notochordal defects in early fetal life may result in a fistula between the gut and the spinal canal with enteric elements ventrally and neural elements dorsally. This abnormality appears to be a variant of the split notochord syndrome. The occurrence of Currarino's triad of anomalies is familial in more than 50% of cases. The most important suggested hypothesis of transmission is an X-linked dominant pattern, but most of the other reports are consistent with an autosomal dominant mode of inheritance. The medical therapy is poorly successful and, therefore, the surgical treatment is recommended for Currarino's syndrome.

A long-term follow-up of autoaugmentation in myelodysplastic children.

OBJECTIVE: To evaluate the long-term results of patients who underwent bladder autoaugmentation (BA) in whom BA was used to treat a neuropathic bladder secondary to myelomeningocele, and who presented with a high-pressure/poorly compliant bladder. PATIENTS AND METHODS: Eleven patients (eight girls and three boys, mean age 12.8 years, mean follow-up 6.6 years) were selected who had undergone BA between June 1991 and June 1994. At surgery, the patients had a poorly compliant bladder with a mean leak point volume (LPV) of 94 mL and a mean leak point pressure (LPP) of 58 cmH2O. None of the patients had vesico-ureteric reflux (VUR) at BA; five with grade III or IV VUR had undergone endoscopic correction in a day-surgery procedure using a suburethral collagen injection 1-3 weeks before BA. The patients were evaluated using clinical, urodynamic, radiological and endoscopic assessments. The LPV, LPP and safe bladder capacity (SBC, the cystometric volume at an intravesical pressure of 40 cmH2O) were recorded. RESULTS: At 1 year after surgery the mean LPV was 297 mL; none of the patients had VUR. The most recent mean LPV was 198 mL and the SBC 167 mL. The mean LPP remained stable at 60 cmH2O. At the last follow-up four patients had recurrent uni- or bilateral grade III-V VUR. At endoscopy the 'augmented' bladder portion had a smooth surface, compared with the grossly trabeculated lower half. From this finding all patients on clean intermittent catheterization were treated with oral oxybutynin. Some patients reported slightly less abdominal pain at maximum bladder volume; four needed pads to treat intermittent incontinence. On voiding cysto-urethrography, one patient had an hourglass-shaped bladder. Five patients recently underwent ileocystoplasty because of recurrent urinary tract infection, high-grade VUR and incontinence. CONCLUSION: These results do not justify the routine use of BA in hypertonic/poorly compliant bladders secondary to myelomeningocele. The mean follow-up of 6.6 years showed that this procedure failed in seven of 11 patients. Further studies might be able to identify subgroups in which this approach may be more appropriate.

[Biliary atresia syndrome and splenic malformation. A case report]. / Sindrome da atresia delle vie biliari e malformazione splenica. Caso clinico.

Biliary atresia is a severe neonatal malformation in which the entire extrahepatic biliary tract or part of it is absent and replaced by fibrosclerotic tissue. Having been described for the first time by Thomson in 1891, biliary atresia has an incidence of 1:10,000-15,000 live neonates with a slight predominance in the female sex; it is associated with other malformations in 25% of cases. In 1993 Davenport et al. identified a subgroup of patients with biliary atresia who presented a splenic malformation. This condition was known as biliary atresia and splenic malformation syndrome (BASM). The cases of biliary atresia and BASM are still not clear and a number of etiopathogenetic hypotheses have been put forward. The treatment is surgical and includes a first stage of hepatic-porto-enteroanastomosis using a defunctionalised Roux loop (Kasal), followed by the definitive operation comprising liver transplant. We report the case of a boy who was brought to our attention with jaundice, polypnea and hepatosplenomegalia; instrumental tests allowed the diagnosis of biliary atresia, median liver, stomach and spleen on the right, polysplenia, atresia of the intrahepatic tract of the inferior vena cava, presence of a superior vena cava to the left that drained into the upper left portion of the common atrium. Moreover, the cardiac apex was present on the right, extensive DIA of the common atrium type, extensive DIV and right aortic arch.

Functional evaluation of tubularized-incised plate repair of midshaft-proximal hypospadias using uroflowmetry.

OBJECTIVE: To determine objectively, using uroflowmetry, the functional results of the tubularized-incised plate urethroplasty to repair midshaft-proximal hypospadias. PATIENTS AND METHODS: Twenty-one patients (mean age 4 years, mean follow-up 1.8 years) were selected from those undergoing surgery between January 1996 and January 1998 at our institution. All patients had midshaft-proximal hypospadias and were treated using the Snodgrass technique. Patients were included if they were able to void volitionally and had no fistula. The flow pattern, maximum (Qmax) and mean flow rate (Qave) were measured; the results were expressed as percentiles and compared with published values from normal children. The Qmax and Qave were considered normal if > 25th percentile, as equivocally obstructed when in the 5-25th percentile and obstructed if < 5th percentile. RESULTS: Fourteen patients were considered normal, four as equivocally obstructed and three as obstructed. Of the second group, one patient had a urethral diverticulum at the native meatus (confirmed by voiding cysto-urethrography) and the remaining three patients had meatal stenosis that responded to dilatation, with normal flow in two and improvement in the other. Of the obstructed group, one patient responded to dilatation and two underwent meatoplasty. CONCLUSION: The tubularized-incised plate repair provides satisfactory functional results for midshaft-proximal hypospadias; uroflowmetry is an important noninvasive tool to evaluate this technique. A long-term follow-up is needed to confirm these results.

[Value of uroflowmetry in a long-term follow-up study in hypospadias surgery]. / Il valore della uroflussometria nel follow-up a lungo termine della chirurgia dell'ipospadia.

BACKGROUND: Many authors have shown the role of uroflowmetry in the follow-up of patients operated on for hypospadias. This technique has also been used to assess the validity of the operative technique. METHODS: Sixty patients have been selected among those operated on for hypospadias from January 1990 till January 1996 at the Pediatric Surgery Department of the Second University of Naples, Italy. These patients underwent uroflowmetry with a rotating disk sensor at clinic. Postoperative time at uroflowmetry ranged between 6 months and 6 years. Flow pattern, maximum flow rate (Q max) and mean flow rate (Q ave) were measured. Results are expressed as percentiles and compared with those obtained from Toguri. Q max and Q ave greater than 25 degrees percentile were considered normal; Q max and Q ave between 25 degrees and 5 degrees percentile were considered query obstructed; Q max and Q ave below the 5 degrees percentile were considered obstructed. RESULTS: Forty-two patients (70%) presented values above the 25 degrees centile, 8 patients (13.4%) presented an obstructed-like flow and 10 children (16.6%) showed a query obstructed flow. Three patients from the latter group presented within three months an obstructed-like flow. CONCLUSIONS: Uroflowmetry is an important tool in the follow-up of patients operated on for hypospadias that are at risk for stenosis and then requiring a further treatment, either dilatation or surgery.

[Clinico-epidemiological considerations of 30 cases of pulmonitis and pleuropulmonitis in childhood]. / Considerazioni clinico-epidemiologiche su 30 casi di polmonite e pleuropolmonite in età pediatrica.

The authors studied 30 children affected with pneumonia or pleuropneumonia. They point out some clinical and epidemiological features pertinent to the pathology examined.

[The correlation between psittacosis-ornithosis and the immune status in childhood: apropos 5 cases]. / Correlazione fra psittacosi-ornitosi ed assetto immunitario in età infantile: a proposito di 5 casi.

The authors studied 5 children affected with a pneumopathy by Chlamydia psittaci. They discuss the effects of a transitory immunodeficiency relative to NK cells in predisposing little patients to the infective disease.