Traumatic duodenal lesions in children due to two-point seat-belt: the seat-belt syndrome.

Two consecutive children, motor vehicle accident victims, were treated between December 2006 and January 2007. They reported duodenal perforations due to lap-belt action. In the first case (a 9-year-old boy), symptoms became immediately evident after the trauma. Early computerized tomography (CT) scan demonstrated biliaryhemoperitonitis and free sub-diaphragmatic air. At the laparotomy a complete duodenal transection, at the level of duodenal bulb, was found. In the second patient, early X-ray and CT scan revealed no free abdominal air; only the second CT scan, repeated after 48 hours, when general conditions of the girl become critical, demonstrated biliary peritonitis and aerial extraluminal gas image in the right peri-kidney space. At the surgical exploration a duodenal laceration was noted, at the passage between the second and the third duodenal portion, in the postero-lateral wall, with an extension of 6 cm. In both children direct suture of duodenal injury without stoma construction was performed. Not early or late postoperative complications were registered (follow-up: 18 months). In the traumatic duodenal lesions an early diagnosis is not always possible because of the paucity of the clinical signs. Tightened clinical and radiological monitoring of the patient is fundamental in the successive hours to the trauma. Early diagnosis and high level of suspicious rest crucial for better prognosis in this group of patients.

Phenotypic variability of Pai syndrome: report of two patients and review of the literature.

Pai syndrome is a rare form of frontonasal dysplasia, first described in 1987. It is a triad consisting of midline cleft of the upper lip, facial skin polyps and central nervous system lipomas. Only 14 cases have been reported in the literature. The authors describe the clinical features, diagnostic workup and treatment of two patients. A review of all cases reported in literature is presented to show the phenotypic variability of this rare syndrome.

Congenital diaphragmatic hernia in CHARGE syndrome.

Congenital diaphragmatic hernia (CDH) has been rarely described in CHARGE syndrome. We report a patient affected by CHARGE syndrome presenting with a right-sided Bochdalek-type diaphragmatic hernia, and collect the pertinent literature. Furthermore, we review the embryogenesis of the diaphragm and the pathogenesis of CDH to highlight if this malformation could be explained by a developmental anomaly of CHARGE. On the basis of our study, we suggest that patients affected by CDH, facial asymmetry and cardiovascular or urogenital malformations, should be actively screened for CHARGE syndrome findings.

The lazy bladder syndrome: a possible urodynamic evolution in patients with idiopatic detrusor and pelvic floor overactivity.

OBJECTIVE: To correlate the urge syndrome due to bladder overactivity and the lazy bladder syndrome, demonstrating that, at least in some cases, the lazy bladder may be the final stage of the evolution of an overactive bladder when associated with overactivity of the pelvic floor during micturition. PATIENTS AND METHODS: From January 1998 to December 1999, 38 children, 30 females and 8 males, 5 to 16 yrs. old (median 7.4 yrs), presenting with urge symptoms and never treated before, have been evaluated with repeated urodynamic investigations. At presentation all the patients underwent complete baseline urodynamics including evaluation of free flow, EMG, cystometrogram, subtracted detrusor pressure and flow/pressure studies. Flow/EMG was repeated every four months during the treatment period and full urodynamic investigation every year. RESULTS: The first urodynamic study showed that 17 patients were affected by pure detrusor overactivity with good detrusor-sphincter co-ordination, while 21 presented both detrusor and pelvic floor muscles overactivity. All the patients have been treated with oxibutinin (0.3-0.5 mg/Kg. in 3 divided doses) and a timed voiding program with the help of a frequency-volume chart. At the end of the study 16 out of the 17 children with pure overactive bladder were cured (94%), while only 12 of the 21 patients with both bladder and sphincter overactivity were clinically and urodinamically normal Nine girls showed a progressive shift towards the development of a lazy bladder syndrome (capacious, hypocontractile bladder with large post-voiding residual and a non relaxing sphincter during micturition). Clinically this shift was signalled by recurrent urinary tract infections. CONCLUSIONS: The transition from an overactive bladder to a hypocontractile one has been reported previously, but it has not been extensively investigated in children and its causes are largely hypothetical. In our cases this phenomenon occurred only in girls that, initially showed both detrusor urethral sphincter overactivity, therefore we can reasonably conceive that, at least in some cases, the lazy bladder syndrome may be due to prolonged and repeated high pressure bladder contractions opposed by a non relaxing sphincter that may lead to a progressive loss of the contractile properties of detrusor muscle fibres. The rather rapid shift observed in our patients might have been favoured by the use of anticholinergic medications.

Testis sparing enucleation of a Leydig-cell tumour in a boy.

Testicular tumours are very rare in paediatric age, accounting only for 1% of all paediatric tumours. Testicular tumours can originate either from germ cells (77.4%) or from stromal cells (7.1%) or from other cells. Leydig-cell tumours account for 1% of all testicular tumours and 39% of gonadal stromal tumours and in the prepubertal male are responsible for causing precocious pseudopuberty. In the past, orchiectomy has been considered the treatment of choice, but in consideration of the fact that Leydig Cell tumours in children invariably show a benign behaviour, in recent years some authors have suggested a more conservative approach. In the herein reported case, a decision was made to simply enucleate the tumour leaving the testis. After one year, imaging shows a normal testis with no sign of recurrence.

[Primary megaureter detected during the first year of life. Review of case reports in the last 10 years and analysis of prognostic factors]. / Megauretere primitivo diagnosticato nel primo anno di vita. Revisione della casistica degli ultimi 10 anni ed analisi dei fattori prognostici.

AIM: We report our experience in the treatment of primary megaureter discovered prenatally or in the first year of life. We also discuss the prognostic role of the first diuretic renogram performed immediately after the discovery of the megaureter. MATERIAL AND METHODS: 45 patients have been observed since January 1990 until December 1999, with 54 primary non refluxing megaureters (9 bilateral cases); 18 discovered prenatally; 24 detected during a postnatal echographic screening of urinary malformations; and 3 symptomatic. All the patients have been studied and followed-up with repeated sonograms and diuretic nephrograhis. RESULTS: 19 patients underwent surgery, 5 at presentation and 14 early during follow up. In this group, 7 patients were operated on because of urinary tract infection, 5 had a functional impairment of the involved kidney and 2 presented a progressive increase of ureteral size. 29 megaureters have been followed non operatively, of these, 6 resolved and 19 improved dilatation, 10 were unchanged and 3 cases underwent progressive worsening. Half time clearance (T 1/2) calculated during the first diuretic renogram had a favorable prognostic significance when the value was under 10', but when the value was above 10' the evolution was unpredictable.

Acute epididymitis and urinary tract anomalies in children.

OBJECTIVES: To evaluate the incidence of urinary tract infection (UTI) and genitourinary malformations in children presenting with acute epididymitis. PATIENTS AND METHODS: Twenty-five children between 2 months and 14 years of age presenting with acute epididymitis underwent urine culture, abdominal ultrasound, voiding cystourethrography and, in selected cases, intravenous pyelography. Eleven patients were infants and 14 were older than 1 year. UTIs and genitourinary malformations were recorded. Fisher's exact test was used to compare the incidence in two age groups. A p value of less than 0.05 was considered significant. RESULTS: Seven UTIs and eight genitourinary malformations were diagnosed in infants, while in older children three UTIs and three malformations were discovered. Infants had a higher rate of associated UTI (p 0.049) and genitourinary malformations (p 0.017) than did older children. CONCLUSIONS: Our data show a close relationship between acute epididymitis in infants for both UTIs and genitourinary malformations. Every infant presenting with epididymitis would undergo a complete evaluation including abdominal ultrasound and voiding cystourethrography. In older children the need for imaging should be dictated by clinical history and physical findings.

[Acute epididymitis in children]. / Le epididimiti acute in età pediatrica.

Fifteen cases of epididymitis in children are reviewed, six of these occurring in children under twelve months of age, and nine in older children. In five of the six cases occurring in infants a U.T.I. and a malformation of the urinary tract were found, while in children over one year only three infections and two urinary malformations were discovered. Authors recommend that in infants under 1 year presenting with epididymitis a thorough investigation of the urinary tract is performed.

[Simple testicular cysts in children. Presentation of a case]. / Cisti testicolare semplice in età pediatrica. Presentazione di un caso.

A case of simple intraparenchymal testicular cyst in a two and half months old child is reported. Dysplastic cysts of the testicle are exceedingly rare, in children only eight such cases have been described in the Literature, all, except one, treated by orchiectomy. In the herein reported case a conservative approach with cyst enucleation and sparing of the residual testicular parenchyma has been satisfactorily employed.

[Scaphoid megalourethra. Description of a case and review of the literature]. / Megalouretra scafoide. Descrizione di un caso e revisione della letteratura.

Authors describe a case of scaphoid megalourethra in a newborn. The child underwent a successful urethroplasty in neonatal period, as described by Nesbitt. In a careful review of the Literature only 47 such cases were found (included the one herein described). Authors discuss etiology, diagnosis and management of this rare malformation and stress the feasibility of an early reconstructive procedure.

[Conservative treatment of esophageal perforation in the newborn infant. Description of a case]. / Trattamento conservativo delle perforazioni esofagee nel neonato. Descrizione di un caso.

A case of oesophageal perforation in a newborn is reported. Authors describe the different clinical pictures due to the various sites of perforation. Authors believe that conservative treatment is satisfactory when applied to most oesophageal perforations and operative approach should be employed only in selected cases and after failure of a trial of non-operative treatment.

[Fibrous polyp of the posterior urethra]. / Polipo fibroso dell'uretra posteriore.

Authors describe a case of fibrous polyp of the posterior urethra, causing complete urinary retention in a five and a half years old boy. On the basis of their own experience and of the data of the Literature Authors discuss etiology, diagnosis and management of this rare anomaly of the urinary tract.

[A case of ectopic vas deferens opening into the bladder]. / Un caso di sbocco ectopico del dotto deferente in vescica.

A case of epididymitis in a two years old boy owing to an ectopic vas deferens opening into the bladder is reported. Only a very few similar cases have been recorded in the world literature.

[Congenital anterior urethral fistulas (author's transl)]. / Le fistole congenite dell'uretra anteriore.

Authors report a case of congenital urethral diverticulum which, in the first days of life, spontaneously fistulized. On the basis of the data reported in the Literature and of their own experience they discuss the etiopathogenesis of these rare types of urethral fistulas.