G. Di Maggio

Voluminous perinatal pelvic mass: a case of congenital hydrometrocolpos

Imperforate hymen is the most frequent congenital malformation of the female genital tract; it usually does not show symptoms until puberty. Only rarely, imperforate hymen manifests itself as an abdominal mass detectable in the prenatal period. We describe a rare case of voluminous hydrometrocolpos, antenatally diagnosed and successfully treated immediately after birth.

Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula

Abstract Bronchopulmonary malformations associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) are extremely rare. The authors describe a case of type II congenital cystic adenomatoid malformation (CCAM) of the right lower lobe associated with EA and TEF (Vogt-Gross type C) in a full-term female infant. The CCAM presented as an incidental radiologic finding, and a contralateral tension pneumothorax developed shortly after surgical repair of the EA. Early recognition of this rare association is essential for correct operative management.

Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy

BACKGROUNDnBladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control.nnnSTUDY DESIGNnFourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5-15xa0ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies.nnnRESULTSnTen children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were also continent, were excluded because of a <5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2-4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong proctoscopy and biopsy protocol.nnnDISCUSSIONnThe ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem.nnnCONCLUSIONnThese significant benefits, at a crucial time in the childs life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with long-term proctoscopic follow-up.

242 Spontaneous Idiopatic Intestinal Perforation (SIPI): A Distinct Clinical Entity in the Preterm Infant

Background: The most common cause of intestinal perforation in newborns is necrotizing enterocolytis (NEC). A novel condition has been increasingly described of idiopathic spontaneous intestinal perforation (SIPI), which does not show the typical clinical and diagnostic features of NEC. The aim of the present study is to define clinical peculiarities and potential risk factors in newborns by comparing SIPI and NEC patients.Methods: In the last 4 years 85 preterm infants necessitated surgical counselling for acute abdominal conditions. Among them, 13 underwent emergency surgery, 6 for SIPI and 7 for NEC respectively. Patients were then subdivided into 3 groups: Group 0, 72 infants, who did not need surgery; Group I,6 patients with SIPI; Group II 7 patients with NEC. Some variables were retrospectively analyzed and were compared by means of CHI 2 test with a significative value of p<0.05.Results: No association emergerd between patients sex gender (p=0.691), membrane ruptures (p=0.400) and maternal infection (p=0.415). A correlation was observed between pneumoperitoneum and SIPI/NEC (p=0.000). The study on Group I and II showes a lower gestational age (27.3 as compared to 30.28 weeks) a higher number of membrane ruptures (4/6 as compared to 3/7) and of maternal infections (4/6 as compared to 3/7). Comparative analysis showed that patients under 28 weeks of gestetional age have a 17 folds higher risk of SIPI (96%, p=0.001) as compared to patients with older gestational age.Conclusions: Our study shows that a determining factor for the etiopathogenesis of SIPI may be neonatal stress consequent to preterm birth. Infants under 28 weeks of gestational age, with low birth weight (<1.200 g) show a particular predisposition to SIPI, with a risk accounting to 96% of our case series.