Spontaneous regression of suspected cystic dysplasia of the rete testis in three neonates.

INTRODUCTION: Cystic dysplasia of the rete testis (CDRT) is a rare cause of scrotal swelling during infancy. It is a benign lesion that is often associated with ipsilateral renal, ureteral or genital abnormalities. Leissring and Oppenheimer described it for the first time in 1973. CASE REPORTS: Since 2006, three neonates were referred to our pediatric surgery department because of a scrotal swelling related to intra-testicular cysts. Physical examination showed enlarged testicles without pain or inflammation. The ultrasound scan (USS) showed enlarged testicles related to multiple small cysts surrounded by normal parenchyma, and no other urinary abnormalities. As CDRT was suspected, a conservative strategy was proposed with repeated clinical examinations and USS. The cysts gradually regressed and disappeared in all cases, with a mean follow-up of 52 months. DISCUSSION: As reported by Jeyaratnam et al., mean age at presentation was around 6 years. The most frequently associated urinary abnormalities were renal agenesis and multicystic dysplasia of the kidney. Differential diagnosis of CDRT included single testicular cyst, epidermoid cyst, albugina cyst, teratoma, cystic lymphangioma, testicular juvenile granulosa cell tumor, cystic transformation after orchitis, or spermatic cord torsion. Ultrasonography was the method of choice for the diagnosis of CDRT and its follow-up. The USS showed multiple small cysts with normal, but compressed, surrounding testicular parenchyma. A high-frequency transducer was mandatory to exclude other causes of scrotal swelling. With the involution of the cysts, the aspect of the parenchyma could be slightly heterogeneous or confused with microlithiasis. Historically, orchidectomy was the proposed treatment. However, because of a more precise diagnosis with USS, a conservative approach has been proposed. Nonetheless, recurrence after sparing surgery was frequent, as reported by many authors. Non-surgical management was reported in selected cases, sometimes after a surgical biopsy to confirm a benign lesion. Spontaneous regression of CDRT without any sequelae has been reported in six cases (see Summary Table) but long-term follow-up in the adult population has never been published. Limitations of the present series were related to the small number of cases, the absence of follow-up beyond puberty, and the absence of biopsy to confirm the diagnosis of CDRT. CONCLUSION: Spontaneous regression of CDRT was possible, and conservative attitude was an option, as CDRT is a benign lesion. Careful and prolonged 'watch and wait' management was a safe alternative to surgery, but other causes of scrotal swelling must be excluded, possibly with a testicular biopsy.

[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

VAC® therapy a therapeutic alternative in giant omphalocele treatment: a multicenter study.

Giant omphalocele is associated to morbidity and mortality because of the strain the reintegrated herniated mass places on the hemodynamic equilibrium and breathing functions of affected infants. Currently, care management consists in a reintegration in one time or progressive reintegration. We report here a multicenter retrospective study about alternative management by VAC® therapy for giant omphaloceles. The study included three patients (1 girl, 2 boys) presenting with giant omphaloceles, born at full term in three different University Hospitals (prenatal diagnosis, normal karyotype). VAC® therapy was implemented at different times according to the cases (at Day 11, Month 1 and Month 5 after birth). The initial pressure applied was -10 mmHg progressively increased to -50 mmHg. A middle size VAC GranuFoam Silver® Dressing was used in all cases. Wound healing occurred at Month 4 for the first case, Month 6 and Month 8 for the other two. VAC® therapy is a good alternative for the care management of giant omphaloceles with more advantages especially when using prosthetic material. We also aimed at refining the most adapted indications in these specific situations, and finally we envisioned a harmonization of care for these children.

Retroperitoneoscopic vs open dismembered pyeloplasty for ureteropelvic junction obstruction in children.

PURPOSE: To compare the effectiveness, potential advantages and complications of classical open pyeloplasty with retroperitoneoscopic pyeloplasty in children. MATERIALS AND METHODS: Two patient cohorts with confirmed ureteropelvic junction obstruction (UPJO) undergoing open or retroperitoneoscopic pyeloplasty over a 7-year period were analysed comparatively. RESULTS: Operative time was significantly longer in the retroperitoneoscopic group (mean 155 min) compared to the open pyeloplasty group (mean 98 min, P<0.05). Mean hospital stay was shorter in the retroperitoneoscopic group (mean 4.1 days, compared to 5.1 days, open). Complication rates were similar (open, 27% vs retroperitoneoscopic, 29%). These included anastomotic urinary leakage, stenosis and infection. Anastomotic leakage was more common in the retroperitoneoscopic group. There was a 6.6% conversion rate in the retroperitoneoscopic group. Success, defined as improved ultrasonic or renographic parameters, with resolution of symptoms where discernable, was noted in 96% of the open group and 97% of the retroperitoneoscopic group with a mean follow up of 38 and 25 months, respectively. CONCLUSIONS: Retroperitoneoscopic pyeloplasty is as safe and effective as open pyeloplasty. This technique is now our procedure of choice for children>4 months old. The advantages are more obvious in children over 4 years than in infants. This technique remains difficult to perform and teach.