Congenital anomalies of the kidney and urinary tract in a cohort of 280 consecutive patients with Hirschsprung disease.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) have been underestimated in Hirschsprung disease (HSCR). This paper aims at reporting results of patients with HSCR who underwent kidney and urinary tract assessment. METHODS: Patients seen between December 2005 and November 2020 underwent a complete kidney and urinary tract diagnostic workup. Data regarding CAKUT, gender, length of aganglionosis, familial history, HSCR-associated enterocolitis (HAEC), RET genotype, and outcome were collected. RESULTS: Out of 472 patients, 280 completed the workup and represented the focus. Male to female ratio was 3.24:1. Familial cases accounted for 9.8% of patients. RET mutations were detected in 19.8%. We encountered a total of 61 patients with 70 nephrological issues (21.8%), including 28 hypoplasia/dysplasia, 12 hydronephrosis, 11 vesicoureteric reflux, 7 duplex collecting system, 2 kidney agenesis, 2 horseshoe kidney, and 8 miscellanea, involving 91 kidneys without side preponderance (50 right, 41 left). Of these 61 patients, 20 (7.1% of the whole series) required medical or surgical treatment. When comparing patients with and without CAKUT, familial history proved to occur with a significantly lower frequency in the former as did better patient perspectives of outcome. CONCLUSIONS: We confirmed that all diagnostic workups in HSCR should include a complete kidney and urinary tract diagnostic workup. Our study suggests that genes other than RET could play a role in determining CAKUT. Given worse patient perspectives of outcome, CAKUT seems to significantly interfere with quality of life thus confirming the need for early diagnosis and tailored prevention strategies.

The THUNDERBEAT system for tissue dissection and vascular control in laparoscopic splenectomy.

The advent of new energy sources for hemostasis has greatly facilitated advanced laparoscopic procedures. We describe a straightforward technique of laparoscopic splenectomy (LS) accomplished using the THUNDERBEAT™ system (TS) (Olympus Medical Systems Corp., Tokyo, Japan) as the sole means of tissue dissection and hemostasis in two patients aged 19 and 6 years, respectively. The specimens were removed intact via a Pfannenstiel incision. Total operative time was 165 and 150 min, and length of hospital stay was three and 4 d, respectively. The TS is an appealing and reliable alternative to currently available energy devices, allowing fast dissection and secure hemostasis during laparoscopic splenectomy.

Umbilical cord sparing technique for repair of congenital hernia into the cord and small omphalocele.

BACKGROUND/PURPOSE: Current repair of small omphaloceles and hernias into the umbilical cord is a straightforward procedure, whose repair may result in a suboptimal cosmetic outcome. We describe a novel repair technique retaining the umbilical cord elements in an attempt to improve the cosmetic appearance of the umbilicus. METHODS: Eight neonates were consecutively treated more than a ten-year period. Size of the fascial defects ranged 1 to 3cm (median, 2). Present technique entails incision of the amniotic sac without its detachment from the skin, reduction of the extruded contents under direct vision, and closure of the abdominal wall defect by circumferential suturing of peritoneum and fascia around the base of the amniotic sac. The amniotic sac is then re-approximated and folded to create an umbilical stump, which is trimmed and left to shed naturally. RESULTS: All patients achieved a scarless abdomen with a normal appearing umbilicus in 6. The remaining 2 patients are awaiting surgery for persisting umbilical hernia repair and umbilicoplasty, respectively. Poor esthetic outcome was significantly associated with initial fascial defect ≥2.5cm in size (p=0.03). CONCLUSIONS: Present technique is a simple and cosmetically appealing repair for umbilical cord hernias and small omphaloceles, especially effective when the size of the fascial defect is less than 2.5cm. LEVEL OF EVIDENCE: IV (Treatment Study).

Magnetic resonance imaging of epiploic appendagitis in children.

In children, epiploic appendagitis has been seldom reported. We describe two children with clinical presentations mimicking appendicitis. A correct diagnosis was eventually achieved by magnetic resonance imaging (MRI) and confirmed at laparoscopy in the initial case. Our preliminary experience suggests that MRI is a valid and non-invasive alternative to computed tomography for characterization of unusual causes of pediatric abdominal pain in the acute hospital care setting.