High-pressure balloon dilatation for the treatment of primary obstructive megaureter: is it the first line of treatment in children and infants?

AIMS OF THE STUDY: To evaluate the efficacy of high-pressure balloon dilatation (HPBD) as treatment of primary obstructive megaureter (POM) in paediatric patients, we analysed the data of our institute from June 2018 to September 2019. METHODS: 14 patients, aged 5 months to 5 years, with POM were treated with HPBD. All patients had a distal ureter dilatation greater than 7 mm associated with obstructive features on a mercaptoacetyl triglycine-3 diuretic renogram scan, and a voiding cystourethrogram without vesicoureteral reflux. HPBD was performed in 12 patients, whereas 2 patients (14%), aged 5 and 6 months, required open surgical treatment because of failure to pass the balloon catheter through the vesicoureteral junction. The procedure was performed with a 5 Fr balloon catheter for two cycles of 5 minutes each at 17 atm. A double-J stent and a urinary catheter were inserted at the end of procedure in all patients. RESULTS: No operative complications or symptoms or recurrence were recorded in our series. The patients were generally discharged 24 hours after surgery. All the patients showed an improvement on ultrasonography at the postoperative follow-up, with no evidence of obstruction. During the procedure a clear stenotic ring was identified in 10 of the 12 patients, which disappeared in all 10 cases after the HPBD technique. CONCLUSIONS: Based on our experience, HPBD may be considered the first-line surgical approach in the treatment of POM in children, avoiding bladder surgery in most cases.

Posterior Urethral Polyp Treated With Endoscopic Transurethral Resection: The Second Pediatric Case Managed With Holmium Laser.

Fibroepithelial urethral polyp is a rare benign tumor more common in children than adult that can cause bladder outlet obstruction. We describe a 3-year-old boy who presented with intermittent acute urinary retention affected by a fibroepithelial urethral polyp managed with endoscopic transurethral resection by HOLMIUM laser; no complications neither recurrence were observed. To our knowledge this is the second pediatric case of fibroepithelial urethral polyp treated by HOLMIUM laser.

Combined capillary-venous-lymphatic malformations without overgrowth in patients with Klippel-Trénaunay syndrome.

OBJECTIVE: Klippel-Trénaunay syndrome (KTS) is described in the literature as a complex syndrome characterized by various combinations of capillary, venous, and lymphatic malformations associated with limb overgrowth. In the first description by Maurice Klippel and Paul Trénaunay, tridimensional bone hypertrophy was believed to be the cause of limb enlargement. The purpose of this study was primarily to assess the presence of real bone hypertrophy as a cause of enlargement of the limb and to underline the rare presence of undergrowth of the affected limb in patients with KTS. METHODS: A two-center retrospective review including 17 KTS patients with various combinations of capillary, venous, and lymphatic malformation affecting the lower limb was performed. Differences in limb dimension were evaluated clinically. Width and length discrepancy of the affected limb was measured with radiologic imaging. RESULTS: We found an increase of length in the affected limb in 80% of the patients. The leg length discrepancy varied from 0.2 to 2.6 cm. The median leg length discrepancy was found to be 1.4 cm. Three patients had a reduced length of the affected limb. Girth enlargement of the affected extremity was noticed in 60% of the patients, and 2 of 17 patients had hypotrophy of the involved limb. Hypertrophy (an increase in both length and width) of the bone was found in none of our cases, and the circumferential enlargement of the affected extremity was related only to soft tissue enlargement. CONCLUSIONS: In the literature, KTS is considered the prototype of overgrowth syndromes associated with complex vascular malformations. The majority of our patients showed limb length increase associated with soft tissue enlargement without an increase of bone width; there were also two patients with limb undergrowth. A real bone overgrowth (an increase in both length and width) was not present in our patients. Therefore, we could consider the absence of real bone hypertrophy as probably a new aspect of such confusing and controversial definitions of KTS. In addition, it would be more accurate to classify KTS patients on the basis of their phenotypic features (type of vascular malformation, types of overgrown tissue) rather than by use of an outdated eponym.

Tracheal and bronchial tumors.

Although primary tracheobronchial tumors are extremely rare in children, recurrent respiratory symptoms resistant to conventional therapy require further investigations to exclude possible malignant obstructive causes. As the matter of fact, early diagnosis may allow minimally invasive surgeries, improving the standard of living and the globally survival rate. The aim of this article is to provide an overview of diagnosis and management of tracheobronchial tumors in the early age, since only few reports are reported in the worldwide literature.

Rare case of an adrenocortical neoplasm: A case report and review of literature.

Adrenocortical neoplasms (ACNs) are rare and poorly characterized in infants. The true incidence of ACNs is not well known and it appears to vary substantially across different geographical areas. ACNs are more common in females and two peaks of incidence have been identified: The first year of life and between the age of nine and 16 years. Due to the heterogeneity and rarity of ACNs, their pathological and prognostic classification is challenging. The current study describes the case of a seven-year-old male, who presented to the Department of Pediatric Surgery, University of Siena (Siena, Italy) with a feminization syndrome and increased somatic growth that was associated with a unilateral adrenal mass, which was diagnosed by magnetic resonance imaging. Surgical excision of the mass was performed and histological analysis determined that it was an ACN, with a low risk of malignity; however, the pathological classification of the tumor was challenging. At present, the future behavior of ACNs is unpredictable. Therefore, increasing the knowledge surrounding this type of tumor may aid in its diagnosis, treatment and prognosis. Due to the rarity of pediatric ACNs, no single pediatric oncology center has acquired extensive experience treating this type of tumor. Thus, the initiation of an international tumor registry may aid with the management of patients presenting with ACNs.

Chromosome 18q-syndrome and 1p terminal duplication in a patient with bilateral vesico-ureteral reflux: case report and literature revision.

BACKGROUND: Vesico-ureteral reflux (VUR) is a dynamic event in which a retrograde flow of urine is present into the upper tracts. VUR may occur isolated or in association with other congenital abnormalities or as part of syndromic entities. We present a patient with a bilateral primary VUR, syndromic disease caused by a large deletion of 18q (18q21.3-qter) and terminal duplication of 1p (1p36.32-p36.33). CASE REPORT: The patient was 8 years old female with a disease including moderate growth retardation, psychomotor retardation, facial dysmorphism, single umbilical artery, umbilical hernia, urachal remnant, bilateral congenital clubfeet and renal-urinary disease. Chromosomal analysis and Array-CGH revealed two heterozygous chromosomal rearrangements: 1p terminal duplication and de novo 18q terminal deletion. She referred to our clinic to evaluation of bilateral hydronephrosis and right renal cortex thinning. Voiding cystourethrography demonstrated bilateral grade IV VUR and dimercaptosuccinic acid renal scintigraphy confirmed right renal cortex thinning and showed a cortical uptake of 75% of the left kidney and 25% of the right kidney. The patient underwent ureterovesical reimplantation after failure of 3 endoscopic submeatal Deflux injections with VUR resolution. CONCLUSIONS: This is the first report involving a patient with 18q-syndrome and contemporary presence of 1p chromosomal terminal duplication. The coexistence of two chromosomal rearrangements complicates the clinical picture and creates a chimeric disorder (marked by characteristics of both chromosomal anomalies). Kidney problems, primarily VUR is reported in 15% of patients affected by 18-q syndrome and no cases is reported in the literature regarding a correlation between VUR and 1p36 chromosomal duplication.

Pain and anxiety management in minimally invasive repair of pectus excavatum.

The Nuss procedure for the correction of Pectus Excavatum (PE) is associated with intense postoperative pain. Our strategy to control early postoperative pain is to combine epidural with intravenous analgesia. Our aim was to analyse our pain control strategy by reviewing all the PE cases treated at our institution. Sixty consecutive patients, aged between 12 and 26 years old, received the PE operation at our institution from January, 2007 to September, 2010. The median age was 16 (12-27) with a male/female ratio of about 7/1. An epidural catheter was employed in all the cases, with 38 patients (63%) requiring additional drugs to control pain, which remained in place for 74 hours (72-96). The pain score was higher in male patients, but lower in those younger than 16 years old. Moreover, patients that consumed benzodiazepines had a significant decrease in cumulative opioid intake (P = 0.0408). Both gender and age had an impact on pain control, while we noticed a synergistic effect between opiates and tranquillizers.