Lorenzo Nanni

Varicocele in pediatric patients: comparative assessment of different therapeutic approaches.

OBJECTIVES The best therapeutic approach for varicocele correction in pediatric patients is still an object of some debate. We analyzed a series of 99 patients to identify the most effective approach in terms of low recurrence rates and preservation of testicular growth. METHODS One hundred four operations were performed on 99 patients between 9 and 16 years of age (mean 13.3). The first 18 patients underwent sclerotherapy of the internal spermatic vein. Twenty others underwent inguinal varicocelectomy. In 12 patients, a modified Palomo procedure (ie, sparing of the internal spermatic artery) was performed (four laparoscopically), and 54 were treated with the original Palomo procedure (ie, sectioning of the entire spermatic cord), using laparoscopy in 20. RESULTS Three recurrences (16.6%) occurred among the patients treated with sclerotherapy. Similar rates of recurrence were observed in those who underwent inguinal varicocelectomy (3 [15%] of 20) and modified Palomo procedures (2 [16.6%] of 12). Only one recurrence occurred in the 54 patients treated with the original Palomo procedure (1.85%). CONCLUSIONS Our experience, together with a review of published reports, leads us to believe that the open Palomo procedure as originally described is the most effective approach to the correction of varicoceles in adolescents.

Mortality and long term morbidity in esophageal atresia: the reduced impact of low birth weight and maturity on surgical outcome

Abstract Progress in perinatal and postoperative techniques has reduced the prognostic role of traditional risk factors in esophageal atresia (EA). This paper reports on 75 cases of esophageal anomalies observed between 1992 and 2002 and followed after surgery from a minimum of six months to a maximum of ten years (mean five years). The impact on survival of birth weight, week of delivery, associated anomalies and need of ventilatory support at birth are discussed. Twenty-four patients were born before 37 weeks of gestation, 18 weighed less than 2000 g.; major anomalies affected 11 neonates, 23 cases required mechanical ventilation at birth. Seventy-four patients were operated on with a 90.6% survival rate; no deaths were related to surgical treatment. Three cases required reoperation for postoperative complications. Birth weight and week of delivery did not seem to influence outcome; this is affected by severe associated cardiovascular anomalies and the need of ventilation at birth. Follow up at 24 months on 51 patients, revealed respiratory problems in 12 cases and severe gastro-esophageal reflux in 16. This affected quality of life of EA patients and required long term medical attention; improvement with growth was observed. No correlation between perinatal conditions and late sequelae could be demonstrated in our series.

FREE PERITONEAL GRAFTS FOR PATCH URETHROPLASTY IN MALE RABBITS

PURPOSE We evaluated the feasibility of urethroplasty using a free peritoneal graft in a rabbit model. MATERIALS AND METHODS In 12 male rabbits a urethral defect was created by excising a 3 x 5 mm. portion of the ventral urethral surface. The defect was immediately repaired with a free peritoneal graft harvested via a left flank incision. RESULTS There were 2 intraoperative deaths and 1 death on day 8 postoperatively. These animals were excluded from study. The remaining 1, 4 and 4 rabbits were sacrificed 3, 5 and 9 weeks after surgery, respectively. Macroscopic examination of the urethra revealed no stenosis or diverticula, while a fistula was present in 3 of the 9 animals. Histological study of the fistulous tracts showed chronic granulomatous inflammation. In the remaining 6 rabbits there was graft adherence. The inner surface of the graft was uniformly lined with normal urothelium, while in some cases acute inflammatory cells were present in the subepithelial layer. CONCLUSIONS Our experience indicates that the ease of harvesting free peritoneal grafts and their satisfactory adherence to the urethra makes them a valid alternative for repairing urethral defects.

Solitary Peutz-Jeghers Polyp in a Paediatric Patient

Hamartomatous polyps of Peutz-Jeghers are mostly found in patients affected by Peutz-Jeghers syndrome (PJS), but they can be rarely encountered in the general population. It is unclear whether a solitary Peutz-Jeghers polyp (PJP) is an incomplete form of PJS or a separate entity. We report a case of solitary PJP in a paediatric patient in whom the other features of PJS were absent. The patient underwent laparotomy due to small bowel intussusception secondary to an ileac polyp. Histological examination showed the characteristic features of PJP, but the patient did not fulfill the WHO criteria for PJS diagnosis (negative family history for PJS and absence of mucocutaneous pigmentation); moreover analysis of the STK11/LKB1 gene did not reveal any genomic abnormality. The clinical and investigative findings in our case suggest that the solitary PJP can be considered a different clinical entity from PJS.

Chromosome 9p deletion syndrome and sex reversal: novel findings and redefinition of the critically deleted regions

Deletions of the short arm of chromosome 9 are associated with two distinct clinical entities. Small telomeric 9p24.3 deletions cause genital anomalies in male subjects, ranging from disorder of gonadal sex to genital differentiation anomalies, while large terminal or interstitial deletions result in 9p‐malformation syndrome phenotype. The critical region for non‐syndromic 46,XY sex reversal was assigned to a 1 Mb interval of chromosome 9p, extending from the telomere to the DMRT genes cluster. The 9p‐syndrome was assigned to bands 9p22.3p24.1, but a phenotypic map has not been established for this condition, probably because of the lack of detailed molecular and/or phenotypic characterization, as well as frequent involvement of additional chromosome rearrangements. Here, we describe a unique patient with a small isolated 9p terminal deletion, characterized by array‐CGH and FISH, who shows a complex phenotype with multiple physical anomalies, resembling the 9p‐syndrome, disorder of sex development with gonadoblastoma, congenital heart defect and epilepsy. The observed deletion includes the 46,XY sex‐reversal critical region, excluding the region so far associated with the 9p‐syndrome. Genotype–phenotype correlations are tentatively established comparing our patient to seven other previously reported males with isolated terminal 9p deletions, finely defined at a molecular level. Our observations expand the 9p deletion clinical spectrum, and add significantly to the definition of a 9p‐syndrome critical region.

Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2

Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus.

Observational retrospective study on acquired megalourethra after primary proximal hypospadias repair and its recurrence after tapering.

INTRODUCTION Acquired megalourethra (AMU) after repair of proximal hypospadias can be a serious complication. An observational retrospective study of its incidence among different types of repair was performed. MATERIALS AND METHODS Clinical charts of patients operated on for proximal hypospadias were reviewed. INCLUSION CRITERIA all primary hypospadias operated in 1991-2004, with the meatus positioned in proximal penile, scrotal or perineal position. RESULTS Of 770 hypospadias cases treated, 130 (16%) were proximal. Seventy-two patients (55%) were treated using preputial flaps: 36 with a tubularized preputial island flap (TIF) and 36 an onlay island flap (OIF). Fifty-eight patients (45%) underwent staged repairs: Belt-Fuquà (BF) in 18 and Bracka procedure in 40 cases. After a mean follow up of 16 years (range 6-19) the overall incidence of complications for each technique was: TIF 36%; OIF 33%; BF 25%; two-stage Bracka 7.5%. The most common complication encountered was neo-urethral fistula. AMU occurred in only 5 cases, none with associated distal urethral stenosis, all in the TIF and OIF groups, and all successfully treated by reduction re-do urethroplasty. CONCLUSION A very small number of the patients operated using preputial island flaps techniques developed AMU. None of the staged repairs developed AMU, and this is the preferred choice in proximal hypospadias when the urethral plate requires division and/or substitution. All cases of AMU resolved after urethral tapering.

Treatment of intraosseous arteriovenous fistulas of the extremities

Intraosseous arteriovenous fistulas of the extremities are rare malformations frequently associated with severe systemic hemodynamic alterations. In many cases, it is quite difficult to eliminate these anomalous vascular structures, but the possibilities for successful treatment are much greater when surgery is combined with interventional radiology. Selective embolization of the malformed vessels can be produced with a variety of agents that are injected into afferent arteries, via percutaneous puncture or through direct surgical access. The intraosseous portion of the fistula should be resected at the time of embolization or later. The authors describe the successful treatment of three patients having intraosseous arteriovenous fistulas of the upper extremities, who have had follow-up for 2 to 10 years.

Gastrostomy placement in children: Percutaneous endoscopic gastrostomy or laparoscopic gastrostomy?

The aim of this study is to compare the outcomes and the complications between the 2 most adopted procedures for gastrostomy placement: percutaneous endoscopic gastrostomy (PEG) and laparoscopic gastrostomy (LG) in children. We present our study on 69 patients (male: 46/female: 23): group 1 (37 patients, 54%) undergoing PEG, group 2 (32 patients, 46%) undergoing LG. A total of 5 major complications were observed all in the PEG group (13.5%), no major complication was observed in the LG group (P-value<0.05). A total of 12 minor complications were observed: 4 occurred in the PEG group (10.8%) and 8 (25%) in the laparoscopic gastrostmoy group, not statistically relevant. We suggest that the LG should be considered the preferred technique for gastrostomy placement in pediatric patients, particularly in newborns, children with significant skeletal malformations, and patients who underwent previous abdominal surgery.

An unusual case of acute pancreatitis and gastric outlet obstruction associated with wandering spleen treated by laparoscopic splenopexy.

Wandering spleen (WS) is an uncommon condition, usually asymptomatic, often recognized as an incidental finding. When symptoms occur, they can vary, although acute abdominal pain is the most common presentation in the pediatric population. In some cases, WS can become a dangerous condition because of the risk of splenic ischemia from persistent pedicle torsion. We describe a case of WS in a 3-year-old boy presenting with vomiting, abdominal swelling, and acute pancreatitis; the diagnosis was obtained by ultrasound and computed tomography. Laparoscopic splenopexy was successfully performed through an extraperitoneal pocket and a Vicryl mesh. To the best of our knowledge, the combination of gastric outlet obstruction and acute pancreatitis has never been reported as presenting symptoms of WS.