Inguinal hernia in neonates and ex-preterm: complications, timing and need for routine contralateral exploration.

BACKGROUND/PURPOSE: Aim of this study was to present a series of neonates and ex-preterm babies who underwent inguinal hernia repair focusing on complications and possible indication to perform routine contralateral groin exploration. METHODS: This is a retrospective study of a series of consecutive patients weighing less than 5 kg who underwent inguinal hernia repair between January 2007 and December 2012. Only the affected side was treated. Patients have been routinely followed up postoperatively. We resorted to available outpatients' charts and admission notes to record demographic data, surgical details, complications and the occurrence of metachronous hernias. A questionnaire was administered to all patients' relatives to confirm the long-term outcome. RESULTS: One hundred fifty-four patients were operated for a total of 184 herniotomies (88 right sided, 36 left sided and 30 bilateral). Median length of follow-up was 42 months (range 6 months-7.5 years). Thirteen patients (13/124 = 10.5 %) developed metachronous hernia that proved to be significantly more frequent in patients weighing less than 1,500 g at birth (p < 0.05). We observed 10 % of complications, including 2.7 % testicular atrophy and 4.5 % recurrence. Atrophy proved to occur more frequently in patients who experienced preoperative incarceration (p < 0.05). No other risk factors were identified. CONCLUSIONS: The results of our series demonstrated that, though technically demanding, herniotomy in the neonate and ex-preterm is associated with a relatively low incidence of complications. Based on our results and in accordance with literature data, we do not advocate routine contralateral exploration in case of unilateral hernia but surgery to be performed only on the symptomatic side, as soon as possible after initial diagnosis. Very low birth weight patients should be followed with care in the early postoperative period due to the higher likelihood of developing a metachronous hernia.

Megacystis, megacolon, and malrotation: a new syndromic association?

Chronic intestinal pseudo-obstruction (CIPO) can occur as a consequence of neuropathies including diffuse Intestinal Neuronal Dysplasia (IND), a relatively rare enteric nervous system (ENS) abnormality. Although various authors reported of diffuse IND associated either with intestinal malrotation or megacystis, the co-existence of these three entities in the same patient has never been described before. The aim of this paper is to report for the first time in literature a series of patient with such association, focusing on one who carries a de novo duplication of chromosome 12, suggesting a new syndromic association (megacolon, megacystis, malrotation).

Shifting from open surgical cut down to ultrasound-guided percutaneous central venous catheterization in children: learning curve and related complications.

PURPOSE: Tunneled indwelling central venous catheters (CVC) are essential in the management of children with cancer, hematological, nephrological disorders and for parenteral nutrition. The aim of this study is to present the experience of a single center of the transition from traditional open surgical cut down procedure (OSC) to ultrasound (US)-guided percutaneous CVC insertion, focusing on learning curve and related complications. METHODS: All CVCs inserted between April 2008 and November 2009 in children at the Gaslini Children Hospital were revised, and data on methods of cannulation, intraoperative and device-related complications and re-intervention were recorded. RESULTS: 194 CVCs were positioned in 188 patients. 128 out of 194 CVCs were positioned through an OSC technique, whereas the remaining 66 CVCs were inserted percutaneously with US guidance. Of the 27 recorded complications, 15 were mechanical events, 7 cases developed infection, whereas the remaining 5 (2.6%) were classified as intraoperative complications. A second surgical procedure was described in 23 (11.8%) cases. CONCLUSION: Shifting from OSC to US-guided percutaneous CVC insertion inevitably involves a challenging learning curve which is generally associated with high complication rates. Complications progressively decrease once a good experience in US guidance and percutaneous technique has been obtained.

Minimally-invasive surgery in paediatric oncology: proposal of recommendations.

The Authors report a proposal of recommendations concerning Minimally Invasive Surgery (MIS) in Paediatric Oncology. Since the exact role of MIS in Paediatric Oncology is still not completely defined, a restrict panel of Italian Paediatric Surgeons, some interested in Oncologic Surgery, others in MIS, prepared a schematic document, mainly founded on literature data, to provide Paediatric Surgeons with recommendations useful to approach paediatric tumours with MIS. The final draft was approved by the Italian Group of Paediatric Oncologic Surgeons. The Authors summarize the feasibility of MIS, when performed with different purposes (biopsy / resection) and timing (initial / delayed surgery) for the most common solid tumours in children. The oncologic criteria must be always followed with MIS as well as with "open" surgery.

Hepatic hydatid cysts: intraperitoneal perforation in a pediatric patient.

We report a rare case of intraperitoneal hepatic hydatid cyst perforation in a 7 years-old boy. Diagnosis was performed by ultrasounds, computerized tomography scan, and serology. Surgical treatment was followed by pharmacologic treatment with the aim of avoiding a relapse. A brief review of the diagnostic and therapeutic management of the disease is provided.

Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis.

Features and outcome of neuroblastoma detected before birth.

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

[Experimental microsurgical gastrocystoplasty] . / La gastrocistoplastica sperimentale microchirurgica.

BACKGROUND: Stomach tract used for bladder augmentation decreases urinary pH and produces the syndrome of dysuria and hematuria; gastric mucosa in contact with urine may develop prominent histopathological changes including proliferative lesions. The aim of this study was to investigate in an experimental model the possibility of detecting the factors involved in the mucosal damage. METHODS: Thirty-five Sprague Dawley rats randomly underwent microsurgical gastrocystoplasty or sham operation (5 controls). During operation elliptical gastric patch was isolated with its gastroepiploic vascular pedicle, bladder was opened with midline incision and anastomosis performed. Urine was aspirated from the bladder for culture, pH and electrolytes evaluation; venous blood was samples for electrolytes, BUN and creatinine. Mean follow-up time was 6 months. RESULTS: Of the 30 rats subjected to gastrocystoplasty 23 survived (77%). All of cultures were negative, the urinary pH decreased after operation and increased gradually two months later. Urinary sodium and potassium ions concentrations increased significantly in gastrocystoplasty (p < 0.05). There were no significant changes in serum electrolytes or renal function. CONCLUSIONS: This experimental model was useful to investigate the effects related to the presence of gastric mucosa in the urinary tract.

Mouse-isolated plexus differentiates neural crest precursors into enteric neuroblasts.

Aim of this study was to investigate, for the first time, whether isolated newborn mouse enteric plexus could induce in vitro differentiation of the vagal neural crest-derived cells into enteric neuroblasts. Fragments of the myenteric plexus were isolated from the small intestine of 6-day-old Swiss mice and were collected and stored in DMEM-F12 medium, then cultured on polymerized human fibronectin layer. The vagal portion of the neural tube, isolated from a 9.5-day-old Swiss mouse embryo, was put in the same chamber slides where the isolated myenteric plexus had been cultured for 3 days. The vagal neural crest-derived cells migrated onto the polymerized human fibronectin layer and formed a crown of cells around the neural tube. After 6 days, the cultures were stopped and studied immunohistochemically for anti-NF160 KD, anti-TH, and RetR5 antibodies to analyse the differentiation stage of the cultured cells. Analysis of results included the comparison of two culture groups: Group 1, used as control, in which vagal neural crest-derived cells were put in DMEM-F12, supplemented only with 10 % of FCS; Group 2, in which vagal neural crest-derived cells were put in the same medium as Group 1, with the addition of myenteric plexus fragments isolated from newborn mice to form the co-culture. The following results were obtained: in Group 1 the neural tubes originated a cell population strongly positive for anti-NF160 and anti-TH Ab, but negative for RetR5 Ab. This positivity was found both in the cells adjacent to the neural tube and in those migrating from it distally. The Group 2 originated cells, which after migration were positive for anti-NF160 and for anti-TH antibodies. In addition, in this culture group, the cells which migrated from the neural tube were positive for anti-RetR5 antibody. The co-culture used in this study induces the differentiation of vagal stem cells into enteric neuroblasts, cells TH+ and RetR5+. These cells, after reaching the embryonic intestine, migrate to colonize the hindgut and form the ENS. Therefore this biotechnology seems a good method to obtain in vitro enteric precursors of ENS.

Leiomyosarcoma of the cecum in pediatric age: a case report and review of Italian reports.

The authors report a case of leiomyosarcoma of the cecum and review all the Italian reports of mesenchymal neoplasms registered in the RMS-Italy register. They emphasize the extreme rarity of intestinal leiomyosarcoma, especially with colon involvement: their case appears to be the only one registered in Italy in the last 12 years.

[Abdominal cystic lymphangiomas and mesenteric cysts: the clinical considerations]. / Linfangiomi cistici addominali e cisti mesenteriche: considerazioni cliniche.

The authors present their experience about intra-abdominal lymphangiomas and mesenteric cysts. The histologic evidence shows that these lesions are different. An exact histologic diagnosis is important because lymphangiomas are more invasive and relapsing than mesenteric cysts. Of 8 cases founded at laparotomy, 6 were lymphangiomas and 2 mesenteric cysts. Larger lesions were caused by lymphangiomas (mean, 11 cm vs 6 cm) when compared with mesenteric cysts. Complete resection was possible in all 8 patients, without recurrence after a mean follow-up of 4 years.

Abdominal wall defects: a 33-year unicentric experience.

AIM: The aim of this paper was to provide the main clinical features, surgical details, and long term outcome of patients with gastroschisis and omphalocele operated on at Giannina Gaslini Institute between 1976 and 2009. METHODS: All patients who were operated on between 1976 and 2009 for omphalocele or gastroschisis were included. Detailed informations regarding demographics, maternal history, type of delivery, associated anomalies, surgical details, complications, morbidity and mortality were collected. RESULTS: Sixty-one patients were included. Type of delivery did not interfere with outcome. Although patients with omphalocele had higher incidence of associated anomalies with their obvious impact on survival and quality of life, they showed a quicker recovery from surgery. Mortality rate was around 5%. Long-term outcome was available in 18 of them and proved to be satisfactory in all although almost 70% of them complained some gastrointestinal issues. CONCLUSION: Gastroschisis and Omphalocele showed improving survival and outcome during the last decades. Caesarean section proved not to confer advantages over vaginal delivery. Associated anomalies have the highest impact on survival being cardiac malformation the most significant risk factors. Although overall outcome is good in the majority of the patients, gastrointestinal and cosmetic issues seem to have a significant impact on quality of life and overall patients' perspectives.

Patch urethroplasty using a fascia lata autograft in male rabbits.

INTRODUCTION: Urethral reconstruction is difficult when the genital skin is not available for surgery. We evaluate the feasibility of using the autologous fascia lata as a graft for urethral repair. MATERIALS AND METHODS: 10 male rabbits underwent urethroplasty after creation of a ventral urethral defect. The defect was repaired using a graft harvested from the fascia lata. The animals were divided into three groups and sacrificed at 2, 4 and 12 weeks postoperatively. Radiologic control was performed after 10-12 days and before sacrifice. RESULTS: In the 10 rabbits subjected to surgery, no case of death or wound infection was observed. During urethrography, a fistula was observed in 2 animals. In the remainder (n = 8), histological analysis showed the preservation of the original laminar structure without graft shrinkage or fibrosis. On the luminal side of the patch, a new line of urothelium appeared in the 2nd week after surgery. After 3 months, the new epithelium was multilayered and the graft edges were not detectable. No voiding dysfunction was detectable in 8 rabbits. CONCLUSIONS: Our study suggests the feasibility of using the autologous fascia lata for urethral patch repair.

Histological changes following two-step flap gastrocystoplasty in rats.

INTRODUCTION: A gastric segment used to increase bladder capacity can undergo considerable changes over time, as can all intestinal segments implanted in the urinary tract and in contact with urine. This experimental study reports the differences between the histological alterations observed in the gastric patch transposed in the bladder both with its own pedicle and after deafferentation from the stomach. MATERIALS AND METHODS: A group of 30 young male Sprague-Dawley rats underwent gastrocystoplasty. Survivors were divided into 3 groups: gastrocystoplasty alone (8 rats); gastrocystoplasty with vascular deafferentation at 15 days (7 rats), and at 2 months (8 rats). 5 rats were used as controls. Urinary pH was evaluated during a 6-month follow-up. RESULTS: Histology showed that early devascularization hindered the fusion of the two mucosae in the junctional area but reduced papillary hyperplasia (p = 0.013) of the gastric mucosa. No changes were observed in urinary pH after patch devascularization. CONCLUSIONS: Vascular deafferentation creates a gastric flap on the bladder which, even if it does not prevent urinary acidification, reduces the frequency of histologically detected changes susceptible for transformation into neoplasms.