Giovanni Ruggeri

Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls

BACKGROUND/PURPOSE The absence of vagina is rare in the pediatric population. It can occur as a result of congenital malformations such as an aplasia of mullerian ducts (46,XX Mayer-Rokitansky-Küster-Hauser syndrome) or a complete androgen insensitivity syndrome (46,XY testicular feminizing syndrome). Intersex patients, who underwent reassessment of a female sex, need a genital reconstruction toward a feminine phenotype. Patients with congenital adrenogenital syndrome with high urogenital sinus could have a severe hypoplastic vagina. In all these cases, a vaginal replacement is required. We reviewed our experience of vaginal replacement using a sigmoid conduit. METHODS In 34 years, we evaluated 47 patients. The observation period was from 1 to 34 years (mean: 12 years). The preoperative diagnosis was Mayer-Rokitansky-Küster-Hauser syndrome in 17 cases, androgen insensitivity syndrome in 24 cases, adrenogenital syndrome with high urogenital sinus in 5 and 1 patient was affected by penile agenesis. Forty-six patients were treated with vaginal reconstruction by interposition of sigmoid colon. Only in 1 case we performed a vaginal construction with an ileal loop: in this case, the sigmoid colon was extremely dilated by a chronic constipation secondary to a high anorectal malformation corrected at birth. RESULTS The outcome for 47 patients is excellent: 18 are sexually active and 4 are married. Only 1 patient with adrenogenital syndrome died of endocrine problems. Complications occurred in 17 cases: in 1 patient a necrosis of the replaced vagina occurred, thus requiring vaginal exeresis; now she is waiting for a second operation. Another patient had an abdominal abscess, which was surgically treated. In 12 cases a second procedure was required: 6 had stenotic new-vaginal introitus, 4 had new-vaginal prolapse, and 2 had intestinal obstruction. CONCLUSIONS We believe that the preferable technique for vaginal replacement is the use of intestinal conduit. The sigmoid colon is the best intestinal tract to be used owing to its size, location and preserved blood supply. Our experience leads us to believe that the sigmoid segment is the segment of choice, although we consider ileal loop as a good alternative when the sigmoid colon is not available.

Italian multicenter survey on laparoscopic treatment of gastro-esophageal reflux disease in children

Background: Skepticism is still present today about the laparoscopic treatment of gastro-esophageal reflux (GER) in children. We present the prospective experience and short-term results of eight Italian pediatric surgical units. Methods: We included all the children with complicated GER, operated after January 1998 by single surgeons from eight different centers. Diagnostic aspects, type of fundoplication, and complications were considered. All the patients were followed for a minimum period of 6 months in order to detect complications or recurrences. Results: 288 children were prospectively included. Mean age was 4.8 years (3 m–14 y). Nissen fundoplication was done in 25%, floppy Nissen in 63%, Toupet in 1.7%, and anterior procedures (Lortat Jacob, Thal) in 10%. Gastrostomy was associated, if neurological impairment or feeding disorders were present. Mean follow-up was 15 months and reoperation was necessary in 3.8% of cases. Conclusions: This experience underlines that minimal invasive access surgery in children is safe and that the laparoscopic approach is considered in eight centers the golden standard for surgical repair of gastro-esophageal reflux disease maintaining the same indications and techniques of the open approach.

PRENATAL DIAGNOSIS OF BLADDER EXSTROPHY: WHAT COUNSELING?

PURPOSE Bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases. MATERIALS AND METHODS At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy. RESULTS We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria. CONCLUSIONS We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy.

Sparing-lung surgery for the treatment of congenital lung malformations☆

PURPOSE Congenital lung malformations (CLM) are traditionally treated by pulmonary lobectomy. The foremost indication for surgery is that these lesions predispose to respiratory tract infections and to malignancy. Owing to the improvement of prenatal diagnosis, most patients are operated in a timely manner and prophylactically. In this context, lung-sparing surgery (LS) has gained interest as a lung preservation strategy, especially for asymptomatic patients. In the present study, we evaluated both thoracoscopic and open lung-preserving resections as an alternative to lobectomy for CLM resection. METHODS We retrospectively reviewed all patients who underwent lung-sparing resection (segmentectomy and atypical resection) for CLM from 2001 to 2010. Data were collected regarding preoperative diagnostic workup, type of intervention, and follow up. RESULTS Fifty-four patients received LS for CLM during the study period. Twenty-six were approached thoracoscopically, with 18 cases requiring open conversion for a complete resection. There were six postoperative complications: three asymptomatic pneumothoraces that resolved without intervention, one tension pneumothorax that required replacement of a drainage catheter, and three instances of intra-operative bleeding requiring blood transfusion. Mean duration of follow-up was 65.2 months. Two patients experienced pneumonia during the follow-up period. A third patient had a cystic lung lesion on postoperative computed tomography (CT) which required a second-look surgery. CONCLUSIONS LS for CLM is a safe and effective means of lung parenchymal preservation in pediatric patients. Complication rates are comparable to that of traditional lobectomy. In our experience, this type of lung surgery does not carry a higher risk of residual disease and recurrence if accurately planned in selected patients, i.e., those with small asymptomatic lesions. The complication rate is acceptable and apparently not affected by preoperative symptoms. The thoracoscopic approach is recommended, although open conversion should be advocated to avoid too long operative times.

Laparoscopic antireflux surgery in neurologically impaired children

From May 1996 to April 2002, 48 laparoscopic fundoplications were performed after failure of medical treatment in 47 neurologically impaired infants and children affected by gastroesophageal reflux. Indications for surgery included vomiting, recurrent upper airway infections, failure of medical therapy, feeding difficulties with failure to gain weight, and instrumental (barium swallow and pHmetry) diagnosis of gastroesophageal reflux. A standard approach was adopted, with minimal access modifications according to the patients’ characteristics. In two patients, laparoscopic surgery had to be converted to open surgery because of severe kyphoscoliosis and accidental left emidiaphragm perforation. In another patient undergoing a laparoscopic Nissen fundoplication, a re-do laparoscopic operation was performed. Postoperative analgesia was administered during the first 12 h, and fluid intake and feeding were begun on days 1 and 2, respectively. All patients clinically improved except two; a paraesophageal hernia developed in one, and a stenosis developed in the other. We strongly believe that laparoscopic fundoplication can be successfully adopted in neurologically impaired children as well as in pediatric patients as a whole, with the same advantages and far fewer drawbacks than are expected in adults.

Long-term follow-up after esophageal replacement in children: 45-Year single-center experience

PURPOSE We evaluated the results of esophagocoloplasty (ECP) in children by performing a 45-year retrospective cohort study in a single center. MATERIALS AND METHODS We collected all of the medical charts of patients who underwent ECP at our hospital from January 1969 to January 2014. We reviewed the medical history for each patient and then contacted the patients by phone to obtain responses to a questionnaire. The questionnaire evaluated the following four areas: overall general state, gastrointestinal function, dependence on the Health Sanitary System, and their aesthetic satisfaction. RESULTS We contacted 96 patients, and 72 completed our questionnaire. There were 45 males and 27 female respondents, with a mean age of 34.5 years. The mean Karnofsky performance status index was 96.4%. There were excellent scores obtained from 55 patients and fairly good scores from 16 patients. There was 1 patient with a low score, and there were no scores reported below 70%. Most patients (58/72) reported regular bowel habits, and no patients required drugs or other measures to facilitate defecation. Gastroesophageal reflux was present in 51 patients, and 62.8% have bothersome symptoms. Twenty-five patients used pharmacological therapies. Forty-eight patients (66.6%) were satisfied with the aesthetic result of surgery (mean score in a scale from 0 to 5, is 3). CONCLUSIONS ECP can be used in children who require esophageal substitution. The resulting long-term QoL is acceptable. However, the aesthetic outcomes remain a problem.

Recurrent posttraumatic urethrovaginal fistula : a new application for ASTRA

The authors describe the results of an application of the surgical technique called ASTRA (anterior sagittal transrectal approach) in a 16-year-old girl with recurrent urethro-vaginal fistula. The young girl had a posttraumatic urethro-vaginal fistula. It recurred after 4 operations by a direct vaginal approach before definitive correction with the ASTRA. Three years after the operation the patient has remained well with complete healing and no fistula recurrence confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. This report suggests that ASTRA is a useful method of treating acquired or developmental anomalies of the perineal region.

Laparoscopic resection of a rare gastrointestinal stromal tumor in children

IntroductionGastrointestinal stromal tumors (GIST) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract, accounting for 0.1% to 3% of all GI malignancies. Paediatric GIST have an annual incidence of 0.02 per million children, have a female predisposition, are usually located in the stomach (50–60%) and in up to 85% of cases CD117-cKit or PDGFRA mutation is absent, resulting in a decreased efficacy of the target therapy.Case descriptionWe report the case of an incidentally diagnosed gastric GIST in a 14-year-old boy with multiple malformations. Genetic tests and Kariotype resulted negative.Recently, an abdominal US visualized an hypoechoic heterogeneous abdominal mass. The common tumor markers resulted negative and the abdominal CT-scan confirmed the presence of a solid round lesion (42×36mm) in contact with the stomach and the pancreas. Laparoscopy allowed the recognition and the removal of the nodular mass at the posterior margin of the stomach. The histopathologic and the molecular biology findings were consistent with a kit-wilde type GIST. Surgical margins were microscopically free of tumor cells.These results justify the decision not to add other surgical or medical therapy. However, for high risk of recurrence and metastasis, a close follow-up was started.Discussion and evaluationGIST are asymptomatic in 10% to 30% of patients or present nonspecific symptoms and signs. These tumors present usually irregular, lobulated and ulcerated. CT-scan of the abdomen and pelvis or magnetic resonance imaging (MRI) are mandatory in the diagnostic work-up. The final diagnosis is based on histology and immunohistochemistry. Surgery is the first-line treatment in patients with localized disease.ConclusionGuidelines for the management of pediatric GIST are not presently available for the paucity of reports and data. However it is widely accepted that surgery is the first-line treatment and gross resection with negative microscopic margins can be considered therapeutic and lead to full remission of the pathology. Laparoscopy is a safe surgical approach for the exploration of the abdominal cavity, the evaluation of the disease and the complete removal of the tumor.

Combined laparoscopic and hysteroscopic approach for the treatment of a hybrid Müllerian duct anomaly: a case report.

Müllerian duct anomalies include a wide spectrum of genital tract defects resulting from a development alteration of the genital tract. An 11-year-old girl with a hybrid septate variety uterus with left hemi-obstruction was identified. Beside preoperative imaging evaluation done with ultrasound, magnetic resonance, and hysterosalpingography, a combined laparoscopic and hysteroscopic procedure was fundamental for the diagnosis. The surgical procedure also allowed us to perform a successful and safe treatment. We propose that the American Fertility Society classification of these anomalies should be revised and that hybrid entities should be considered in the differential diagnosis of genital tract malformations.

One-trocar-assisted pyeloplasty in children: an 8-year single institution experience.

Introduction  Ureteropelvic junction obstruction (UPJO) is the most common pediatric obstructive uropathy. We report our 8-year experience with the use of a retroperitoneal laparoscopic-assisted pyeloplasty (one–trocar–assisted pyeloplasty [OTAP]). Materials and Methods  This is a retrospective analysis of 88 patients treated in 8 years. Surgery was performed on the basis of standard indications and it consisted of 88 OTAP (the ureteropelvic junction is identified through a retroperitoneoscopic inspection and it is exteriorized to perform a traditional dismembered pyeloplasty). Operative time, hospital stay, complication, success of the technique, and aesthetical results are analyzed. Results  In the 8-year period we performed 88 OTAP. Mean age was 19 months. Prenatal diagnosis was the most common presentation ( n  = 73), followed by occasional identification ( n  = 8), and symptomatic ( n  = 7). Mean operating time was 139 minutes (range 60–225 minutes). Conversion was required in seven cases. The success rate was 87.5% (reduction of the severity of hydronephrosis on ultrasound) and 100% among symptomatic patients (symptoms resolution). Aesthetical results were excellent. Discussion and Conclusions  OTAP is safe, feasible, and efficacious. Operative times are similar or even shorter than those of other minimally invasive techniques and complication rate agrees with the findings of literature. OTAP can be considered a reasonable alternative.