Tommaso Gargano

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.

Ovarian Tissue Collection for Cryopreservation in Pediatric Age: Laparoscopic Technical Tips

As the number of young girls subjected to chemo and radiotherapy is steadily increasing it is our duty to try to preserve their future fertility and ovarian function. A possibility is to collect ovarian samples, cryopreserve them and reimplant the tissue when the treatments are over. We report our 11 year experience with the use of laparoscopy to collect ovarian tissue samples. In particular we focus on the details of the surgical technique. The procedure was performed in 54 patients affected by malignancies or pathologies requiring chemo or radiotherapy, with good results. Cryopreservation seems to be a good option to preserve future fertility although it is still an experimental method.

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.

An Interesting Case of Double Compound Intussusception without Intestinal Occlusion in a 5-Year-Old Boy

Intussusception is a very common surgical finding in children. Idiopathic intussusception is relatively frequent in children within the first year and it usually causes an acute abdomen. Instead, complex intussusception involving multiple or remote intestinal segments is a very rare entity and it is frequently diagnosed intraoperatively. It is often because of an anatomical cause and it can occur at any age. We report a unique case of contemporary double site anterograde and retrograde ileoileal intussusception without intestinal occlusion because of a submucous intestinal lipoma. In our case, imaging studies were important for clinical suspicion and laparoscopy was essential for final diagnosis and its resolution.

3D virtual rendering before laparoscopic partial splenectomy in children.

INTRODUCTION Partial splenectomy in children is a good surgical option for hematological diseases and focal splenic tumors because it allows the preservation of the spleens immunological function. Furthermore, it can be performed by laparoscopy in children as it is a safe procedure, offering the benefits of a minimally invasive approach. MATERIALS AND METHODS The software VR-render LE version 0.81 is a system that enables the visualization of bidimentional 3D images with magnification of anatomical details. We have applied this system to five cases of non-parasitic splenic cysts before laparoscopic partial splenectomy. RESULTS The images obtained with VR rendering software permitted the preoperative reconstruction of the vascularization of the splenic hilum, allowing the surgeon safe vessel control during laparoscopic procedures. All five partial splenectomies were carried out with no complications or major blood loss. CONCLUSIONS Laparoscopic partial splenectomy should be a first choice procedure because it is feasible, reproducible, and safe for children; furthermore, it preserves enough splenic tissue thereby preventing post-splenectomy infections. Volume rendering provides high anatomical resolution and can be useful in guiding the surgical procedure.

Two cases of abdominal pain in children with mesenteric lymphadenitis due to Yersinia pseudotuberculosis infection.

Journal of Pediatrics, The - In Press.Proof corrected by the author Available online since lundi 5 mai 2014

Secondary Omental Torsion as a Rare Cause of Acute Abdomen in a Child and the Advantages of Laparoscopic Approach

Omental torsion is an underdiagnosed cause of acute abdomen in children. Most cases occur in adults, with only 15% presenting in children. It is estimated that omental infarct is found in approximately 0.1 to 0.5% of children undergoing operations for appendicitis. It may present with various signs and symptoms, although the clinical presentation usually mimics that of acute appendicitis. Definitive diagnosis is often established during surgery. We report a case of a 9-year-old boy who came to our attention for acute abdomen. We performed a laparoscopy-assisted procedure suspecting acute appendicitis. We therefore identified a secondary omental torsion associated with patency of the right inguinal duct and herniated omentum. Laparoscopy allowed a correct diagnosis and therapy.

Transverse Testicular Ectopia and Persistent Mullerian Duct Syndrome: Video-assisted Treatment

for karyotyping. Two bilateral tubular structures resembling a vas deferens were observed starting from the gonads and proceeding parallel to the uterus towards the urethra. Histology showed normal testicular tissue without any ovarian tissue and karyotyping gave a result of 46 XY, confi rming the suspicion of PMDS. The second operation, one month later, started with a cystoscopy showing the “ vaginal ” opening into the prostatic urethra. Then, using a three-trocar laparoIntroduction & Persistent Mullerian duct syndrome (PMDS) is a rare autosomal recessive disorder in which, due to a defi ciency of the Mullerian inhibiting substance (MIS), the complete development of the Mullerian duct structures occurs in the presence of a male phenotype and a 46 XY karyotype. In these patients, a uterus, tubes and vagina are often present which open into a normal male prostatic urethra. These female structures are usually found during surgical correction of an inguinal hernia or during a laparoscopic exploration for non palpable testis. Transverse testicular ectopia (TTE) is frequently present. Here we present the case of a 6-monthold boy with this rare association and its treatment by video-assisted excision of the Mullerian remnant, video-assisted orchidopexy and laparoscopic closure of the inguinal defects.

Robotic removal of Müllerian duct remnants in pediatric patients: our experience and a review of the literature

Persistent Müllerian duct syndrome is a disorder of sexual development, which features a failure of involution of Müllerian structures. An enlarged prostatic utricle is a kind of Müllerian duct remnant (MDR) with a tubular shaped structure communicating with the prostatic urethra. Treatment is aimed at relieving symptoms when present, preserve fertility and prevent neoplastic degeneration. We describe 3 cases of successful robot assisted-removal of symptomatic MDRs. The first case came to our attention for pseudo-incontinence; the other two for recurrent urinary tract infections. The patients have not presented such symptoms anymore on follow-up. We then reviewed existent literature on authors who have recently investigated the main issues concerning MDRs and have attempted a roboticassisted approach on them. Robot-assisted laparoscopy can be considered a valid, safe and effective minimally-invasive technique for the primary treatment of prostatic utricle.

Robot-assisted Pyeloureterostomy in Bifid Renal Pelvis With Ureteropelvic Junction Obstruction of the Lower Moiety

Duplications of the urinary collecting system and pyelo-ureteral junction obstruction (PUJO) are common, but the simultaneous presence of both anomalies is rarely encountered. In incomplete duplicated systems, PUJO usually affects the lower moiety. We present the case of a 5-year old boy with left bifid renal pelvis and hydronephosis of the lower moiety treated by robot-assisted pyeloureterostomy.