Marcello Dòmini

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.

Bifid sternum: neonatal surgical treatment.

The sternal cleft is a rare congenital anomaly resulting from a fusion failure of the sternum, generally observed at birth and asymptomatic. Surgery is indicated to protect the heart and major vessels from trauma, to improve respiratory dynamics, and for aesthetic reasons. We observed 2 neonates for a superior and medial thoracic mass. The defect involved the upper two thirds of the sternum. The surgical operation for both patients consisted in the primary closure of the defect.

A simple and safe method to visualize the inferior pouch in esophageal atresia without fistula.

Abstract The authors studied the true “dynamic” distance between the esophageal stumps in type I atresia in order to perform the delayed anastomosis at the most favorable time. The position of the inferior pouch was fluoroscopically evaluated in four patients, inserting a Hegar dilator through the gastrostomy. The superior esophageal pouch was delineated by a Replogle tube. No anesthesia was required. In all cases the procedure was simple, safe, fast, and accurate. No complications occurred, and patients could be operated upon at the optimal time.

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.

Drenaggio pleurico e mediastinico nei versamenti pleurici e nello pneumotorace

Il pneumotorace (Pnx) e una raccolta d’aria nel cavo pleurico che determina uno schiacciamento del polmone e il conseguente collasso [1]. Questo collasso polmonare puo essere parziale o totale, arrivando, nelle forme ipertese, a determinare anche uno sbandieramento del mediastino verso l’emitorace del lato opposto.

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.

Pneumothorax and Pneumomediastinum

PNX is a collection of air in the pleural cavity which leads to crushing of the lung and its consequent collapse [1]. Lung collapse may be partial or total and in some hypertensive forms it can also lead to the shift of the mediastinum towards the opposite hemithorax.

The Gastroesophageal Reflux Secondary to Malformations

The gastroesophageal reflux (GER) is a common problem in the neonatal period and in early infancy, when an effective maturation of the low esophageal sphincter (LES) [7] (with its definitive competence from the fifth to the seventh weeks of life) and an increasing length of the intra-abdominal esophagus, from 0.5–1 cm at birth to 2.5–3 cm at 3 months of age, have been demonstrated [8].

Gastric duplication cyst in a child treated by extramucosal excision

Gastric duplication is a very rare malformation with a distinctive clinical and anatomical picture that differs from other duplications of the gastrointestinal tract. The authors describe a case of gastric duplication in an 18-month-old child presenting with melena and anemia.