Pier Luca Ceccarelli

Anterior sagittal transanorectal approach to the urogenital sinus in adrenogenital syndrome: Preliminary report

In the female adrenogenital syndrome, the treatment of the urogenital sinus with high implanted vagina still presents a surgical challenge. The conventional technique (perineal vaginal pull-through) has been plagued by a high incidence of vaginal stenosis. A posterior sagittal transanorectal approach was proposed as an alternative to obtain an excellent exposure and thus an adequate mobilization of the vagina. But bivalving a normal rectum and anus has the potential for compromising bowel control and represents a bold maneuver, and the rectum and sphincteric mechanism must be meticulously reconstructed. In the original procedure a protective colostomy must be performed before the operation. To reduce these disadvantages, the authors made the following modifications: sagittal incision of only the anterior rectal wall (Anterior Sagittal Transanorectal Approach--ASTRA) and protective colostomy at the same time as the operation. In this way, maintaining the same excellent exposure and reducing the number of operations from three to two, we operated on 10 girls with adrenogenital syndrome: 4 with high, 3 with intermediate urogenital sinus, and 3 who had previously undergone vaginal pull-through but experienced a vaginal retraction with severe stenosis. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six were from 2 to 11 years of age (mean, 6.5 years). After closing the colostomy, all patients were continent in stools and urine, and the vagina looked normal. The authors suggest using this modified approach as an alternative to the conventional operation and for those patients in whom other techniques have failed.

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.

ANTERIOR SAGITTAL TRANSANORECTAL APPROACH TO THE POSTERIOR URETHRA IN THE PEDIATRIC AGE GROUP

PURPOSE Surgical access to the posterior urethra is often difficult and several surgical solutions have been proposed. We suggest an anterior sagittal transanorectal approach based on splitting the anterior rectal wall only. This alternative technique provides excellent exposure to the retrourethral region, permitting simple and safe surgery. MATERIALS AND METHODS Between 1994 and 1996 we performed surgery via the anterior sagittal transanorectal approach in 8 patients with a mean age of 9.06 years. Patients included 1 girl with a posttraumatic urethrovaginal fistula, 3 with intersex disorders (2 with mixed gonadal dysgenesis raised as boys and 1 with male dysgenetic pseudohermaphroditism with an enlarged urtricle) and 4 boys (1 with penile agenesis raised as girl, 2 with urethral duplication and 1 with prostatic rhabdomyosarcoma). The patient was placed in a knee-chest position. A midline sagittal incision was made through the anterior anorectal wall only and deepened through the perineal body to expose the posterior urethra and retrovesical space. After the pathological condition was corrected the anterior rectal wall and perineal body were reconstructed. The operation was completed with protective colostomy. In our final patient with prostatic rhabdomyosarcoma the anterior sagittal transanorectal approach was used without colostomy. Anorectal manometry was done 6 months postoperatively. RESULTS All patients were completely continent of stool and urine. Convalescence was unremarkable in all cases. Postoperative manometry in 7 patients revealed no differences from preoperative measurements. CONCLUSIONS This procedure should be considered a useful alternative to other techniques for various congenital and acquired pelvic disorders.

Jejunal obstruction as initial presentation of polyarteritis nodosa in a 13-month-old boy

Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality.

Bilateral xanthogranulomatous funiculitis and orchiepididymitis in a 13-year-old adolescent boy

Xanthogranulomatous orchitis is an extremely rare inflammatory nonneoplastic lesion of the testis. We report a case of a 13-year-old adolescent boy who presented a painless left hemiscrotal swelling. The subsequent ultrasonography and magnetic resonance imaging revealed the presence of abnormal expanding tissue located in both testes and spermatic cord, reaching the internal inguinal ring. Testicular tumor markers were normal. The frozen section examination of the surgical specimen showed only inflammatory tissue and not neoplastic tissue. No orchiectomy was performed. Definitive histopathologic diagnosis was xanthogranulomatous inflammation. To our knowledge, this is the youngest case of xanthogranulomatous orchiepididymitis and funiculitis found in medical literature.

Intraluminal duodenal diverticulum: a rare cause of abdominal pain in childhood

The intraluminal duodenal diverticulum (IDD) is an uncommon pathological condition that causes a mild clinical picture, arising in most cases in the pediatric age group. Often found incidentally, it is discovered in most patients on contrast X-ray examination, but in uncertain cases duodenoscopy or i.v. cholangiography can be performed. Surgical excision of the diverticulum by duodenotomy makes possible the remission of symptoms and allows complete recovery. A 10-year-old girl with IDD is reported who had recurrent epigastric, meal-related pain that led to appendectomy 1 year prior to admission. The diagnosis was made on X-ray examination and the patient underwent a laparotomy with complete excision of the diverticulum. The girl, at present, is the youngest patient with a true IDD ever reported in the literature.

FIRST CASE OF LAPAROSCOPIC PARTIAL SPLENECTOMY IN A CHILD WITH HAMARTOMA: CASE REPORT AND REVIEW OF THE LITERATURE

Highlights • Laparoscopic surgery as technique of choice in children for benign solid lesions of the spleen.• Multidisciplinary treatment of rare disease, Hamartoma of the spleen, in childhood.• Role of chemoembolization in the pre-operative surgical setting to reduce intraoperative bleeding.• Partial splenectomy as safe procedure to preserve hematological function of the spleen.

Transperitoneal laparoscopic adrenalectomy in children.

Purpose: The use of a minimally invasive approach for adrenalectomy is poorly defined in pediatric patients, although laparoscopic adrenalectomy is considered a standard procedure in adults. The aim of our study was to describe the safety and feasibility of minimally invasive adrenalectomy in children on the basis of surgical skills and results. Materials and Methods: This was a retrospective study of 4 pediatric laparoscopic adrenalectomies performed at our center between 2009 and 2012. All patients underwent transperitoneal lateral laparoscopic adrenalectomies (2 right and 2 left adrenalectomies). Results: Four laparoscopic adrenalectomies were performed. Indications for surgery were neuroblastoma in 2 patients, secernent adrenocortical tumor in 1 patient, and adrenocortical nodular hyperplasia in 1 patient. Patients had a mean age of 87 months (range, 17–156 months) at diagnosis, and the average lesion size was 3.23 cm (range, 0.7–6.4 cm). All laparoscopic adrenalectomies were successful, no conversions to open surgery were required, and no postoperative complications or deaths occurred. The average operating time was 105 minutes (range, 80–130 minutes), blood loss during surgery was minimal, and the mean postoperative hospital stay was 3.75 days (range, 3–5 days). None of the patients showed signs of recurring disease at 15-month follow-up. Conclusions: Laparoscopic adrenalectomy is a safe, feasible, and reproducible technique offering numerous advantages, including shortening of operating times and postoperative hospital stays, as well as reduction of blood loss and complications. It also provides good visibility and easy access to other organs.

Chirurgia Pediatrica Toracoscopica

La rivoluzione tecnologica di questi ultimi anni in tema di innovazioni riguardanti il grande capitolo della chirurgia mininvasiva ha coinvolto anche la chirurgia toracica, nel cui ambito queste tecniche hanno guadagnato un grande spazio diagnostico e terapeutico.