Remigio Dòmini

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.

Multiple intestinal atresia with apple peel syndrome: successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent.

The association of apple peel bowel with multiple intestinal atresias is a rare event. The authors report a case of a 2.200-g newborn affected by this uncommon condition. The interruptions of small bowel continuity were treated successfully with 5 end-to-end anastomoses and with the construction of 2 ostomies. A gastroduodenal silicone tube (Vygon S.A., Ecouen, France) was used as an intraluminal stent.

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.

Intestinal bypass of the oesophagus: 117 patients in 28 years.

Abstract This study includes 117 patients operated upon in the period from 1970 to 1999. Indications, surgical techniques, complications, and results are reviewed. Indications included: long-gap oesophageal atresia with or without fistula in 81 patients; peptic stenosis in 19; caustic stenosis in 12; oesophageal varices in 2; and 1 case each of oesophageal epidermolysis bullosa, total oesophageal leiomyomatosis, and a non-functioning antiperistaltic retrosternal colic graft operated upon in another hospital. A retrosternal bypass was performed 106 times: 98 first operations and 8 redos; the intrathoracic technique was used 19 times. The left transverse colon was used in 107 cases (85.6%), the right transverse colon in 8 (6.4%), and the ileocecum in 10 (8%). All the intestinal bypasses were placed in the isoperistaltic direction. There were 5 deaths in the first 11 years of our experience; no patient died from 1982 on. Ten complications were treated conservatively (8%): 2 wound infections healed with medical treatment, and 8 leaks of the cervical anastomosis closed spontaneously. The major surgical complications were 8 gangrenous bypassess (6.4%), removed and reopeated about 1 year later utilizing an ileocolic retrosternal graft. Three cases of peptic disease of the colic bypass (2.4%) were successfully treated with the authors technique. Nine patients had minor surgical complications (7.2%): 3 strictures of the oesophagocolic anastomosis in a retrosternal bypass (resected and reoperated) and 6 cases of adhesive occlusion. In our opinion, the best substitute of the oesophagus is the colon, particularly the left transverse segment, which may be placed behind the sternum or in the oesophageal bed, always in the isoperistaltic direction. The low mortality (4%), restricted to the early period of our experience, and few major surgical complications (6.4%) are acceptable considering the importance of the operation, and the long-term results may be considered very satisfactory.

Fetus in fetu: report of an additional, well-developed case

Abstract An 8-month-old boy presenting with a fast-growing abdominal mass was operated upon to remove the tumor, which was confirmed to be a well-formed fetus in fetu. The authors describe the clinical, radiologic, and surgical findings and review the literature.

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.