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Biomedical subjects

R Dòmini

Publications and source records attributed to R Dòmini.

At least 19 recordsLinked to original sources

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.

Abnormalities, Multiple↗

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

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.

Abdomen↗

Pleuropulmonary blastoma in congenital cystic adenomatoid malformation: report of a case.

Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic lesion. The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made. Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.

Antineoplastic Combined Chemotherapy Protocols↗

Laparoscopic surgery of diaphragmatic diseases in children: our experience with five cases.

AIMS: In paediatric surgery the laparoscopic approach can be used to repair diaphragmatic anomalies originating from the abdomen or containing abdominal viscera. Candidates for laparoscopic correction are children with mild symptoms and good respiratory and haemodynamic conditions. The authors present their experience with 5 patients treated successfully for different types of diaphragmatic lesions. PATIENTS AND METHODS: Five children were treated laparoscopically since 1998. Two true Morgagni-Larrey hernias, one recurrent left Bochdalek hernia, one diaphragmatic dysontogenetic cyst and one huge congenital sliding and rolling hiatal hernia. All the herniated viscera were repositioned in the abdomen and the defects--including the diaphragmatic hole at the level of the dysontogenetic cyst--were directly sutured without the use of a mesh. RESULTS: All patients are healthy without signs of recurrence observed at chest X-ray after a follow-up of 3 months to 1 year. DISCUSSION: Under specific conditions the laparoscopic approach can be an effective and more advantageous alternative to laparotomy for diaphragmatic congenital diseases in a paediatric population.

Child↗

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

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 bypasses (6.4%), removed and reoperated 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 author's 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.

Burns↗

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 the case of a 16-year-old girl with a posttraumatic urethrovaginal fistula. The young girl with a posttraumatic urethrovaginal fistula had recurrence after 4 surgical attempts at closure performed by the vaginal approach before it was definitively corrected with the ASTRA. Two years after the ASTRA operation the patient is well with a complete restitution of function confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. The successful recovery confirmed that ASTRA may be an excellent method of treating the developmental or acquired anomalies of the perineal region.

Adolescent↗

Laparoscopic repair of Morgagni-Larrey hernia in a child.

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.

Child, Preschool↗

Recurrent posttraumatic urethro-vaginal 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.

Adolescent↗

Multicystic kidney joined to the homolateral testis by an ectopic vas deferens: embryological hypothesis for a new description.

Ectopic location of the vas deferens is a rare congenital anomaly with multiple variations. The frequent association with ano-rectal abnormalities and hypospadias is well known. We report two cases of a newborn and a 3-year-old child with ectopic vas deferens coupled with a left multicystic kidney without any ureteral structure. Furthermore, the latter had a contralateral vesicoureteral reflux and anal agenesis with recto-bulbar fistula. Our attempt with this report is to explain the development of such anomalous deferential outlet into the multicystic kidney, according to the most qualified theory of the Wolffian duct embryology.

Child, Preschool↗

Esophageal leiomyomatosis in children: report of a case and review of the literature.

The authors describe a case of a 5-years-old girl affected by diffuse oesophageal leiomyomatosis presenting with progressive dysphagia, cyanosis, recurrent pneumonia and retrosternal pain. She also suffered from occasional constipation due to perineal involvement by the disease and had a past history of recurrent microscopic hematuria, suggesting an association with an Alport-like syndrome. Only 24 cases of esophageal leiomyomatosis could be found in the medical literature in children aged less than 14 years, confirming the rarity of the disease. A clinical analysis of all cases reviewed from the literature is made, stressing the importance of an accurate preoperative diagnosis for the choice of proper surgical treatment to avoid recurrence.

Child, Preschool↗

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.

Adolescent↗

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.

Adrenal Hyperplasia, Congenital↗

Microvascular autotransplantation of the testis: the "refluo" technique.

We think that the microvascular orchidopexy is the best technique for the treatment of the high intraabdominal testis. To prevent the problems related to the performing of the anastomosis between the spermatic and the inferior epigastric arteries we changed the traditional way for microvascular orchidopexy performing the venous anastomosis only, relying on the collateral deferential circle for the arterial supply; we called this technique the "refluo testicular autotransplantation". We supported our idea with an experimental investigation in rats and rabbits, with which we confirmed the efficacy of our proposal. This original technique, performed in 41 cases, allowed us to gain three important ends: a) reduction of the age at operation (under two years); b) reduction of the operating time (2 hours); c) improved success rate compared with the Fowler-Stephens technique or staged orchidopexy.

Adolescent↗

A case of non-functioning antiperistaltic retrosternal colic conduit replaced in situ and substituted with an isoperistaltic segment of ileum-caecum.

The authors report the case of a female patient, affected by long-gap oesophageal atresia, who, at 5 months old, was operated on for retrosternal substitution with a right-transverse antiperistaltic colic segment, in her local district hospital. Due to the anomalous position the neooesophagus never worked, and the baby was seriously dysphagic and failed to thrive. For this reason, when she was 11 months old, she was reoperated in our department. Through a medium sternolaparotomy the antiperistaltic colon was removed and replaced between the left and right colon; reconstruction was carried out with a retrosternal and isoperistaltic segment of ileo-caecum. The reoperation resolved her problems. This case is reported to confirm the author's opinion that all intestinal conduits must be positioned in the isoperistaltic direction.

Cecum↗

Wilms' tumor involving the inferior vena cava: preoperative evaluation and management.

Neoplastic invasion of the inferior vena cava due to renal tumors (especially Wilms' tumor) is uncommon in children. The tumor thrombus, according to the aggressiveness of the original neoplasm, can extend in diverse ways, obliterate the vascular lumen completely, and even reach the right atrium. The luminal thrombus might be accompanied by the involvement of the caval wall, which requires wide vascular resection. The purpose of this paper is to present our experience with 7 children, aged 18 months and 6 years, affected by caval invasion due to Wilms' tumor. Furthermore, the diagnostic techniques and the surgical treatment in simple caval thrombosis and in associated invasion of the caval wall are described.

Child↗

Adrenocortical tumors in children: a report of 12 cases.

Adrenocortical tumors in children are extremely rare, accounting only for 0.3-0.4% of all neoplasms in this age. Most frequently they secrete hormones, resulting in virilization, Cushing's syndrome or feminization, while the non-functioning ones are unusual. The authors describe 12 cases observed in 13 years (1976-1989), with a mean age of 5 years. 9 cases showed virilization, 4 presented with Cushing's syndrome and in 5 patients an abdominal mass was palpable. One case was affected by Beckwith-Wiedemann's syndrome. I.V. urography was performed in 8 patients, arteriography in 4 and since 1982 all patients were submitted to abdominal sonography and CT scan or MR imaging. Urinary 17-ketosteroids, 17-hydroxycorticoids and serum testosterone and cortisol were tested in all children. Dexamethasone suppression test was performed in 7. All patients were treated with surgery which seems to be the most suitable treatment, while the real effectiveness of treatment by drug therapy with suppressors of steroidogenesis is not confirmed in children. Histopathological examination showed typical features of adenoma in 5 cases, of adenocarcinoma in 4, while three cases revealed border line forms classified as "atypical adenomas". At the moment 10 patients are alive with a follow-up ranging from 18 months to 14 years, while 2 children with adenocarcinoma are dead.

Adenocarcinoma↗

Indications for bladder augmentation in the exstrophy-epispadias complex.

OBJECTIVE: To determine whether bladder augmentation has a role in avoiding urinary diversion in patients with exstrophy-epispadias complex (EEC), whether it can improve the lifestyle in patients who have previously undergone diversion or improve the result of any single surgical step in the staged functional reconstruction of the bladder in these patients. PATIENTS AND METHODS: From 1970 to 1991, 85 patients were treated for EEC. Between 1981 and 1991, 12 bladder augmentations were performed in 11 patients (seven girls and four boys) with bladder exstrophy, male epispadias (one case) and cloacal exstrophy (one case). RESULTS: No significant early surgical complications were reported. Follow-up ranged from 18 months to 11 years. Late complications included bowel obstruction in one patient, a partial left ureteric stenosis at the level of the anastomosis with the gastric patch wall in one patient and bladder lithiasis in five patients (sigmoidocystoplasty in one and ileocystoplasties in four). No metabolic problems, no bladder perforations or malignancies were observed. The results on continence were good and, with the one exception reported, the condition of the upper urinary tract following surgery was satisfactory in all patients. CONCLUSION: Augmentation cystoplasty is of use in the treatment of patients with bladder exstrophy when staged functional reconstruction is unsuccessful. This technique greatly reduces the indications for urinary diversion and can be used in the surgical treatment of EEC. The only disadvantage is that clean intermittent catheterization must be performed, sometimes only temporarily, but patient acceptance is usually high.

Bladder Exstrophy↗

Duplication of the entire small intestine--a case report.

The authors report a case of duplication of the entire small intestine manifested by frequent haemorrhages originating from a peptic ulcer located distally to the duplication. The peptic ulcer was provoked by the ectopic gastric mucosa of the duplication. Surgery consisted "stripping" the mucosa of the duplicated tract according to Wrenn's technique. Other surgical methods for treating this malformation are discussed in detail. The literature describing similar cases is reviewed.

Choristoma↗