Biomedical subjects
Publications and source records attributed to P G Gamba.
Isolated congenital urethrocutaneous fistula is uncommon, and its repair has been associated with high incidence of recurrence. However, the use of buccal mucosal graft offers a satisfactory closure after previous failures. We report a new case in whom we adopted the buccal mucosal urethral replacement to treat the recurrence.
Urokinase is an enzyme with a fibrinolytic effect that facilitates pleural empyema drainage through a chest tube. The aim of this study was to assess the risk of pneumothorax, the need for pleural debridement surgery, the persistence of fever, and the number of days in hospital in a group of children with parapneumonic pleural empyema treated with urokinase. This was an uncontrolled retrospective study on children suffering from parapneumonic empyema. Data collected on 17 children treated with urokinase were compared with 11 children treated prior to the advent of urokinase (the "historic" group). The urokinase was instilled in the pleural cavity over a period ranging from 2-8 days, amounting to a median total dose per kilogram of body weight of 18,556 IU (range, 7,105-40,299). Surgical treatment of the empyema involved drainage tube placement and/or debridement of the pleural cavity. Three children developed pneumothorax during their hospital stay, and one more case occurred 6 months after the child had recovered from his empyema; there were 3 cases of pneumothorax during the acute phase in the "historic" group (P = 0.54). Five children in the urokinase group were debrided and 12 were only drained, as opposed to 9 and 2, respectively, in the "historic" group (P = 0.02). The overall hospital stay was 17 days for the urokinase group, and 24 for the "historic" group (P = 0.02). No bleeding or other major complications were reported in the group treated with urokinase. In conclusion, urokinase treatment does not carry a risk of pneumothorax, while it does reduce hospital stay and the need for pleural debridement.
In the surgical repair of congenital abdominal-wall defects (AWD), the ready availability of a non-immunogenic and non-prosthetic biomaterial that could guide the regeneration of normal tissue is a fascinating possibility. Biomaterials are already in use, but in our experience, an acellular matrix (ACM) can stimulate exact regeneration of the absent tissue. We explored the possibility of using an ACM to repair a muscular AWD in an animal model. Male New Zealand white rabbits (3-4 kg, n = 18) were anesthetized and the abdominal wall was shaved and scrubbed; a vertical incision was made in the left lower quadrant and a large patch of external-oblique muscle was resected (3 x 3 cm). The animals underwent reconstruction with homologous diaphragm acellular matrix (HDAM) grafts that were previously prepared using a detergent enzymatic method. The patches were evaluated histologically at 9 (n = 6), 40 (n = 6), and 90 (n = 6) days post-surgery in each group; moreover, 90 days post-surgery an electromyogram (EMG) (n = 6) of the implanted matrix was recorded. Histologic analysis demonstrated that the HDAM supported fibroblast migration, deposition of newly-formed collagen, and neovascularization. No signs of necrosis, or evidence of skeletal-muscle-cell ingrowth were detected. The EMG revealed minimum muscular electrophysiologic activity, probably due to muscle underlying the patch. The HDAM we employed was thus not able to produce reconstruction of the skeletal muscle, and was progressively remodeled into fibrous tissue. Since the ultimate reason for failure of muscle regeneration is a lack of myogenesis, future studies will use ACMs preconditioned by various regulators of myoblast proliferation and differentiation.
We report the case of a 14-month-old child with Kasabach-Merritt Syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.
Aim of the study was to present the first two Italian cases of C-section performed with the EXIT procedure (EX-utero Intrapartum Technique). Deliveries were performed at the Division of Obstetrics and Gynecology of the Hospital of Padua in cooperation with the Pediatric Surgery Department, both tertiary care centers. The first case was a twin with a huge neck mass (cystic hygroma) and the second a fetus with an oropharyngeal mass (epignathus). Airway patency could have been compromised at birth in both of them. EXIT procedure consists in securing the airway of the fetus partially delivered and still connected with the placenta. This technique leaves an intact feto-placental circulation and guarantees a normal fetal oxygenation while fetal airway patency is secured. Both the fetuses were successfully intubated and the C-section ended up in a short period of time without maternal and fetal complications. The EXIT technique, performed for the first time in 1989 and now in many centers abroad, can be considered a safe procedure as long as a multidisciplinary approach is carried out. The EXIT procedure is indicated whenever fetal airways can be compromised at birth, that is when oropharyngeal masses, laryngeal atresia, cystic hygroma and goiter are encountered during prenatal ultrasound.
Congenital respiratory tract-biliary fistula, including tracheo- and broncho-biliary fistulae, are rare developmental anomalies. To date, only 18 cases have been reported. We present two additional cases that came to our attention after a long and difficult attempt to make a diagnosis. After surgical excision of the tract both children have remained symptom-free for 6 and 4 years, respectively. Bronchoscopy allows an early diagnosis, but patients also have to be investigated for associated biliary tree malformations.
Urethral reconstruction following failed hypospadias repair or post-traumatic chronic stricture requires adequate amounts of tissue. Many surgical techniques utilizing different types of biological tissues have been attempted: (a) vascularized skin flaps from the prepuce, scrotum or penile shaft; (b) full-thickness free skin grafts; (c) vesical or buccal mucosa grafts; (d) ureter; artery; vein and appendix tissue. More recently, biodegradable polymers have also been used as delivery vehicles of urothelial cells in animals. It has been demonstrated that the implant of an acellular tissue matrix in the bladder can guide the regeneration of urothelium, blood vessels, smooth muscle and nerves. The aim of this study was to create an experimental model of urethral defect, and then repair it by implanting homologous acellular aortic grafts as urethral substitutes. An acellular matrix was obtained by detergent enzymatic treatment of rabbit thoracic aorta. The growth of urethral epithelium was verified in vitro, and homologous acellular vessels were then implanted in rabbits, bridging a previous surgical urethral defect. The outcome of reconstructive surgery was evaluated histologically at 10 days, 3 weeks, 3 and 12 months. As the time after surgery increased, the neourothelium became less thick, signs of inflammatory response disappeared, and the orientation of collagen fibrils and smooth muscle fascicles resembled that of a normal urethra. The implants displayed abundant vascularization, and the luminal surface started to become irregular. Acellular blood vessels may represent a promising approach to urethral defect therapy for different reasons: (a) unlimited availability, (b) readily obtainable in different lengths and gauges, (c) the potential for being organized as tissue bank, and (d) that just one simple surgical procedure is needed. Nevertheless, before this technique can be applied in humans, it must be tested in more species and animals.
Lipid-based vectors are a promising tool for gene therapy applications. Several studies have reported their use in vivo to transfect different organs. Few data, however, are available about lipid-mediated gene transfer in skeletal muscle. Here we report the initial results obtained after systemic administration of lipopolyplexes based on the DODAC cationic lipid in an animal model of muscle regeneration. In particular, we compared three routes of administration: intravenous (i.v.), intracardiac (IC) and intra-arterial (IA). Analysis of reporter gene expression (luciferase) showed that regenerating muscle is more efficiently transfected in all cases and that IA injection is by far the best approach.
Hypertrophic pyloric stenosis (HPS) is rare in premature infants. We report a case of HPS in an extremely low birth weight neonate (28 weeks of gestation, 622 gr), discovered on the 10th week of life. Although the diagnosis and treatment of pyloric stenosis has ben well established for many years, the presentation in preterm babies is atypical and the diagnosis often delayed. A brief review of literature is included.
PURPOSE: We describe 3 cases of testicular cystic dysplasia that were diagnosed only by sonography to avoid an invasive approach. MATERIALS AND METHODS: Three patients 5, 8 and 12 years old, respectively, had increased testicular volume and/or intermittent pain. Sonographic examination of the testis by high frequency (7.5 mHz.) probes showed the typical onset of testicular cystic dysplasia, characterized by several small focal or diffuse intraparenchymal cystic formations. RESULTS: Biopsy or orchiectomy was not considered. At 16, 18 and 24 months of followup, respectively, testicular pain was absent in our 3 cases and sonographic findings were unchanged. CONCLUSIONS: Clinical and sonographic followup is considered sufficient to evaluate possible changes in the clinical course of this pathological condition which, although benign, still remains to be defined.
Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported--all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed.
BACKGROUND: Medical treatment of gallstones with ursodeoxycholic acid (UDCA) or chenodeoxycholic acid (CDCA) has not been evaluated in children. AIM: The purpose of this study was to assess the effectiveness of UDCA in the treatment of gallstones in children. METHODS: UDCA was used to treat 15 patients, (7 boys and 8 girls; mean age, 7.8 years; range, 3 months to 15 years) for 1 year. All had radiolucent stones with a maximum diameter of 10 mm and a normally contractile gallbladder. RESULTS: The stones disappeared completely in two children but returned later. All symptomatic patients became symptom free. CONCLUSION: UDCA is ineffective in the treatment of gallstones in children except in terms of relieving symptoms while on treatment.
Nodular regenerative hyperplasia (NRH) of the liver is a condition of unknown origin, rarely occurring in children, usually accidentally discovered, described in association with a variety of clinical conditions and drugs. Confusion with other types of hepatic masses may pose a problem and for this reason NRH is considered a "tumor-like lesion." Histologically it consists of single or multiple regenerative foci. Hepatic failure and rupture of the liver have been rarely described as complications in adults, and not in children, and malignant transformation has not been demonstrated. Neither surgical removal nor other treatment is needed. These features are described as they were found in two patients we encountered.
Although rarely observed, vascular diseases in children constitute a complex clinical problem. Until recently the diagnostic approach to infant vascular diseases was based on invasive methods such as arteriography, but now the use of ultrasonographic methods such as Doppler c.w. and Echo Doppler Color Flow (EDCF) in angiology can offer new and interesting application even in the pediatric age range. In the present study 15 children affected by vascular diseases (eight with arteriovenous fistulas (AVF), two with pseudoaneurysms, and five with angiomas or cysts) either of iatrogenic or congenital etiology, were examined by Doppler c.w. and EDCF. In three additional cases a suspected vascular disease was not confirmed. The data obtained by Doppler c.w. and EDCF proved useful to perform a preoperative diagnosis; however some diagnostic criteria differed from those described in adulthood, as in the AVF cases, for instance, we observed the prevalence of indirect signs such as alterations of venous flow, and the presence of "multicolored speckled mass" and of "the spreading of the color in extravasal space" rather than a direct connection between an artery and a vein. Also pseudoaneurysms were easily detected by EDCF. In two cases of congenital AVF, the diagnosis was confirmed by arteriography. Surgery was performed in 9 children, 7 with AVF and 2 with pseudoaneurysm; in all cases, the vascular defect detected by ultrasonography was visualized and corrected. In 7 cases EDCF was also used in the follow-up. In conclusion this study suggests a possible more extensive use of ultrasonographic methods in the diagnostic approach to vascular diseases in pediatric age groups, and points out their validity in screening very young patients and in their surgical follow-up.
Fine-needle aspiration cytology (FNAC) is now considered a useful tool in the evaluation of adult patients with mass lesions. We reviewed the experience with FNAC in our Paediatric Surgical Department. One hundred and eleven FNACs were performed in children with a superficial mass and no definite diagnosis. All the exams were done by the same physician using a fine needle and no anesthesia. We routinely used May-Grüwald-Giemsa and Papanicolaou staining. Patients age ranged from 20 days to 17 years, with a mean age of 6.5 years. A clinically benign pathology was cytologically confirmed in 90 cases (81%). All children did well at follow-up. Malignancy was diagnosed in eight cases (7.2%) and in all was confirmed with a surgical biopsy. In nine children (8.1%) the specimen was considered insufficient for definite diagnosis. In four cases (3.6%) the pathologist diagnosed a possible malignancy that was excluded at surgical biopsy. The sensitivity was 100% and the specificity was 96%. Our experience confirms that FNAC is a fast, cheap, simple, and accurate diagnostic method and should be used for screening in all children with doubtful superficial masses.
This study is based on the experience of the Italian Cooperative Studies "AIEOP-RMS 79 and RMS 88". Between October 1979 and December 1989 299 children under 15 years of age with a histologically proven rhabdomyosarcoma (RMS) were examined. We analyzed the problem of the paratesticular localization paying particular attention to the role of surgery in dealing with paraaortic nodes. In the first study (RMS 79) the surgical exploration of retroperitoneal nodes was required: this procedure performed in 11 out of 14 patients affected by paratesticular RMS confirmed the negative data observed with diagnostic investigations. In RMS 88 study the sampling of retroperitoneal nodes was not required in cases with negative radiological findings but suggested in those cases with uncertain signs; two out of 8 patients underwent a laparotomy. In these two cases the results were negative. These 22 children were treated with an adjuvant chemotherapy after surgery and at present they show no evidence of disease.
The article describes a rare case of congenital A-V fistula between descending aorta and hemiazygos vein in a 9-year old girl. Differential diagnosis in these cases is difficult and only sonography and angiography can lead to a correct diagnosis. The recent literature of this subject is reviewed.
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