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Biomedical subjects

C T Albanese

Publications and source records attributed to C T Albanese.

At least 19 recordsLinked to original sources

Fetal neurenteric cyst causing hydrops: case report and review of the literature.

Routine prenatal ultrasound revealed a unilocular cystic mass associated with upper thoracic hemivertebrae that grew to 6 cm at 28 weeks and was associated with hydrops. A thoraco-amniotic shunt decompressed the cyst and resolved the hydrops, but the shunt occluded 17 days later. Preterm labour led to vaginal delivery at 31 2/7 weeks. Postnatally, the cyst was decompressed by thoracentesis due to respiratory distress. It was resected on day four of life. Severe tracheobronchomalacia was present post-operatively, presumably due to prenatal mass effect of the cyst. At one year of age, the child has recovered completely without adverse respiratory or neurological sequelae.

Adult

A strategy for primary reconstruction of long gap esophageal atresia using neonatal colon esophagoplasty: a case report.

Treatment options for long gap esophageal atresia without tracheoesophageal fistula generally require several stages over many months. An early neonatal vascularized conduit would allow a tension-free anastomosis, but the precarious blood supply of the neonatal bowel makes mobilization and immediate interposition hazardous. This report describes the successful application of a strategy for primary reconstruction in the neonate using a short piece of colon mobilized into the mediastinum for subsequent delayed anastomosis.

Colon

One-stage correction of high imperforate anus in the male neonate.

PURPOSE: The aim of this study was to examine the feasibility, safety, and short-term outcome of complete one-stage repair of high imperforate anus in the newborn boy. METHODS: A retrospective review was conducted of five full-term male infants who underwent posterior sagittal anorectoplasty without a colostomy within the first 48 hours of birth. Preoperative imaging was performed to assess associated anomalies. All infants underwent cystoscopy before the perineal operation to determine the level of the urinary tract fistula, if present. After completion of the anoplasty, all were turned supine and the colon irrigated free of meconium. Follow-up ranged from 10 to 24 months. RESULTS: Laparotomy was not required for any patient. Three patients had a rectoprostatic urethral fistula, one a rectovesical fistula, and one no fistula (common wall at level of prostate). Tapering rectoplasty was required for only the one patient with a rectovesical fistula. There were no intraoperative complications. All patients passed stool within 12 hours after operation and took full feeding by 48 hours. The average hospital stay was 7 days. Postoperative and stenosis occurred in one patient secondary to parental noncompliance with the postoperative dilation regimen. There were no perineal wound complications. All patients have a strong urinary stream and defecate spontaneously without the aid of oral medication or rectal stimulation or enemas. CONCLUSIONS: One-stage repair of high imperforate anus in the male neonate is feasible without short-term genitourinary or gastrointestinal morbidity. Whether it is preferable compared with a delayed (two or three stage) repair depends on ultimate long-term anorectal function, which cannot be assessed for several years.

Anal Canal

Transabdominal oxygenation using perfluorocarbons.

PURPOSE: Evaluation of the intraabdominal (intraperitoneal and intraluminal) administration of oxygen-saturated perfluorocarbon on both portal and arterial blood oxygenation. METHODS: Eight male rabbits were divided into the test (n = 5) and control (n = 3) groups. Each underwent intrajejunal, intraperitoneal, and intravascular (artery, portal vein) catheter placements along with ligation of the duodenum and the terminal ileum under general anesthesia. The test group received oxygen-saturated perfluorotripropylamine (FTPA), and the control group received oxygen desaturated FTPA. The oxygen delivery was assessed by serial blood gas measurements before and after the administration of FTPA. RESULTS: The administration of oxygen-saturated FTPA significantly increased the partial pressure of oxygen within both the arterial and the portal venous blood (PaO2, PpVO2) without significant changes in PCO2 values. Oxygen desaturated FTPA failed to show any effects on blood gas values. Compared with oxygen desaturated FTPA, oxygen-saturated FTPA increased PaO2, PpVO2, and oxygen saturation (artery, portal vein) significantly at some, but not all of the time-points measured. CONCLUSIONS: The intraabdominal administration of saturated FTPA improved both the portal venous and the arterial oxygenation. This new mode of oxygenation may be helpful as an adjunct to conventional oxygen delivery systems.

Animals

Perineal one-stage pull-through for Hirschsprung's disease.

PURPOSE: The aim of this study was to present the strategy of a one-stage repair of Hirschsprung's Disease (HD) performed via a transanal approach. METHODS: Ten consecutive neonates and one toddler underwent transanal repair for biopsy-proven HD. A rectosigmoid transition zone was suggested by contrast enema in all patients. The mean age at operation for the neonates was 4 days. A mucosal dissection was begun 0.5 cm proximal to the dentate line. Once the correct plane was established, up to 15 cm of bowel can be resected without ligating vessels or performing a transabdominal dissection. The proximal extent of dissection was delineated by the presence of ganglion cells seen on frozen section analysis. RESULTS: The mean operating time was 105 minutes. There were no intraoperative or postoperative complications. All children had the presence of ganglion cells confirmed postoperatively on permanent sections. The mean hospital stay was 2 days. All children averaged three to six bowel movements per day without oral or enema therapy. CONCLUSIONS: The perineal one-stage operative pull-through (POOP) procedure for Hirschsprung's disease is a quick and easy adaptation of a well-described technique of transanal mucosectomy. Long-term follow-up will be required to determine whether bowel function is better that that seen after traditional staged repairs.

Child, Preschool

Prenatal cellular transplantation.

Based on the ontogeny of fetal immunologic development, the strategy of fetal cellular transplantation may prove to be the most physiological way to achieve replacement of abnormal hemoglobin, immune cells, or enzyme defects diagnosed early prenatally. Similarly, if cellular transplantation can induce tolerance, postnatal solid organ transplantation may be performed without the need for chronic immunosuppression or with a reduced risk of graft-versus-host disease. This review presents experimental data from the late 1940s until the present for both small and large animal models; it also describes the limited clinical experience with prenatal cellular transplantation.

Animals

Surgical treatment for fetal disease. The state of the art.

Sophisticated imaging and fetal sampling techniques have defined the natural history and pathophysiologic characteristics of many previously mysterious conditions of newborn. Although most prenatally diagnosed malformations are best managed by appropriate medical and surgical therapy after delivery, an increasing number of simple anatomic abnormalities with predictably devastating developmental consequences have been successfully corrected before birth. Many of the technical intricacies of open fetal surgery have been solved, but preterm labor remains an omnipresent risk to the mother and fetus. The recent development of minimally invasive techniques to treat the fetus prenatally has significantly lessened preterm labor. Minimally invasive surgical techniques, in combination with new tocolytic strategies, promise to extend the indications for fetal surgical intervention.

Congenital Abnormalities

Fetal liver position and perinatal outcome for congenital diaphragmatic hernia.

Despite advances in postnatal care, patients born with a congenital diaphragmatic hernia (CDH) suffer substantial morbidity and mortality. The present study was undertaken to determine the prognostic influence of prenatally-diagnosed liver herniation in the hemithorax in fetuses with CDH. The medical records of 48 patients evaluated for a prenatally-diagnosed left CDH were retrospectively reviewed. Patients were analysed according to the position of the liver by prenatal ultrasound; 32 fetuses had a major portion of the liver herniated into the left hemithorax ('liver up') and 16 had an intra-abdominal liver ('liver down'). Liver position was determined using colour-flow Doppler ultrasonography. There were two fetal deaths in the liver-up group and one in the liver-down group. The liver-up group more frequently required extracorporeal membrane oxygenation (ECMO) support (53 per cent) compared with the liver-down group (19 per cent). Postnatal survival was significantly less in the liver-up group (43 per cent) vs. the liver-down group (93 per cent). Fetuses with congenital diaphragmatic hernia and liver herniated into the hemithorax have a much worse prognosis than similarly afflicted fetuses without liver herniation. Prenatal ultrasonographic diagnosis of congenital diaphragmatic hernia allows for preparation for a critically ill newborn and aids in prenatal family counselling.

Adolescent

A surprising histological evolution of preterm sacrococcygeal teratoma.

In fetuses with sacrococcygeal teratomas (SCT), the development of hydrops is a harbinger of imminent fetal demise. In such instances, tumor debulking, either at fetal surgery or postnatally after urgent cesarean section, may prove lifesaving. Definitive SCT resection is carried out in the newborn period when the infant has stabilized. In five such patients, retrospective comparison of the SCT histological findings from the initial debulking procedure and later definitive resection or, in one case, autopsy results, showed a surprising change. After the initial debulking procedure, SCT histology findings from three patients were grade 3 immature teratoma without malignant elements, and in two patients grade 3 immature teratoma with nests of malignant yolk sac elements. In the three patients having grade 3 immature tumor without malignant elements, histological examination after definitive resection (two cases) and autopsy (one case) showed only fibrosis; there was no evidence of the SCT despite the fact that residual tumor had clearly been left at the initial debulking. In the patients in whom the initial histology finding was grade 3 immature with malignant elements, SCT histology results from the definitive resection showed grade 3 immature teratoma without malignancy in one case, and mature, benign tissue in the other. Preterm SCT debulking may induce tumor maturation or, alternatively, maturation of SCT that occurs naturally during gestation may be observed.

Female

Anterior cervical approach for repair of laryngotracheoesophageal cleft.

Laryngotracheoesophageal cleft is a very rare congenital malformation characterized by a long midline communication between the esophagus and trachea extending from the larynx down the trachea for a variable distance. The most common repair strategy involves separation of the trachea and esophagus by a lateral approach via both a thoracotomy and a neck incision. The authors report a case of complete repair (without thoracotomy) via a neck incision through the anterior wall of the trachea.

Abnormalities, Multiple

Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion.

BACKGROUND/PURPOSE: Fetuses with congenital diaphragmatic hernia (CDH) who have a "poor prognosis" with postnatal treatment now can be identified on the basis of liver herniation, early diagnosis (before 25 weeks' gestation) and a low lung-to-head ratio (LHR). Because complete in utero repair proved unsuccessful for this group, the strategy of temporary tracheal occlusion was developed to gradually enlarge the hypoplastic fetal lung. The purpose of this study is to compare the outcome of patients in the poor-prognosis group treated by one of three methods: (1) standard postnatal care, (2) fetal tracheal occlusion via open hysterotomy, and (3) the recently developed video-fetoscopic (Fetendo) technique of tracheal occlusion without hysterotomy. METHODS: In the past 3 years, 34 of 86 fetuses with an isolated left CDH met criteria for the poor-prognosis group. Thirteen families chose postnatal treatment at an extracorporeal membrane oxygenation (ECMO) center, 13 underwent open fetal tracheal occlusion, and eight underwent fetoscopic tracheal occlusion. RESULTS: The survival rate was 38% in the group treated by standard postnatal therapy, 15% in the open tracheal occlusion group, and 75% in the Fetendo group. There were less postoperative pulmonary complications noted in mothers who underwent the Fetendo procedure versus the open tracheal occlusion. All but one Fetendo clip patient had a striking physiological response demonstrated by sonographic enlargement of the small left lung that was documented postnatally by plain radiographs and its subjective appearance during repair of the CDH. In contrast, only 5 of the 13 open tracheal occlusion patients demonstrated lung growth. CONCLUSION: Fetuses with a left CDH who have liver herniation and a low LHR are at high risk of neonatal demise and appear to benefit from temporary tracheal occlusion when performed fetoscopically, but not when performed by open fetal surgery.

Adult

Successful treatment of liver hemorrhage in the premature infant.

Intraoperative neonatal liver hemorrhage usually results in exsanguination and death. The parenchyma of a neonatal liver is fragile, and the capsule is very thin, unlike that in the pediatric and adult patient. This renders the treatment of a neonatal liver fracture almost universally unsuccessful. The current report describes two cases of successful management of intraoperative neonatal liver hemorrhage during surgery for necrotizing enterocolitis (NEC).

Enterocolitis, Pseudomembranous

Tissue repair in the fetal intestinal tract occurs with adhesions, fibrosis, and neovascularization.

Cutaneous wound healing in the fetus can occur in a nonfibrotic, regenerative manner. However, other fetal tissues such as bone and stomach heal with scar formation. In light of potential ramifications for adult hollow visceral scarring (biliary and intestinal strictures), this study was undertaken to determine if tubular visceral tissue repair in the fetus is regenerative or fibrotic. Fetal rabbits underwent laparotomy on day 24 of gestation, during which a controlled intestinal enterotomy was created and suture repaired immediately using microsurgical techniques. Maternal rabbits and adult male rabbits also underwent enterotomy and repair. After 5 days all animals were sacrificed and the wounds analyzed histologically by a pathologist in a blinded fashion. All animals demonstrated a similar degree of peri-intestinal adhesion formation. Fetal and maternal wounds contained fibroblastic and smooth muscle cell proliferation, mild inflammatory infiltration, and new blood vessel formation. The male adult wounds demonstrated a more pronounced fibrovascular healing response. Immunohistochemical staining for CD31 (endothelial cell marker) was quantitated on a scale of 0 to 4+, indicating degree of neovascularization. The mean scores for the fetal and maternal groups were similar (1.70 +/- 0.68 and 1.23 +/- 1.07 respectively), but were significantly greater for male adults (2.93 +/- 0.12; p = 0.001 by analysis of variance). The results of this study indicate that hollow visceral tissue repair in the fetal rabbit intestine occurs in a similar fibrotic manner as adult healing. This provides further evidence that regenerative healing in the fetus is not ubiquitous. Differences in the degrees of fibrosis and neovascularization between adult male and pregnant female wounds deserve further investigation.

Animals

Transmucosal passage of bacteria across rat intestinal epithelium in the Ussing chamber: effect of nutritional factors and bacterial virulence.

Transmucosal passage of bacteria across the intestine, the essential and prerequisite step for bacterial translocation, cannot be effectively studied using in vivo models of translocation. We have adapted the Ussing chamber into a fresh, sterile organ culture system that can facilitate the study of bacterial-epithelial interactions. Intestinal membranes were mounted in the Ussing chamber and perfused with a solution rich in putative mucosal micronutrients. The transmembrane potential difference was constantly monitored as a marker of intestinal integrity. Transmucosal passage of various bacteria across the normal intestinal epithelium was quantitated, and the mucosal membrane was examined by light and transmission electron microscopy. The addition of potassium cyanide to the mucosal perfusate resulted in an irreversible loss of potential difference. Oxygen deprivation also led to a precipitous drop in potential difference, but it was reversible with prompt reoxygenation. In contrast, intestinal membranes perfused with a solution consisting of Dulbecco's modified Eagle's medium + 20 mM glutamine maintained their potential difference for a sustained period (>180 min). Both the viability and structural integrity of the ileal intestinal membrane were maintained in culture ex vivo using this perfusate. Qualitative differences were observed in the mechanism of transmucosal passage of mild to moderately virulent bacteria such as Escherichia coli C-25 and Proteus mirabilis M-13, which pass through the intestinal epithelium without causing overt damage to the mucosa, and more virulent organisms such as Salmonella typhimurium, which cause extensive mucosal damage by light and transmission electron microscopy. The Ussing system should provide a useful model of intact organ culture for the study of the mechanisms of bacterial translocation and the pathogenesis of enteric infections.

Animals

Hypercalcemia in malignant paraganglioma due to parathyroid hormone-related protein.

A 15-year-old boy had hypercalcemia in association with malignant retroperitoneal paraganglioma. He had suppressed circulating levels of intact parathyroid hormone, whereas parathyroid hormone-related protein (PTHrP) immunoreactivity was elevated in plasma. Both the serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels were normal. Preoperatively the patient required control of hypercalcemia with intravenous pamidronate therapy. His circulating calcium and PTHrP concentrations became normal after a successful surgical resection of the primary retroperitoneal tumor. To our knowledge, this is the first reported case of elevated PtHrP levels in a patient with paraganglioma which resolved postoperatively.

Adolescent

Computed tomography grade of splenic injury is predictive of the time required for radiographic healing.

It is largely unknown when a child who has suffered a splenic laceration can return to full unrestricted activity. The purpose of this prospective study is to establish whether the grade of splenic injury is predictive of the length of time required for radiographic healing, and to determine whether there are any adverse long-term sequelae after resumption of unlimited activity. Sixty-nine patients underwent successful nonoperative management (NOM) of computed tomography (CT)-documented splenic injury over a 4-year period. Fifty-eight patients completed follow-up. Mean age was 9.8 years (range, 1 to 17) and mean injury severity score (ISS) was 14.4 (range, 4 to 38). Mechanisms of injury were motor vehicle accident (n = 11), motor vehicle pedestrian (n = 5), falls (n = 13), bike crashes (n = 12), sports (n = 8), all-terrain vehicle (n = 4), and horse (n = 5). The CT-documented injury was identified by discharge ultrasound scan (US) in all cases. There were no long-term complications. Mean time to US healing in grade I (n = 9), II (n = 26), III (n = 19), IV (n = 4) injuries was 3.1, 8.2, 12.1, and 20.7 weeks, respectively. P values were significant (P < .01) in all cases when compared with the next lower injury grade. The time to radiographic healing is directly proportional to the severity of the splenic injury. There was excellent correlation between the initial CT scan and identification of the injury on the discharge US. No long-term complications leg, delayed splenic rupture, splenic pseudocyst) were seen in this study. Pediatric patients who have suffered splenic injury can safely return to full unrestricted activity when the US documents healing.

Adolescent

Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia.

BACKGROUND: Accurate prenatal prediction of outcome for fetuses who have congenital diaphragmatic hernia (CDH) is very difficult. The authors previously reported a retrospective analysis of risk factors for fetal CDH and proposed a new index of severity: the lung-to-head ratio (LHR). The authors now report a prospective study to test whether this new index predicts neonatal outcome. METHODS: Fifteen patients who had left-sided CDH were sonographically evaluated at the University of California, San Francisco (UCSF) and followed prenatally and postnatally. LHR was measured at 24 to 26 weeks' gestation. Outcome variables included survival and the need for extracorporeal membrane oxygenation (ECMO). RESULTS: Overall survival was 47%. LHR ranged from 0.62 to 1.86. No patient with an LHR of less than 1.0 (n = 3) survived despite ECMO, whereas all patients with an LHR greater than 1.4 survived (n = 4), one requiring ECMO. LHR values between 1.0 to 1.4 were associated with 38% survival (n = 8), 75% requiring ECMO. Overall, survivors had a mean LHR of 1.4 +/- 0.33 and nonsurvivors, 1.05 +/- 0.3 (P < .05). CONCLUSION: The LHR is a useful index to help predict neonatal outcome in patients who have left-sided CDH.

California