Local Delivery of Basic Fibroblast Growth Factor Increases Both Angiogenesis and Engraftment of Hepatocytes in Tissue-engineered Polymer Devices

Transplantation. May, 2002  |  Pubmed ID: 12042644

We investigated heterotopic hepatocyte transplantation on biodegradable polymers as a potential treatment for end-stage liver disease. The primary problem has been insufficient engraftment of transplanted cells partly because of insufficient vascularization. Increasing vascularization through locally delivered angiogenic factors may increase angiogenesis and hepatocyte engraftment.

Management of Congenital Esophageal Stenosis

Journal of Pediatric Surgery. Jul, 2002  |  Pubmed ID: 12077763

The authors report the incidence, diagnosis, and treatment methods of congenital esophageal stenosis (CES) at their institution.

Cholelithiasis and Perforated Gallbladder in an Infant

Journal of Pediatric Surgery. Sep, 2002  |  Pubmed ID: 12194140

An infant presented with abdominal distension and failure to thrive. This patient was a 3.5-month-old, ex-26-week premature infant at the time of presentation who required supplemental parenteral nutrition until day 9 of life. Workup found ascites and a complex cystic mass in the porta hepatis. A perforated gallbladder with gallstones was found at laparotomy. The gallstones were removed, and the bed of the gallbladder was drained. Output from the drain was minimal by postoperative day 6, and the drain subsequently was removed. The child did well postoperatively; hematologic and metabolic workups were unrevealing. Perforation of the gallbladder is an exceedingly uncommon finding in infants. Appropriate management includes laparotomy and drainage of the gallbladder bed.

Selective Reduction of Acardiac Twin by Radiofrequency Ablation

American Journal of Obstetrics and Gynecology. Sep, 2002  |  Pubmed ID: 12237640

Acardiac/acephalic twinning is a rare anomaly in which a normal "pump" twin perfuses an acardiac twin, which results in twin reversed arterial perfusion sequence. A novel technique for selective reduction and obliteration of blood flow in the acardiac twin is described.

Fetal Endoscopic Surgery: Lessons Learned and Trends Reviewed

Journal of Pediatric Surgery. Dec, 2002  |  Pubmed ID: 12483634

Fetal surgery is performed increasingly with minimal access approaches. The authors report their experience with fetal endoscopic procedures (fetendo) with emphasis on changing techniques and outcome trends.

Spectrum of Intrapartum Management Strategies for Giant Fetal Cervical Teratoma

Journal of Pediatric Surgery. Mar, 2003  |  Pubmed ID: 12632365

The management of the fetus with a large neck mass that obstructs the airway remains a clinical challenge. The authors review their experience with giant fetal cervical teratoma and discuss options for management.

Resolution of Hydrops Fetalis in Congenital Cystic Adenomatoid Malformation After Prenatal Steroid Therapy

Journal of Pediatric Surgery. Mar, 2003  |  Pubmed ID: 12632377

Development of hydrops fetalis in fetuses with congenital cystic adenomatoid malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented.

Thoracoscopic Lobectomy for Prenatally Diagnosed Lung Lesions

Journal of Pediatric Surgery. Apr, 2003  |  Pubmed ID: 12677564

The aim of this report is to assess the technique and outcome of thoracoscopic lobectomy for asymptomatic prenatally diagnosed lung lesions.

Comparison of Drainage Techniques for Biliary Atresia

Journal of Pediatric Surgery. Jul, 2003  |  Pubmed ID: 12861527

Traditional Kasai portoenterostomy and porto-appendiceal duodenostomy have been utilized for biliary atresia. Differences in outcome between patients who underwent either Kasai portoenterostomy or porto-appendiceal duodenostomy were compared.

In Utero Repair of Myelomeningocele: Experimental Pathophysiology, Initial Clinical Experience, and Outcomes

Archives of Surgery (Chicago, Ill. : 1960). Aug, 2003  |  Pubmed ID: 12912746

Experimental work raises the possibility that in utero repair of myelomeningocele (MMC) may improve lower extremity, bladder, and bowel function, ameliorate the Arnold-Chiari malformation, and decrease the need for postnatal shunting.

A Randomized Trial of Fetal Endoscopic Tracheal Occlusion for Severe Fetal Congenital Diaphragmatic Hernia

The New England Journal of Medicine. Nov, 2003  |  Pubmed ID: 14614166

Experimental and clinical data suggest that fetal endoscopic tracheal occlusion to induce lung growth may improve the outcome of severe congenital diaphragmatic hernia. We performed a randomized, controlled trial comparing fetal tracheal occlusion with standard postnatal care.

Effect of Implantation Site on Hepatocytes Heterotopically Transplanted on Biodegradable Polymer Scaffolds

Tissue Engineering. Dec, 2003  |  Pubmed ID: 14670110

We investigated the engraftment of heterotopically transplanted hepatocytes in three sites: the subcutaneous space, the small intestinal mesentery, and the omentum to determine the optimal location for tissue-engineered liver constructs. Hepatocytes were isolated from inbred Lewis rats and placed on polymer constructs. Cell-polymer constructs were implanted into the subcutaneous space of the abdominal wall, the small intestinal mesentery, and the omentum of Lewis rats. One group of rats had undergone previous portacaval shunt. Animals were killed 2 or 4 weeks after implantation and the constructs were analyzed for engraftment, using computer-assisted morphometric analysis. Engraftment was greatest in the omentum with less engraftment in the mesentery. There was minimal engraftment in the subcutaneous space in all specimens. Prior portacaval shunt increased engraftment in the mesentery and the omentum, but not the subcutaneous space. The omentum is the most favorable bed for engraftment of hepatocyte-polymer tissue-engineered constructs and the addition of a portacaval shunt increases survival of transplanted hepatocytes in the omentum and mesentery.

The Ex Utero Intrapartum Treatment Procedure: Looking Back at the EXIT

Journal of Pediatric Surgery. Mar, 2004  |  Pubmed ID: 15017555

The ex utero intrapartum treatment (EXIT) procedure was developed originally for management of airway obstruction after fetal surgery, and indications have continued to expand for a variety of fetal anomalies. The authors review their single-institution experience with EXIT.

Initial Experience with Complex Laparoscopic Biliary Surgery in Children: Biliary Atresia and Choledochal Cyst

Journal of Pediatric Surgery. Jun, 2004  |  Pubmed ID: 15185200

Laparoscopic approaches are possible for many pediatric surgical diseases, including complex biliary disorders. The authors describe their early experience with laparoscopic procedures for biliary atresia and choledochal cysts.

Haploidentical Donor T Cells Fail to Facilitate Engraftment but Lessen the Immune Response of Host T Cells in Murine Fetal Transplantation

British Journal of Haematology. Aug, 2004  |  Pubmed ID: 15257710

The effects of donor T cells, or their CD8+ subset, on engraftment and tolerance induction in fetal transplantation were evaluated using an F1-into-parent mouse-model that does not permit a graft-versus-host effect. Gestational day 13 C57BL/6 (H-2Kb) fetuses were transplanted with B6D2F1 (H-2Kb/d) light density bone marrow cells (LDBMC) containing 1-2% T cells, T-cell depleted bone marrow cells (TDBMC, < 0.1% T cells), or TDBMC with enriched CD8+ T cells (CD8). Chimaerism levels in the peripheral blood, spleen and bone marrow were usually below 0.2% in all groups, indicating that T cells do not improve engraftment without a graft-versus-host effect. A significant, but transient, wave of donor cells was seen in the peripheral blood at 1 month of age in the CD8 and LDBMC groups. Relatively high levels of chimaerism (< 17%) were sometimes detected in the peritoneal cavities of recipients. T-cell tolerance specific to donor cells was evaluated in mixed lymphocyte cultures. The CD8 and LDBMC groups had significantly lower T-cell responses than untransplanted controls. These findings indicate that in utero transplantation of haploidentical donor CD8+ or CD3+ cells can help to lessen the immune response of host T cells towards donor cells. The persistence of donor cells in the peritoneal cavity also correlated with tolerance induction.

Infant Pulmonary Function in a Randomized Trial of Fetal Tracheal Occlusion for Severe Congenital Diaphragmatic Hernia

Pediatric Research. Nov, 2004  |  Pubmed ID: 15319458

Congenital diaphragmatic hernia (CDH) carries a high mortality risk secondary to pulmonary hypoplasia and respiratory failure. In experimental animals, fetal tracheal occlusion (TO) induces lung growth and morphologic maturation. We measured indicators of pulmonary function in 20 infants who were enrolled in a randomized trial of fetal TO as treatment for severe CDH [nine with conventional treatment (controls); 11 with TO]. We hypothesized that TO would improve lung function. At birth, the TO group had a lower mean gestational age (30.8 +/- 2.0 versus 37.4 +/- 1.0 wk; p=0.0002). All infants required assisted ventilation. Mortality did not differ between groups (64 versus 78%, TO and control, respectively; p=0.64). We measured respiratory mechanics at four study points: 1) first 24 h, 2) before CDH operative repair (5.9 +/- 2.2 d), 3) immediately after repair (7.0 +/- 2.2 d), and 4) before elective extubation (32.5 +/- 16.1 d). We calculated perioperative oxygenation index and alveolar-arterial oxygen difference to assess efficiency of pulmonary gas exchange. Data were analyzed by univariate and repeated measures techniques. Respiratory system compliance (Crs) was low. The rate of increase in Crs over the four study points was greater in the TO group than in control subjects. Crs in the TO group was significantly greater at study 2 (0.28 +/- 0.12 versus 0.17 +/- 0.04 mL.cm H2O(-1).kg(-1); p=0.02) and study 4 (0.93 +/- 0.45 versus 0.51 +/- 0.16 mL.cmH2O(-1).kg(-1); p=0.02). oxygenation index did not differ between groups, but alveolar-arterial oxygen difference was lower in the TO infants. We conclude that fetal TO for severe CDH results in modest improvements in neonatal pulmonary function that are of questionable clinical significance.

The Influence of Portoenterostomy on Transplantation for Biliary Atresia

Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. Oct, 2004  |  Pubmed ID: 15376306

After portoenterostomy (PE) for biliary atresia (BA), many patients suffer progressive deterioration of liver function and ultimately require liver transplantation. We retrospectively reviewed a single center's experience with pediatric liver transplantation for BA from 1988 to 2002. Sixty-six patients underwent 69 liver transplants for BA. Forty-two (63%) patients had previously undergone Kasai PE, 11 (17%) biliary appendicoduodenostomy (BAD), and 13 (20%) had no prior biliary drainage (NBD). The BAD procedure offered only short-term biliary drainage--the mean interval between PE and transplant was more than twice that for Kasai patients than for BAD patients (132 versus 49 weeks). The transplants included 11 cadaveric partial, 27 cadaveric whole, and 31 living related transplants. Three patients required retransplant. Prior PE did not increase the incidence of major perioperative complications or unplanned reexploration. After transplant, the 1-, 5-, and 10-year actuarial graft survival rates were 87%, 86%, and 80%, respectively. The 1-, 5-, and 10-year actuarial patient survival rates were 91%, 89%, and 83%. PE remains an important bridge to transplant. In conclusion, transplantation for BA offers excellent long-term graft and patient survival.

Prevention of Graft Rejection by Donor Type II CD8(+) T Cells (Tc2 Cells) is Not Sufficient to Improve Engraftment in Fetal Transplantation

Fetal Diagnosis and Therapy. Jan-Feb, 2005  |  Pubmed ID: 15608458

Tc2 cells, a subset of CD8(+) T cells, are able to facilitate engraftment in a murine model of postnatal allogeneic bone marrow transplantation. The purpose of this study was to evaluate whether Tc2 cells could improve engraftment in fetal transplantation.

Extrapleural Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula

Pediatric Surgery International. Apr, 2005  |  Pubmed ID: 15789240

Traditionally, open repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) required thoracotomy. Innovations in minimal access surgery have created a thoracoscopic technique resulting in violation of the pleural space. Most pediatric surgeons favor an extrapleural approach for open repair. We present a novel minimal access, extrapleural technique for repairing EA with TEF. A 2-day-old infant with EA and distal TEF underwent thoracoscopic extrapleural repair that utilized three ports. Initial creation of the extrapleural space was achieved through one of the port sites and was completed thoracoscopically. A thoracoscopic repair of EA with distal TEF was achieved within the extrapleural space. A small tear in the pleura was inadvertently created during the dissection. The child began feeding normally. At 1 year of age, the patient had dysphagia requiring a single esophageal dilatation. This is the first known report of an extrapleural thoracoscopic repair of EA with TEF. Although thoracoscopic repairs of EA/TEF have been previously reported, these were all done transpleurally. Many pediatric surgeons favor the extrapleural approach for two reasons: (1) containment of a potential leak within the extrapleural space, avoiding an empyema, and (2) easier transpleural access for future thoracic procedures.

Survival of Severe Congenital Diaphragmatic Hernia Has Morbid Consequences

Journal of Pediatric Surgery. Jan, 2005  |  Pubmed ID: 15868556

Fetal tracheal occlusion (TO) was developed in an attempt to enhance prenatal lung growth and improve survival in fetuses with severe congenital diaphragmatic hernia (CDH). We conducted a randomized, controlled clinical trial in 24 fetuses with severe left CDH (liver herniated into the thorax and low lung-to-head ratio) to compare survival after endoscopic fetal TO vs standard perinatal care (control) and prospectively followed up the 16 survivors (9 control, 7 TO) to compare neurodevelopmental, respiratory, surgical, growth, and nutritional outcomes.

Subcutaneous Endoscopically Assisted Ligation (SEAL) of the Internal Ring for Repair of Inguinal Hernias in Children: a Novel Technique

Journal of Pediatric Surgery. Jul, 2005  |  Pubmed ID: 16034766

Subcutaneous endoscopically assisted ligation (SEAL) is a technique for high ligation of the patent processus at the internal ring without a groin incision or dissection of the vas and vessels. Under endoscopic visualization through a single umbilical port, a suture is guided extraperitoneally around the internal ring, avoiding the vas and vessels. The safety, efficacy (recurrence risk), and cost-effectiveness of this unproven procedure must be tested against standard open repair in a trial.

Pre-emptive Placement of a Presealant for Amniotic Access

American Journal of Obstetrics and Gynecology. Sep, 2005  |  Pubmed ID: 16157137

Amniotic access for fetal diagnosis and therapy can lead to membrane leaks, separation, and preterm premature rupture of membranes. Morbidity limits the beneficial effects of fetal intervention. We propose to examine a novel preventive "presealant" membrane puncture technique and evaluate it in vitro.

Prosthetic Patches for Congenital Diaphragmatic Hernia Repair: Surgisis Vs Gore-Tex

Journal of Pediatric Surgery. Jan, 2006  |  Pubmed ID: 16410103

The sequelae of congenital diaphragmatic hernia (CDH) continue well beyond the perinatal period. Up to 50% of these patients have subsequent recurrent herniation or small bowel obstruction (SBO). A recent trend has been toward the use of bioactive prosthetic materials. We reviewed different patch closure techniques used for CDH repair at our institution and their association with these sequelae.

Long-term Follow-up After Thymectomy for Myasthenia Gravis: Thoracoscopic Vs Open

Journal of Pediatric Surgery. Jan, 2006  |  Pubmed ID: 16410107

The aim of this study was to determine if patients are in remission or weaning off medication after thymectomy for myasthenia gravis (MG) and to examine the thoracoscopic versus open approaches.

Prenatal Diagnosis of Familial Type I Choledochal Cyst

Pediatrics. Mar, 2006  |  Pubmed ID: 16452322

Familial choledochal cysts are extremely rare. High-resolution ultrasound now allows for the antenatal diagnosis of these anomalies. After delivery, elective surgical resection should be planned; however, increases in size, deterioration of liver function, and ascending cholangitis frequently force early intervention. We report an unusual occurrence of siblings with type I choledochal cysts and review the existing literature on cause, genetics, classification, diagnosis, and management of this disease.

Maternal Morbidity After Maternal-fetal Surgery

American Journal of Obstetrics and Gynecology. Mar, 2006  |  Pubmed ID: 16522421

There is a paucity of published data on the maternal risks of fetal surgical interventions. We analyzed maternal morbidity and mortality that were associated with different types of fetal intervention (open hysterotomy, various endoscopic procedures and percutaneous techniques) to quantify this risk.

Laparotomy Versus Peritoneal Drainage for Necrotizing Enterocolitis and Perforation

The New England Journal of Medicine. May, 2006  |  Pubmed ID: 16723614

Perforated necrotizing enterocolitis is a major cause of morbidity and mortality in premature infants, and the optimal treatment is uncertain. We designed this multicenter randomized trial to compare outcomes of primary peritoneal drainage with laparotomy and bowel resection in preterm infants with perforated necrotizing enterocolitis.

Monochorionic Twin Gestation Complicated by a Sacrococcygeal Teratoma with Hydrops--optimal Timing of Delivery Allows for Survival of the Unaffected Twin

Journal of Pediatric Surgery. Aug, 2006  |  Pubmed ID: 16863829

Sacrococcygeal teratomas are the most common tumors found in newborns and have a favorable outcome when diagnosed late in gestation. Solid, highly vascularized tumors diagnosed early in gestation have a higher incidence of hydrops fetalis and fetal demise. The following is a case report of a monochorionic twin gestation complicated by a solid highly vascular sacrococcygeal teratoma and the development of hydrops in the affected twin. In this case, serial ultrasound evaluations and timing of delivery optimized the outcome of the unaffected twin.

Spontaneous Rupture of Fetal Sacrococcygeal Teratoma

Fetal Diagnosis and Therapy. 2006  |  Pubmed ID: 16912491

With recent advances in technology, fetal sacrococcygeal teratoma is being diagnosed increasingly during the early prenatal period by ultrasound examination. In addition, early detection of tumor related complications such as polyhydramnios, congestive heart failure, hydrops, hemorrhage, urinary tract or bowel obstruction can be followed closely in utero. Active prenatal management can improve fetal perinatal outcome by allowing planned delivery for neonatal surgery [Chisholm, C.A. et al.: Am J Perinatol 1999;16:47-50] or in some cases, fetal intervention. Additionally, families can be counseled appropriately regarding the range of outcomes. We report a case of fetal sacrococcygeal teratoma Type I diagnosed at 20 weeks with a prominent vessel supplying the tumor mass. At 23 weeks, there was a sudden appearance of an additional lobular mass, consistent with intrauterine spontaneous ruptured of a sacrococcygeal teratoma mass.

Thoracoscopic Resection of Foregut Duplication Cysts

Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A. Oct, 2006  |  Pubmed ID: 17004883

Foregut duplications are rare entities that include both esophageal and bronchogenic cysts. The diagnosis of foregut duplication cyst is made most often from an incidental finding on chest radiograph, or due to respiratory compromise due to mass effect or infection. Treatment consists of complete resection. Recurrences are associated with incomplete resection. Six cases of foregut duplication cysts are presented that were resected thoracoscopically.

Fetal Intervention for Mass Lesions and Hydrops Improves Outcome: a 15-year Experience

Journal of Pediatric Surgery. Jan, 2007  |  Pubmed ID: 17208551

The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with fetal mass lesions can be halted and even reversed with fetal intervention and treatment of the underlying defect. We examined our patients with fetal mass lesions to evaluate survival after intervention.

Congenital Diaphragmatic Hernia Associated with Spinal Anomalies

Obstetrics and Gynecology. Feb, 2007  |  Pubmed ID: 17267867

Increased mortality is seen in patients with congenital diaphragmatic hernia who have associated anomalies, such as cardiac defects. We reviewed our series of patients with congenital diaphragmatic hernia and spinal anomalies to evaluate if their prognosis was altered.

Efficacy of Radiofrequency Ablation for Twin-reversed Arterial Perfusion Sequence

American Journal of Obstetrics and Gynecology. May, 2007  |  Pubmed ID: 17466701

We report our experience in the treatment of patients with twin-reversed arterial perfusion (TRAP) sequence using radiofrequency ablation to stop perfusion to the acardiac twin and protect the pump twin.

Obstructing Ileal Duplication Cyst Infected with Salmonella in a 2-year-old Boy: a Case Report and Review of the Literature

Journal of Pediatric Surgery. May, 2007  |  Pubmed ID: 17502171

Alimentary tract duplications (ATDs) are rare congenital anomalies, with an incidence of 1 in 4500 individuals. Patients with these lesions present in a variety of ways, and although histologically benign, ATDs can lead to lethal complications including volvulus, intussusception, or bowel obstruction. Irrespective of anatomical location, the epithelial lining of ATDs contains ectopic acid-secreting mucosa in more than 50% of cases, which can lead to ulceration, bleeding, or transmural erosion and perforation. We report an unusual case of a child who presented with small bowel obstruction caused by an ileal duplication cyst that had become infected with Salmonella. Although reports of infected mesenteric cysts and duodenal duplication cysts have been published, this is the first reported case of an infected ileal duplication cyst. We also present a review of the literature regarding these interesting lesions.

Reliability of the Lung-to-head Ratio As a Predictor of Outcome in Fetuses with Isolated Left Congenital Diaphragmatic Hernia at Gestation Outside 24-26 Weeks

American Journal of Obstetrics and Gynecology. Jul, 2007  |  Pubmed ID: 17618746

The purpose of this study was to investigate the relationship between lung-to-head ratio (LHR) and gestational age (GA) in fetuses with isolated left congenital diaphragmatic hernia and to determine the applicability and reliability of LHR to predict postnatal outcome beyond 24-26 weeks of gestation.

Surgical Treatment of Childhood Recurrent Pancreatitis

Journal of Pediatric Surgery. Jul, 2007  |  Pubmed ID: 17618881

Surgical intervention that improves pancreatic ductal drainage is a reasonable treatment strategy for recurrent pancreatitis in children.

Characteristics of Congenital Cystic Adenomatoid Malformations Associated with Nonimmune Hydrops and Outcome

Journal of Pediatric Surgery. Aug, 2007  |  Pubmed ID: 17706495

In fetuses with congenital cystic adenomatoid malformations of the lung (CCAMs), hydrops fetalis and large masses are associated with poor outcomes. This study attempts to (1) determine sonographic features (in addition to large size) that correlate with hydrops and (2) characterize the features that correlate with outcome among hydropic fetuses.

A Prospective, Randomized, Multicenter Trial of Amnioreduction Vs Selective Fetoscopic Laser Photocoagulation for the Treatment of Severe Twin-twin Transfusion Syndrome

American Journal of Obstetrics and Gynecology. Oct, 2007  |  Pubmed ID: 17904975

The objective of the study was to examine the effect of selective fetoscopic laser photocoagulation (SFLP) vs serial amnioreduction (AR) on perinatal mortality in severe twin-twin transfusion syndrome (TTTS).

Fetoscopic Transuterine Release of Posterior Urethral Valves: a New Technique

Fetal Diagnosis and Therapy. 2008  |  Pubmed ID: 18033963

Fetal urinary tract obstruction with oligohydramnios produces pulmonary hypoplasia and renal dysplasia. Decompression of the obstructed urinary tract may restore amniotic fluid and allow lung growth, but transabdominal catheter shunt decompression is often inadequate and does not allow for cycling of the bladder, while open procedures cause significant maternal morbidity. Disruption of the anatomic obstruction, usually posterior urethral valves in a male fetus, would be ideal but has proven technically difficult. Here we describe a new technique of percutaneous fetal cystoscopy and disruption of posterior urethral valves, and the case report of our first application of this technique. We pre-sent a case of a 17-week male fetus with posterior urethral valves which underwent fetal cystoscopy for mechanical disruption of posterior urethral valves. This minimally invasive approach to disruption of posterior urethral valves in a fetus is a novel method for decompressing the urinary tract. The technique offers a minimal degree of maternal morbidity and, if instituted early enough, can restore amniotic fluid volume, avert fatal pulmonary hypoplasia and may preserve renal function.

Thoracoscopic Versus Open Resection for Congenital Cystic Adenomatoid Malformations of the Lung

Journal of Pediatric Surgery. Jan, 2008  |  Pubmed ID: 18206452

This study evaluated the potential advantages of thoracoscopy compared to thoracotomy for resection of congenital cystic adenomatoid malformations (CCAM).

Does Congenital Diaphragmatic Hernia Associated with Bronchopulmonary Sequestration Portend a Better Prognosis?

Fetal Diagnosis and Therapy. 2008  |  Pubmed ID: 18417988

Congenital diaphragmatic hernia (CDH) continues to be a devastating disease in the newborn population, with well-documented morbidity and mortality. Bronchopulmonary sequestration is a separate congenital defect that has been associated with CDH. While the association of sequestration with CDH has been reported to be as high as 30-40%, the prognosis associated with the two simultaneous defects is unknown. We reviewed our experience to evaluate if prognosis was better in the CDH infants with associated bronchopulmonary sequestration.

Determination of Risk Factors for Deep Venous Thrombosis in Hospitalized Children

Journal of Pediatric Surgery. Jun, 2008  |  Pubmed ID: 18558189

Our objective was to determine the time trend and risk factors for deep venous thrombosis (DVT) of the lower extremities among pediatric inpatients.

Indication for Pediatric Muscle Biopsy Determines Usefulness

Journal of Pediatric Surgery. Dec, 2008  |  Pubmed ID: 19040934

Diagnostic skeletal muscle biopsy is an invasive procedure used for evaluation of neuromuscular disorders. We hypothesize that the yield of biopsy varies with its indication or suspected diagnosis.

Sacrococcygeal Teratoma with Spinal Canal Invasion Prenatally Diagnosed

Journal of Pediatric Surgery. Apr, 2009  |  Pubmed ID: 19361623

Fetal sacrococcygeal teratoma (SCT) is rarely associated with spinal invasion. The prognostic significance of spinal invasion is unknown. In the past, invasion has been discovered by postnatal imaging or in the operating room. As screening ultrasounds have become more prevalent in the United States, more SCTs have been discovered and characterized prenatally. This screening has allowed for better birth planning and neonatal therapy. We report a case of SCT invading the spinal canal seen on prenatal ultrasound. To our knowledge, this is the first documented case of prenatally diagnosed SCT intraspinal invasion.

Conflicts in Wound Classification of Neonatal Operations

Journal of Pediatric Surgery. Jun, 2009  |  Pubmed ID: 19524742

This study sought to determine the reliability of wound classification guidelines when applied to neonatal operations.

Fetal Surgery is a Multidisciplinary Field That Relies on the Participation and Expertise of a Variety of Clinicians and Support Staff. Preface

Clinics in Perinatology. Jun, 2009  |  Pubmed ID: 19559315

Tracheal Occlusion for Fetal Congenital Diaphragmatic Hernia: the US Experience

Clinics in Perinatology. Jun, 2009  |  Pubmed ID: 19559324

Congenital diaphragmatic hernia (CDH) is characterized by a defect in the diaphragm that permits abdominal viscera to herniate into the chest. These herniated viscera are thought to compress the growing lung and cause lung parenchymal and vascular hypoplasia. The genetic defects that cause the diaphragmatic defect may also contribute primarily to lung hypoplasia. Postnatal reduction of the herniated abdominal viscera and correction of the diaphragmatic defect are easily achievable, but the lung hypoplasia persists, often leading to persistent fetal circulation and respiratory failure. This article reviews the experimental basis of fetal therapy for CDH and the US clinical experience with tracheal occlusion.

Complicated Monochorionic Twin Pregnancies: Updates in Fetal Diagnosis and Treatment

Clinics in Perinatology. Jun, 2009  |  Pubmed ID: 19559328

Monochorionic (MC) twin pregnancies may develop significant complications, and twin-to-twin transfusion syndrome (TTTS) has become among the best known to obstetricians and patients alike. A significant percentage of patients referred for suspected TTTS have a different underlying pathologic condition, however, and differentiating the subcategories of MC pathophysiologic conditions may change treatment course and outcome. The key to understanding complicated MC pregnancies lies in the placental angioarchitecture and intertwin vascular communications between the fetuses.

Prognostic Role of Tumor-head Volume Ratio in Fetal Sacrococcygeal Teratoma

Fetal Diagnosis and Therapy. 2009  |  Pubmed ID: 19752518

To evaluate the ability of a tumor-head volume ratio to predict outcome and incidence of hydrops in fetuses with sacrococcygeal teratoma.

Long-term Survival and Renal Transplantation in a Monozygotic Twin with Cloacal Dysgenesis Sequence

Journal of Pediatric Surgery. Dec, 2009  |  Pubmed ID: 20006002

Cloacal dysgenesis sequence (CDS) is a severe hindgut malformation occurring in 1:50,000 to 250,000 live births (Qureshi et al. Prenatal diagnosis of cloacal dysgenesis sequence: differential diagnosis from other forms of fetal obstructive uropathy. Fetal Diagn Ther 1998;13:69-74; Bargaje et al. Cloacal dysgenesis sequence. Ann Diagn Pathol 2008;12:62-66). It is characterized by a smooth perineum with no urethral, vaginal, or anal openings, and lack of labioscrotal development. Typically, the bladder, vagina, and colon each end blindly, although persistent cloaca without perineal orifice can be seen. With no egress for urine, infants have renal insufficiency and pulmonary hypoplasia, usually making CDS lethal (Sahinoglu Z et al. The prenatal diagnosis of cloacal dysgenesis in six cases: can the termination of pregnancy always be the first choice? Prenat Diagn 2004;24:10-16). Reported survivors have had a persistent urachus or have been spared the effects of oligohydramnios by the presence of a twin (Liang X. Cloacal dysgenesis sequence: observations in four patients, including three fetuses of second trimester gestation. Pediatr Dev Pathol 1998;1:281-288). We report a case of long-term survival, currently to 25 months of age, and renal transplantation in a monochorionic, diamniotic twin girl with CDS.

Colonic Atresia and Choledochal Cyst: a Rare Combination

Pediatric Surgery International. Jan, 2009  |  Pubmed ID: 18985360

Colonic atresia and choledochal cyst are rare congenital abnormalities and there are few cases of their coexistence reported in the literature. We report a case of choledochal cyst and colonic atresia in a term neonate. Our prenatal ultrasonographic and intraoperative findings support the idea that these two entities may be related.

Prenatal Steroids for Microcystic Congenital Cystic Adenomatoid Malformations

Journal of Pediatric Surgery. Jan, 2010  |  Pubmed ID: 20105595

The purpose of this study is to evaluate the effect of prenatal steroid treatment in fetuses with sonographically diagnosed congenital cystic adenomatoid malformations (CCAMs).

Long-term Surgical Outcomes in Congenital Diaphragmatic Hernia: Observations from a Single Institution

Journal of Pediatric Surgery. Jan, 2010  |  Pubmed ID: 20105597

Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors.

The Effect of Fiber Alignment and Heparin Coating on Cell Infiltration into Nanofibrous PLLA Scaffolds

Biomaterials. May, 2010  |  Pubmed ID: 20122725

Biodegradable nanofibers simulate the fibril structure of natural extracellular matrix, and provide a cell-friendly microenvironment for tissue regeneration. However, the effects of nanofiber organization and immobilized biochemical factors on cell infiltration into three-dimensional scaffolds are not well understood. For example, cell infiltration into an electrospun nanofibrous matrix is often limited due to relatively small pore size between the fibers. Here we showed that biophysical and biochemical modification of nanofibrous scaffolds facilitated endothelial cell infiltration in three-dimensional scaffolds in vitro and in vivo. Aligned nanofibers significantly enhanced cell infiltration into the nanofibrous matrices in vitro. In a full-thickness dermal wound model, the nanofiber scaffolds enhanced epidermal skin cell migration across the wound when compared to a control group without scaffold. Aligned nanofibers promoted the infiltration of endothelial cells into the scaffolds. Furthermore, heparin-coated nanofibers also increased cell infiltration significantly. These results shed light on the importance of biophysical and biochemical properties of nanofibers in the regulation of cell infiltration into three-dimensional scaffolds and tissue remodeling.

Perinatal Outcome of Conservative Management Versus Fetal Intervention for Twin Reversed Arterial Perfusion Sequence with a Small Acardiac Twin

Fetal Diagnosis and Therapy. 2010  |  Pubmed ID: 20215730

To examine the outcomes of patients with twin reversed arterial perfusion (TRAP) sequence in which the acardiac twin was

The Anatomic Pattern of Biliary Atresia Identified at Time of Kasai Hepatoportoenterostomy and Early Postoperative Clearance of Jaundice Are Significant Predictors of Transplant-free Survival

Annals of Surgery. Oct, 2011  |  Pubmed ID: 21869674

The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes.

Diastolic Cardiac Pathology and Clinical Twin-Twin Transfusion Syndrome in Monochorionic/Diamniotic Twins

American Journal of Obstetrics and Gynecology. Sep, 2011  |  Pubmed ID: 21909155

OBJECTIVE: to identify differences in echocardiographic profiles of monochorionic/diamniotic pregnancies with early or mild twin-twin transfusion syndrome (TTTS), compared to monochorionic/diamniotic twins affected only by discordant growth or discordant fluid. STUDY DESIGN: retrospective evaluation of sonograms and echocardiograms of twin pregnancies referred for suspected TTTS. RESULTS: 112 monochorionic/diamniotic pairs were studied. 41 did not have/develop TTTS, 61 had Stage I/II TTTS. Ten developed TTTS after initially not meeting criteria. TTTS recipients had a higher rate of venous Doppler or tricuspid inflow abnormalities than purported "recipients" in non-TTTS pregnancies (86% vs. 37%, P<0.001). TTTS recipients had shorter tricuspid inflow duration/RR intervals than non-TTTS fetuses (32+/-6% versus 37+/-4%, P<0.001). Logistic regression and recursive partitioning identified shorter tricuspid inflow duration, longer isovolumic relaxation, and ductus venosus abnormality associated with TTTS. CONCLUSION: Diastolic pathology, specifically shorter tricuspid inflow duration, may be considered a hallmark of TTTS distinguishing these pregnancies from other monochorionic/diamniotic twin complications.

Echocardiographic Risk Stratification of Fetuses with Sacrococcygeal Teratoma and Twin-reversed Arterial Perfusion

Fetal Diagnosis and Therapy. 2011  |  Pubmed ID: 22086180

To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP).

Microcystic Congenital Pulmonary Airway Malformation with Hydrops Fetalis: Steroids Vs Open Fetal Resection

Journal of Pediatric Surgery. Jan, 2012  |  Pubmed ID: 22244389

Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis.