Biomedical subjects

Roman M Sydorak

Publications and source records attributed to Roman M Sydorak.

18 recordsLinked to original sources

PubMed2005

Laparoscopic partial nephrectomy in infants and toddlers.

BACKGROUND/PURPOSE: The aim of this report is to assess the technique and outcome of laparoscopic partial nephrectomy in infants and toddlers. METHODS: From January 2001 to January 2005, 7 consecutive patients, ages 5 to 15 months, underwent laparoscopic partial nephrectomies. All patients had duplicated systems associated with ureteroceles (5), severe reflux (1), ectopic ureter (1), and nonfunctioning systems. Follow-up ranged from 4 to 51 months. RESULTS: All procedures were completed successfully using 4 ports (2 x 5 and 2 x 3 mm) except one, which required an additional port. The distal ureter, renal parenchyma, and hilar vessels were all transected using the harmonic scalpel. The mean operating time was 179 minutes with minimal blood loss in each case. The average hospital stay was 2.4 days (range, 1-5 days). The first case in the series, initially attempted retroperitoneally, was converted to a transabdominal approach because of lack of space. All subsequent approaches were transabdominal. One patient required ureteral stump reexcision because of frequent urinary tract infections associated with a distal ureteral diverticulum. CONCLUSIONS: Laparoscopic partial nephrectomy can be performed safely. The harmonic scalpel divides the parenchyma bloodlessly. The cosmetic result is excellent. A transabdominal approach with division of the ureteral cuff flush with the bladder is recommended.

Adolescent↗

PubMed2004

Spontaneous resolution of prenatal megalourethra.

Urethral obstruction in the fetus is rare. Whereas proximal obstruction most often is caused by posterior urethral valves, causes of distal obstruction are less well recognized and can include urethral atresia, urethral webs, and anterior urethral valves. These latter abnormalities can lead to urinary retention, incontinence, enuresis, spontaneous bladder rupture, and megacystis. The authors present 3 fetuses (gestational age range, 18 to 20 weeks) in whom distal urethral obstruction was suspected by prenatal ultrasonography in the absence of a demonstrable lesion. All 3 experienced spontaneous resolution of the presumed obstruction. On follow-up, all are alive and well with no adverse genitourinary tract sequelae. No postpartum intervention was required.

Adult↗

PubMed2003

Fetal surgery for repair of myelomeningocele allows normal development of anal sphincter muscles in sheep.

One major problem for patients with myelomeningocele (MMC) is fecal incontinence. To prevent this problem, fetal surgery for repair of MMC has been recently undertaken. The strategy behind this surgery is to allow normal development of anal sphincter muscles. The purpose of this study was to determine whether fetal surgery for repair of MMC allows normal development of anal sphincter muscles. Myelomeningocele was surgically created in fetal sheep at 75 days of gestation. At 100 days of gestation, fetal surgery for repair of the MMC lesion was performed. Three repair methods were used: standard neurosurgical repair (4 fetal sheep), covering the MMC lesion with Alloderm (2 fetal sheep), and covering the MMC lesion with Gore-Tex (2 fetal sheep). After the sheep were delivered (140 days of gestation), external and internal anal sphincter muscles were analyzed histopathologically. In control fetal sheep (not repaired) anal sphincter muscles did not develop normally. In contrast, in fetal sheep that underwent repair of the MMC, the external and internal anal sphincter muscles developed normally. Histopathologically, in the external sphincter muscles, muscle fibers were dense. In the internal sphincter muscles, endomysial spaces were small, myofibrils were numerous, and fascicular units were larger than those in unrepaired fetal sheep. There was no difference in muscle development for the repair methods. Fetal surgery for repair of MMC allows normal development of anal sphincter muscles.

Anal Canal↗

PubMed2003

Minimal access fetal surgery.

The development of fetal surgery has led to promising therapeutic options for a number of congenital malformations. However, preterm labor (PTL) and premature rupture of membranes continue to be ubiquitous risks for both mother and fetus. To reduce maternal morbidity and the risk of prematurity, minimal access surgical techniques were developed and are increasingly employed. Congenital diaphragmatic hernia (CDH), obstructive uropathy, twin-to-twin transfusion syndrome (TTTS), and sacrococcygeal teratoma have already been successfully treated using minimal access fetal surgical procedures. Other life-threatening diseases as well as severely disabling but not life-threatening conditions are potentially amenable to treatment. The wider application of minimal access fetal surgery depends on a continued improvement in technology and a better understanding of complications associated with fetal intervention.

Congenital Abnormalities↗

PubMed2003

Fetal surgery for repair of myelomeningocele allows normal development of the rectum in sheep.

To determine whether fetal surgery in a fetus with myelomeningocele (MMC) allows normal development of rectal muscles and nerves, we analyzed the rectum after fetal surgery in a sheep model. An MMC lesion was surgically created in 13 fetal sheep at 75 days of gestation. One fetal sheep died after the lesion was created. Eight fetal sheep were repaired at 100 days of gestation; the others were not repaired, as a control (n=4). Three methods were used for fetal surgery of MMC: standard neurosurgical repair (4 fetal sheep), covering of the MMC lesion by Alloderm (2 fetal sheep), and covering of the MMC lesion by Gore-Tex (2 fetal sheep). At 140 days of gestation, fetal sheep were harvested and histo-pathological analysis was performed on the rectum using hematoxylin and eosin staining for muscles and S-100 protein staining for nerves. One fetal sheep treated by standard neurosurgical repair died before harvesting. The four untreated fetal sheep had hypoplastic longitudinal muscles of the rectum but well developed-circular muscles. In addition, the untreated fetuses had a hypoplastic submucosal nerve plexus but a well-developed intermuscular nerve plexus. In contrast, treated fetal sheep had well-developed longitudinal and circular muscles except for one sheep treated with standard neurosurgical repair. In addition, except for the same fetal sheep, treated fetal sheep had well-developed nerve plexuses. There was no difference in muscle and nerve development of the rectum among the three repair methods. Fetal surgery for repair of MMC allows normal development of rectal muscles and nerves.

Animals↗

PubMed2003

Pathophysiologic patterns influencing fetal surgery.

There are a growing number and variety of fetal disorders that may benefit from intervention prior to birth. Despite the diversity, there are common pathophysiologic denominators or patterns that tie together many seemingly disparate disorders. The purpose of this article was not to review disorders presented in other, accompanying articles but, rather, to present pathophysiologic patterns that common influence fetal surgery within a cohesive framework.

Airway Obstruction↗

PubMed2003

Congenital diaphragmatic hernia: advances in prenatal therapy.

Congenital diaphragmatic hernia (CDH) is one of the most common causes of neonatal morbidity and mortality. The clinical spectrum of CDH ranges from minimally affected infants who do well with modern neonatal care to severely affected infants who die despite all interventions. Two decades of research have led to advances in the prenatal diagnosis of CDH and have better defined the natural history of CDH. Fetuses with CDH now can be stratified into "low" and "high" risk groups based on sonographic parameters. "Low risk" fetuses have an excellent chance of survival with postnatal therapy. Prenatal intervention is reserved for "high risk" fetuses. Ongoing research is focused on improving both prenatal and postnatal treatment of these severely affected infants.

Extracorporeal Membrane Oxygenation↗

PubMed2003

Minimal access techniques for fetal surgery.

The impetus for the development of minimal access techniques for fetal surgery was the unusual occurrence with open hysterotomy of preterm labor, premature rupture of membranes, and the maternal complications resulting from tocolytic therapy. This strategy involves a constellation of techniques that allow surgical procedures to be performed inside the uterus without a hysterotomy. The unique requirements of this approach necessitated modifications of existing endoscopic techniques, development of novel fetoscopic instruments, and utilization of the expertise of a wide variety of specialists. Technical expertise in the field and a natural evolution of techniques have given rise to innovative repairs previously not envisioned. Severe congenital diaphragmatic hernia, diseases of monochorionic twins, and obstructive uropathy have already been successfully treated using fetoscopic surgical techniques. Fetoscopic correction of many other non-life-threatening anomalies continues to evolve. The future of fetoscopic surgical intervention depends on the continual evolution of novel techniques, the elucidation of the pathophysiology and treatment of other fetal disorders, and a better understanding of treatment of complications of intervention.

Amniotic Band Syndrome↗

PubMed2003

Fetoscopic temporary tracheal occlusion for congenital diaphragmatic hernia: prelude to a randomized, controlled trial.

OBJECTIVE: As previously reported, high postnatal mortality seen in fetuses with congenital diaphragmatic hernia (CDH) with liver herniation and low lung-to-head ratio (LHR) appears to be improved in fetuses who undergo fetoscopic temporary tracheal occlusion (TO). To test whether further evolution of this technique produces results that justify a randomized controlled trial comparing prenatal intervention to postnatal care, the authors analyzed 11 additional cases and the cumulative experience with 19 cases. METHODS: The authors analyzed retrospectively the outcome of 11 new and 8 previously reported cases of fetoscopic temporary tracheal occlusion. Various factors were studied including maternal morbidity, antenatal outcome, physiologic lung response, and neonatal course. RESULTS: Temporary TO can be accomplished using 3 5-mm radially expanding uterine ports without hysterotomy. Obstetric morbidity included mild pulmonary edema in 6 cases, chorioamniotic separation and premature rupture of membranes in 12 patients, and preterm labor and delivery in all patients. Thirteen of 19 (68%) neonates survived for 90 days after delivery; one died in utero, and 5 died after birth. Late mortality included one death caused by sepsis and 2 by complications associated with tracheostomies. Morbidity from gastroesophageal reflux requiring Nissen fundoplication, tracheal injury requiring repair or tracheostomy, and recurrent hernias after diaphragmatic repair were characteristic in longterm survivors. CONCLUSIONS: Fetoscopic temporary TO may improve outcome in poor-prognosis fetuses with CDH. However, complications related to tracheal dissection, premature delivery and late morbidity are significant. This experience has led to simpler techniques for fetoscopic tracheal occlusion and to an National Institutes of Health-sponsored randomized controlled trial comparing fetoscopic tracheal occlusion with optimal postnatal care.

Fetal Diseases↗

PubMed2003

Congenital diaphragmatic hernia: advances in prenatal therapy.

Despite intensive clinical and experimental efforts, mortality from CDH remains high. More than two decades of research in multiple centers has led to a better understanding of the pathophysiology, prognosis, and treatment options for fetuses that have CDH. It now appears that fetuses that have prenatally diagnosed CDH can be stratified into high- and low-risk groups based upon sonographic parameters. Fetuses that do not have liver herniation into the chest that have a favorable LHR have an excellent chance of survival with postnatal therapy. Prenatal diagnosis allows the time and place of delivery to be planned in advance so these infants can be treated in a tertiary care nursery that has maximal medical and surgical therapy. Fetuses that have liver herniation into the chest and an unfavorable LHR have a grim prognosis. These fetuses might benefit from in utero intervention. There is no role for open fetal repair of the diaphragmatic detect; however, fetoscopic temporary tracheal occlusion might improve lung growth and development and might decrease morbidity and mortality in these infants. The FETENDO strategy appears to work, and for the first time it offers hope to the fetus that has high-risk CDH, but its efficacy must be proven in a proper randomized, controlled trial.

Animals↗

PubMed2003

Spectrum of intrapartum management strategies for giant fetal cervical teratoma.

BACKGROUND/PURPOSE: 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. METHODS: A retrospective review of all patients referred since 1994 for prenatal management of a fetal neck mass was performed. Variables examined included gestational age at diagnosis and delivery, size and location of the neck mass, presence of fetal hydrops, associated anomalies, management methods, operating time, and outcome. RESULTS: Seven patients were identified with a prenatal diagnosis of giant cervical teratoma. Four patients had fetal hydrops; of these, 2 died in utero of hydrops, and a third fetus underwent elective termination. The remaining hydropic and previable fetus underwent fetal surgery for resection of the mass. The 3 nonhydropic patients underwent ex utero intrapartum treatment (EXIT) procedures for airway control. Endotracheal intubation was possible in one patient, and one received a tracheostomy. In the third fetus, neither intubation nor tracheostomy were possible, and resection of the neck mass was performed on placental support. There were no deaths in the surgical group. CONCLUSIONS: The management of fetal giant cervical teratoma includes a spectrum of options. For the rare previable fetus with hydrops, fetal resection may be indicated. In patients with airway obstruction, EXIT procedure provides the luxury of time to obtain airway control either by intubation, tracheostomy, or, if necessary, tumor resection on placental support.

Abortion, Eugenic↗

PubMed2003

Thoracoscopic lobectomy for prenatally diagnosed lung lesions.

PURPOSE: The aim of this report is to assess the technique and outcome of thoracoscopic lobectomy for asymptomatic prenatally diagnosed lung lesions. METHODS: From June 1999 to March 2002, 14 consecutive asymptomatic patients with a prenatal diagnosis of congenital cystic adenomatoid malformation or pulmonary sequestration, ages 3 to 15 months, underwent postnatal thoracoscopic lobectomy. Single-lung ventilation and controlled pneumothorax with low pressure (4 torr) and low flow (1.0 L/min) were used in all. Follow-up ranged from 4 to 35 months. RESULTS: All procedures were completed successfully using 3 ports. Rather than using stapling devices or clips, pulmonary vessels were sealed and the fissure completed (when necessary) with the Ligasure thermal energy device. Eleven lesions were on the left (10 lower lobe), and 3 were in the right lower lobe. The mean operating time was 110 minutes. The average hospital stay was 38 hours. There were no intraoperative or postoperative complications. CONCLUSIONS: This is the first report of a completely thoracoscopic technique for pulmonary lobectomy in small children. Thoracoscopic lobectomy is a relatively quick and safe procedure, and the cosmetic result is excellent. Early resection obviates the risk of infection in these lesions.

Bronchopulmonary Sequestration↗

PubMed2002

Fetal endoscopic surgery: lessons learned and trends reviewed.

PURPOSE: 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. METHODS: All fetal endoscopic cases performed at a single institution from January 1996 to August 2001 were reviewed (n = 66). Cases were examined with respect to year performed, type of operation, operative data, and outcome. RESULTS: Twin-twin transfusion syndrome (26 cases) and congenital diaphragmatic hernia (35 cases) were the most common diseases treated. From 1996 to 2001, there was a decrease in average operating time (256 to 127 minutes [P =.0006]), number of ports utilized (3.8 to one [P =.00001]), pump volume (28.7 to 2.7 L [P =.00001]), and estimated blood loss (408 to 29 mL [P =.008]). In addition, port size changed from 10 mm to 5 mm. Chorioamniotic separation (31 of 66), premature rupture of membranes (32 of 66), chorioamnionitis (12 of 66), and fetal death (10 of 66) continued to be significant complications. CONCLUSIONS: Fetal endoscopic surgery over the last 6 years has evolved toward shorter operating time, the use of smaller and fewer ports, decreased pump fluid exchange, and decreased blood loss, with the types of cases centered on twin-twin transfusion syndrome and congenital diaphragmatic hernia.

Endoscopy↗

PubMed2002

Laparoscopic repair of high imperforate anus.

Numerous laparoscopic operations have replaced the traditional open procedure in both adults and children. These new procedures have allowed access to body cavities without significantly traumatizing intervening tissue. The laparoscopically assisted anorectal pull-through (LAARP) for high anorectal malformations (ARM) uses fundamental concepts learned from decades of high ARM repair and incorporates modern technologic advancements in surgical instrumentation and techniques. This laparoscopic approach offers good visualization of an infant's deep pelvis with a reconstruction technique that minimizes trauma to important surrounding structures. The laparoscopic repair can be completed in one stage, 2 stages, or 3 stages. Currently, either the 2-stage or 3-stage operation is recommended. With the 3-stage approach, a temporary colostomy is created initially followed by LAARP in several weeks to months. The colostomy then is closed several months later.

Anus, Imperforate↗

PubMed2002

Laparoscopic antireflux procedures in children: evaluating the evidence.

A growing number of the pediatric antireflux procedures are performed laparoscopically. Although there are no prospective randomized studies comparing conventional open surgery to laparoscopic surgery, there are retrospective and anecdotal data suggesting that the laparoscopic approach is at least as good and, in many cases, better than the open procedure. Once the significant learning curve is achieved, one may attain similar operative times with the benefit of magnification and enhanced visualization of the operative field compared to open surgery. The greatest benefits of laparoscopic antireflux surgery are the cosmetic result, a decrease in postoperative analgesia requirements, and an earlier return to normal daily life for both parents and their children.

Child↗

PubMedDate not supplied

Lung pathology in patients with congenital diaphragmatic hernia treated with fetal surgical intervention, including tracheal occlusion.

Fetal intervention for congenital diaphragmatic hernia was developed to lessen the high morbidity and mortality of pulmonary hypoplasia. Lung pathology and morphometry in patients treated with fetal intervention have not been described. We report clinical and autopsy findings, as well as basic lung morphometry in 16 cases of congenital diaphragmatic hernia with fetal intervention (12 cases tracheal occlusion; 4 cases hernia repair), and 19 cases of congenital diaphragmatic hernia without fetal intervention. All patients who underwent fetal intervention were born premature. Lung enlargement with increased lung-to-body weight ratio was observed with fetal tracheal occlusion, accompanied by lower than normal radial alveolar counts and increased alveolar size. Patients treated with tracheal occlusion also had early alveolar development (at 29.8, 30.6, and 30.9 wk postconceptual age) as well as mucous fluid pooling in airways and alveoli. All cases showed severe alveolar septal widening, more extensive in patients without fetal intervention. When grouped by postconceptual age, no statistically significant difference was found between patients with and without fetal intervention with respect to lung-to-body weight ratio, radial alveolar count, mean alveolar length, and relative arteriolar media thickness. Lung enlargement has been observed with fetal tracheal occlusion sonographically; our studies suggest that this is due in part to emphysema and mucous fluid pooling. The lung remains abnormal with low radial alveolar counts and increased alveolar size. Tracheal occlusion did not prevent development of lung pathology associated with pulmonary hypoplasia.

Female↗

PubMedDate not supplied

Chorioamniotic membrane separation following fetal surgery.

OBJECTIVE: As the volume of fetal surgery cases has steadily increased, an increasing incidence of chorioamniotic membrane separation (CMS) has been noted. Due to the potential adverse consequences from this abnormality, we reviewed the last decade of experience with fetal intervention at our institution and examined the incidence and outcomes of fetuses given this diagnosis. STUDY DESIGN: A retrospective chart review of 75 fetal surgery cases at our institution was performed. Variables analyzed included preoperative, operative, and outcome data. Postoperative ultrasounds were evaluated for the presence of CMS. RESULTS: Excluding operative deaths, the incidence of CMS was 47%. There were significant differences (p<0.05) in time to delivery (7 vs 5 weeks), cases using a perfusion pump (80% vs 60%), and number of trocars (2.13 vs 1.54) in cases of CMS versus those without. Ultrasounds showed normal to high levels of amniotic fluid in 97% of cases. There was an increased incidence of premature rupture of membranes (63% vs 45%), preterm labor (57% vs 38%), and chorioamnionitis (29% vs 15%) with CMS, but no difference in mortality rate. CONCLUSION: CMS is a frequent finding following fetal surgery. It is associated with significant morbidity but is manageable with close follow-up in a hospital setting. Following fetal surgery, the finding of CMS can be a life-threatening complication that warrants further study to understand its etiology and prevention.

Amnion↗

PubMedDate not supplied

Prenatal resection of a fetal pericardial teratoma.

OBJECTIVE: Pericardial teratomas are rare congenital tumors which invade the developing mediastinum, compressing the venous return to the heart, leading to hydrops. Tumors, with large cystic components, have been treated previously with in utero pericardiocentesis with some success. We present the first reported case of in utero open resection of a fetal pericardial teratoma. METHODS: A 31-year-old G1P0 woman was found to have a fetus with a pericardial teratoma. Hydrops developed at 24 weeks' gestation. After counseling, open fetal resection was performed via a fetal median sternotomy. RESULTS: Although the tumor was successfully removed, the hydrops did not resolve. In addition, over the course of 3 weeks, the mother developed maternal mirror syndrome which prompted an emergent cesarean section. Neonatal death ensued shortly after birth. CONCLUSIONS: The fetus with a pericardial teratoma complicated by hydrops is compromised. Treatment options include early delivery, aspiration of the pericardial effusion, and in utero operative resection.

Adult↗