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Biomedical subjects

T M Crombleholme

Publications and source records attributed to T M Crombleholme.

At least 73 records · Page 4Linked to original sources
PubMed1994

Intestinal necrosis from congenital hypercoagulopathy.

Protein-S deficiency is a rare congenital disorder that results in a hypercoagulable state, typically presenting as recurrent venous thrombosis in adults. Few cases have been reported in children. A 2-year-old girl presented in septic shock. During laparotomy, ischemic necrosis of the intestine was noted, which extended from the gastroesophageal junction to the midjejunum. Total gastrectomy, pancreaticoduodenectomy , and partial resection of the small intestine were performed. She was left with 140 cm of distal small bowel, and had reconstruction with a single limb for the pancreaticojejunostomy, choledochojejunostomy, and esophagojejunostomy to preserve intestinal length. Postoperative evaluation eventually disclosed a previously unrecognized protein-S deficiency. The hypercoagulopathy of protein-S deficiency can be complicated by catastrophic thrombotic events that require surgery. This congenital disorder should be considered in any child who has an unexplained thrombotic episode, because failure to recognize protein-S deficiency may have potentially fatal consequences.

Child, Preschool↗
PubMed1994

Diagnosis and management of fetal neuroblastoma.

At 36 weeks' gestation, in a 30-year-old patient, routine prenatal ultrasound examination revealed a cystic mass in the left adrenal gland. The pregnancy was uneventful, and at delivery the infant had a palpable left flank mass confirmed by ultrasound and computed tomography scan. There were no metastases. On the second day of life, a 5 cm cystic stage-I neuroblastoma was removed by adrenalectomy. The infant had an uneventful recovery, required no further treatment, and remains free of disease 26 months later.

Adrenal Gland Neoplasms↗
PubMed1994

Bronchopulmonary sequestration: prenatal diagnosis with clinicopathologic correlation.

A patient was referred at 21 weeks' gestation for evaluation of an echogenic mass in the fetal right chest. Color-doppler sonography facilitated the diagnosis of a bronchopulmonary sequestration. Clinicopathologic correlation was possible after termination at 2.3 weeks' gestation. Pathologic examination confirmed the prenatal diagnosis and demonstrated the systemic vascular supply seen on color-doppler sonography. Color doppler is a useful adjunct in the sonographic evaluation of the fetus with an echogenic chest mass.

Adult↗
PubMed1994

Prenatal diagnosis and management of gastrointestinal anomalies.

The increased use of prenatal sonography has led to earlier and more frequent diagnosis of a wide range of gastrointestinal anomalies. Many of these anomalies are associated with other severe cardiac, renal, and genetic abnormalities that may impact on decisions regarding timing and site of delivery. The majority of these patients should be referred to a center that provides perinatal, neonatal, and pediatric surgical expertise. After a complete prenatal evaluation, a decision regarding the site of delivery and the need for subspecialty referral can be made. Prenatal diagnosis of the conditions discussed in this article does not influence the mode of delivery, but subsequent management of the newborn is improved by delivery in a tertiary care center.

Digestive System Abnormalities↗
PubMed1994

Prenatal diagnosis and management of the fetus with an abdominal wall defect.

Prenatal ultrasound has advanced our understanding of congenital abdominal wall defects. In addition to providing insights into the divergent embryological origins and natural history of abdominal wall defects, ultrasound has had an important impact on the management of these anomalies. For fetuses with gastroschisis, the changes in appearance of the bowel may suggest expeditious delivery. In cases of omphalocele, the presence of additional anomalies is significantly associated with the ultimate prognosis for these fetuses. Giant omphalocele may preclude vaginal delivery secondary to dystocia. Exstrophies of the cloaca and bladder are rare congenital abnormalities that often present complex management issues, including gender reassignment in cases of cloacal exstrophy, for those couples wishing to continue the pregnancy. We believe that the optimal management of a fetus diagnosed with an abdominal wall defect requires a coordinated effort among specialists from maternal fetal medicine, pediatric surgery, and pediatrics.

Abdominal Muscles↗
PubMed1994

Prenatal diagnosis and management of fetal thoracic lesions.

There have been significant strides made during the last decade in understanding the natural history and pathophysiology of fetal thoracic lesions. Largely as a result of advances in prenatal ultrasound, we are not only able to diagnose these lesions and advise parents about prognosis, but also offer the possibility of fetal intervention for the most severely affected fetuses. However, large gaps remain in the current state of knowledge of fetal thoracic lesions. We are unable to accurately predict pulmonary hypoplasia, the most devastating consequence of fetal thoracic lesions. In lesions, such as CDH, the selection criteria for fetal intervention remain ill defined. Proof of the efficacy and superiority of fetal surgery over conventional postnatal therapies for diaphragmatic hernia await the results of prospective trials. Fetal surgery in lesions such as CCAM and BPS is currently reserved for only those fetuses with hydrops and a uniformly fatal outcome. Whether fetal surgery in these cases would be beneficial in the absence of hydrops is difficult to say especially given the possibility of spontaneous regression. During the next decade we anticipate continued growth in our understanding of these lesions, refinement in selection criteria for intervention, and advances in techniques for salvaging these severely compromised fetuses. The diagnosis and treatment of fetal thoracic lesions remains a formidable challenge, but one which can be met with cautious optimism, due to the availability of fetal interventions not previously available.

Bronchopulmonary Sequestration↗
PubMed1993

The desmoplastic round cell tumor: a new solid tumor of childhood.

Three patients with a new, pathologically distinct solid tumor of childhood have been treated recently. The disease is characterized by male predominance, adolescent onset, an extensive abdominal primary tumor, and aggressive metastases to regional lymph nodes, liver, and lung. Two patients presented with vague abdominal pain and the third with testicular pain. All three noted fatigue and malaise of less than two months' duration with minimal associated weight loss. Computed tomography (CT) scans of the abdomen and chest were obtained for initial preoperative staging, and then all three underwent surgical exploration. Widespread disease was found in each case. In no instance was complete tumor extirpation possible because of extensive peritoneal spread and lymphatic and hepatic metastases. Histologically, all three tumors consisted of round blue cells with a dense desmoplastic reaction and focal rhabdoid features. Immunohistochemical markers for epithelial, neural, and muscle elements were positive. Aggressive multidrug chemotherapeutic regimens were used in each case, and all three patients are alive and well but with known residual disease. We conclude that in cases of the desmoplastic round cell tumor of childhood, CT scans underestimate the extent of disease, and exploratory laparotomy is necessary for diagnosis and appropriate staging. Surgery is usually palliative because of extensive spread. Awareness of this newly recognized aggressive solid tumor of childhood is essential to define its natural history and guide the development of effective multidisciplinary therapeutic regimens.

Adolescent↗
PubMed1993

Simplified "push" technique for percutaneous endoscopic gastrostomy in children.

Percutaneous endoscopic gastrostomy (PEG) by the "pull" technique is the standard method in pediatric patients. Modifications have been reported for adults but few in children. Problems with the "pull" technique including pericatheter infection due to contamination of the tube tract with oral flora, repeated insertion of the endoscope, potential esophageal injury from the catheter, and the possible need for another endoscopy for catheter removal, prompted our interest in a simpler technique. The "push" technique requires insertion of the endoscope only once to insufflate and visualize the insertion site. A modified Seldinger technique is used to insert a 14F acrylic Foley catheter. We have used this technique to place PEG tubes in 8 children age 6 weeks to 17 years (mean, 6 years), for failure to thrive due to cystic fibrosis (3), neurological impairment (4), and undetermined cause (1). Operative time averaged 15 minutes. All PEGs were used within 24 hours. This "push" technique of PEG insertion is safe, simple, quick, and obviates many of the potential risks inherent in the "pull" technique. The "push" technique deserves a more widespread application in children.

Adolescent↗
PubMed1992

Effect of erythropoietic stress on donor hematopoietic cell expression in chimeric rhesus monkeys transplanted in utero.

We have previously reported the successful development of hematopoietic chimerism after the in utero transplantation of fetal hematopoietic stem cells (HSC) in rhesus monkeys (Macaca mulatta). These animals exhibit sustained engraftment without immunosuppression or graft-versus-host disease (GVHD). To assess the functional response of the donor-derived erythropoietic population, we assayed the relative expression of donor and recipient hematopoietic progenitors in chimeric monkeys before and after anemic stress. Anemia in our chimeric animals resulted in increased erythropoietin (EPO) production comparable to controls. This was accompanied by changes in erythroid progenitor profiles, again similar to controls. Chimeric animals demonstrated normal reticulocytosis and reconstituted their hematocrit after hemorrhage at the same rate as controls. The donor-derived erythropoietic population exhibited normal responses to recipient regulatory signals and did not seem to expand at the expense of other hematopoietic lineages. Thus the proportions of engraftment for the myeloid and erythroid precursors in bone marrow and for blood lymphocytes remained stable. Our results demonstrate that the in utero transplantation of fetal HSC results in stable engraftment of donor erythropoietic progenitors, which appear to be fully integrated within the recipient's regulatory system. The abnormalities reported in the postnatal transplantation setting can then be attributed to immunologic reactions requiring conditioning myeloablative regimens. Fetal transplantation bypasses all these factors.

Anemia↗
PubMed1991

Transplantation of fetal cells.

Cellular transplantation is an attractive alternative to whole-organ transplantation when only a discrete function of the organ is deficient. Early fetal donor cells have an advantage because they engraft readily and do not cause graft-versus-host disease. Similarly, the fetus is an ideal recipient of allogeneic fetal cells as it is incapable of rejecting them early in gestation. This review presents the theoretical rationale, recent research advances, and clinical implications for adults with diabetes mellitus and Parkinson's disease; we also describe in utero transplantation of fetal hematopoietic stem cells and hepatocytes for the treatment of inherited hematologic and hepatic deficiencies, as well as the use of fetal islet cells and dopamine-producing cells to treat postnatal conditions.

Animals↗
PubMed1990

Carotid artery reconstruction following extracorporeal membrane oxygenation.

Right hemispheric brain injury has been noted in surviving infants treated with venoarterial extracorporeal membrane oxygenation (ECMO). This phenomenon may be secondary to permanent ligation of the right carotid artery. At our institution, conventional ventilatory therapy failed in five neonates with respiratory insufficiency, and they were treated successfully with ECMO. In four of the five neonates, the right carotid artery was reconstructed at the time of decannulation. At discharge, all newborns with carotid artery repair showed no signs of unilateral brain injury and had excellent antegrade flow in the right carotid artery as assessed by both duplex and transcranial Doppler ultrasound scanning. Carotid artery reconstruction after ECMO is a technically simple procedure that may reduce the incidence of right hemispheric brain injury and long-term consequences of marginal cerebral perfusion.

Anastomosis, Surgical↗
PubMed1990

Fetal intervention in obstructive uropathy: prognostic indicators and efficacy of intervention.

Management of the fetus with bilateral hydronephrosis is controversial; ability to predict outcome and efficacy of prenatal intervention are unknown. We studied 40 fetuses referred for ultrasonography, examination of fetal urine, and possible therapy. We retrospectively assigned fetuses to a good prognosis group if fetal urine was hypotonic (sodium less than 100 mEq/L, chloride less than 90 mEq/L, osmolarity less than 210 mOsm/L) and there was no ultrasonographic evidence of dysplasia; we assigned fetuses to a poor prognosis group if even one criterion was abnormal. Survival was greater in the good prognosis group than in the poor prognosis group (81% vs 12.5%; 87% vs 30%, excluding abortions) (p less than 0.005). We then attempted to assess the efficacy of prenatal urinary decompression by comparing outcome within the good and poor prognosis groups. Survival with intervention was greater in both the good prognosis group and the poor prognosis group (89% vs 70% and 30% vs 0%). In 6 of the 8 survivors in the good prognosis group, severe oligohydramnios was reversed by decompression. We conclude the fetal urine electrolyte levels and ultrasonographic appear helpful in predicting residual fetal renal function and neonatal outcome and that prenatal decompression may prevent the development of fatal pulmonary hypoplasia.

Amniotic Fluid↗
PubMed1990

Reduced-size lung transplantation in neonatal swine: technique and short-term physiological response.

Lung transplantation is now a clinical reality in adults but is limited by the scarcity of appropriate donors. The donor shortage is even more acute for neonatal and pediatric patients. Reduced-size lung grafts would expand the pool of appropriate cadaveric donors and allow HLA-matched living related lobar or segmental lung transplants. To evaluate this experimentally, we developed a model of pulmonary lobar transplantation in neonatal pigs and studied the acute hemodynamic response after transplanting the left lower lobe from a more mature donor to a neonatal recipient. Technical considerations included using the recipient atrial appendage for the pulmonary venous anastomosis. Nine pairs of pigs underwent left lateral thoracotomy. The recipient left atrial and pulmonary arterial pressures, cardiac output, and pulmonary vascular resistance were measured before pneumonectomy and after left lower lobe transplantation. Although the left atrial and pulmonary arterial pressures remained unchanged after transplantation, there was a 15% increase in pulmonary vascular resistance and a 23% reduction in cardiac output. Neither change was statistically significant. The distribution of blood flow through the left and right pulmonary arteries was unchanged after transplantation. We conclude that lobar transplantation is technically feasible in immature animals and that the pulmonary venous anastomosis to the left atrial appendage facilitates the procedure. This model may prove useful in studying lung transplantation in immature recipients and expedite implementation of reduced-size lung transplantation in neonatal and pediatric patients.

Anastomosis, Surgical↗
PubMed1990

Mediastinal pancreatic pseudocysts in children.

Mediastinal pseudocyst is an unusual complication of pancreatitis, with only four cases previously reported in children. The extent of the pseudocyst can be defined by computed tomography or magnetic resonance imaging scan and preoperative aspiration of cyst fluid for amylase level can establish the diagnosis. Endoscopic retrograde cholangiopancreatography to define ductal anatomy can help plan the appropriate drainage procedure. Although exceedingly rare, the diagnosis of pseudocyst should be considered for any cystic mass in the abdomen or thorax, even in the absence of elevated amylase or history suggesting pancreatitis.

Amylases↗