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Biomedical subjects

H Flageole

Publications and source records attributed to H Flageole.

32 records · Page 2Linked to original sources

Intrauterine tracheal obstruction, a new treatment for congenital diaphragmatic hernia, decreases amniotic fluid sodium and chloride concentrations in the fetal lamb.

OBJECTIVE: To evaluate the effect of fetal tracheal occlusion on sodium and chloride concentrations in amniotic and tracheal fluid. SUMMARY BACKGROUND DATA: Intrauterine tracheal occlusion has been proposed to reverse pulmonary hypoplasia, an important prognostic factor in congenital diaphragmatic hernia. In early human trials, technical failure of the obstructive device has been reported. METHODS: Eight fetal lambs (gestational age = 95 days) were subjected to fetal tracheoscopy, and amniotic and tracheal fluid samples were taken. In multiple pregnancies (n = 6), amniotic fluid was also sampled from the contralateral amniotic sac and used as a control. Subsequently, endotracheal obstruction, using a detachable balloon, was performed. After 14 days, all fetuses were delivered, and sodium and chloride concentrations in amniotic and tracheal fluid were measured again. Statistical analysis was done using a two-tailed Student's t test, paired or unpaired as appropriate. RESULTS: In controls, between 95 and 109 days gestational age, no significant changes occurred in sodium or chloride concentrations in amniotic or tracheal fluid. After 2 weeks of tracheal obstruction, however, chloride and sodium concentrations in amniotic fluid decreased (chloride = 76.7 mEq/L vs. 107.6 mEq/L, p = 0.0003; sodium = 109.6 mEq/L vs. 125.9 +/- 5.2 mEq/L, p = 0.019). A concomitant increase in chloride and sodium concentration was observed in tracheal fluid (chloride = 145.4 mEq/L vs. 130.0 mEq/L, p = 0.047; sodium = 153.1 mEq/L vs. 142.9 mEq/L, p = 0.051). When comparing groups at 109 days, chloride and sodium concentrations in amniotic fluid were markedly lower in the treated group versus controls (p = 0.0004 and p = 0.05 for chloride and sodium, respectively). CONCLUSION: Complete tracheal occlusion in ovine fetuses results in a significant decrease of amniotic fluid sodium and chloride concentrations.

Amniotic Fluid↗

Hirschsprung's disease, imperforate anus, and Down's syndrome: a case report.

Patients with trisomy 21 have a higher incidence of several gastrointestinal anomalies. However, the coexistence of imperforate anus, Hirschsprung's disease, and trisomy 21 had not been reported previously. This report describes the case of an infant girl born with trisomy 21 and imperforate anus, without a fistula, who presented with bowel obstruction 3 months after anoplasty. The obstruction was attributable to Hirschsprung's disease. This was managed by a leveling colostomy in the descending colon, followed by an endorectal pull-through after 4 weeks. She has a normal stooling pattern 11 months after colostomy closure. Hirschsprung's disease should be suspected in infants with trisomy 21 who have constipation after repair of imperforate anus. The authors believe that the endorectal pull-through is the safest technique to use for Hirschsprung's disease after a previous anoplasty.

Abnormalities, Multiple↗

Recurrent intussusception: safe use of hydrostatic enema.

Recurrent intussusception (RI) occurs in 5% to 8% of patients with intussusception. The authors reviewed their 15-year experience to better define the management of RI. Among 258 patients, 28 (10.8%) had episodes of RI (37 episodes altogether). Twenty-two patients had a single recurrence, four had double recurrence, one had triple recurrence, and one had quadruple recurrence. RI and non-RI patients were compared with respect to symptoms. The incidence of vomiting and bloody stools was significantly lower in the RI group. Ten percent of RI patients had lead points. Thirty percent of RI episodes occurred within 24 hours, and 74% occurred within 6 months. The success rate of hydrostatic enema reduction for recurrent episodes was 62.8%, which is comparable to that for the initial episode (68.9%). Among patients with previous surgery, 36.3% of enemas were successful; after previous enema reduction, 75% were successful. There was no complication related to the enema. Enema reduction is safe and has a good success rate in cases of RI, even after previous surgical reduction.

Chi-Square Distribution↗

Fetoscopic Nd:YAG Laser Coagulation for Twin-Twin Transfusion Syndrome in Cases of Anterior Placenta

Laser coagulation of anastomosing placental chorionic plate vessels was suggested as a treatment of severe twin-twin transfusion syndrome in midtrimester. An anteriorly located placenta may hamper access and visualization of the vessels involved. We treated six women with completely anterior placenta at 18 to 22 weeks' gestation. Through a 2- to 3-cm minilaparotomy the uterus was accessed after retraction of the viscera. A flexible cannula was inserted through the uterine fundus, and a 35-degree, 0.20-mm minifibroscope and 400-mm fiber were introduced. All vessels crossing the intertwining membrane were coagulated in a continuous mode and a maximum output of 45 W. All fetuses survived the operation. One fetus died in utero 6 weeks postoperatively from cardiac failure, and its receptor twin was born alive. All other pregnancies are either continuing, or the women delivered two healthy babies. Thus far, total fetal survival is 91.6%, compared with results with posterior-sided placentas. Neodymium:yttrium-aluminum-garnet laser coagulation is also possible in case of anterior placenta, but requires a minimal maternal incision and general anesthesia to allow safe cannula placement. A bent scope allows for a larger placental surface to be treated.

Journal Article↗

Endoscopic In Utero Tracheal Plugging in the Fetal Lamb to Treat Congenital Diaphragmatic Hernia

A case control surgical trial was performed in 13 time-dated pregnant ewes to evaluate the feasibility and pulmonary effects of intratracheal obstruction by an endoscopically positioned balloon. Fifteen fetuses were subjected to tracheoscopic balloon obstruction for progressively longer periods (range 2-18 days). In case of multiple pregnancies, 10 other fetuses were used as negative controls. A 1.2-mm miniscope with a double-lumen sheath and a detachable balloon were used. Feasibility of the technique was assessed in all animals based on operating time, intraoperative complications, and tracheal obstruction. Pulmonary effects were assessed by lung-to-body weight ratio (LBWR) and morphometric terminal brachial density (MTBD). In 14 animals the trachea was successfully obstructed until delivery. Tracheoscopic manipulation time ranged from 3 to 14 minutes. One intraoperative death occurred, but the contralateral control fetus also died during the procedure. When obstruction lasted for more than 7 days, the mean LBWR was 0.060 &plusmn; 0.01 (range 0.051-0.075) and in controls 0.031 &plusmn; 0.01 (range 0.017-0.039, p <0.0005). The MTBD was 0.65 &plusmn; 0.59, compared with 1.30 &plusmn; 0.80 for controls. Using a simple and fast technique of fetoscopic tracheoscopy, the trachea can be obstructed, leading to pulmonary hyperplasia, which is of potential value when treating congenital diaphragmatic hernia.

Journal Article↗

Salvage of Soave-Boley endorectal pull-through by conversion to a classical Soave procedure.

The endorectal pull-through procedure described by Soave and subsequently modified by Boley to include primary coloanal anastomosis is one of the most commonly performed procedures for Hirschsprung's disease. The Boley modification carries a 5-10% risk of dehiscence of the colo-anal anastomosis. This is generally treated with proximal colonic diversion and delayed revision of the pull-through. We report the use of the original Soave procedure to treat this complication.

Anal Canal↗

Repair of congenital diaphragmatic hernia after weaning from extracorporeal membrane oxygenation.

Stabilization and delayed operation for patients with congenital diaphragmatic hernia (CDH) is now widely accepted. When preoperative extracorporeal membrane oxygenation (ECMO) is needed, most centers have CDH repaired on ECMO to minimize the risk of postoperative deterioration. The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks. They reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned. The records of all high-risk CDH patients with a gestational age of at least 34 weeks were reviewed. Eighteen patients were identified. None of the eight who were stabilized and operated on without ECMO required bypass postoperatively; all survived. Ten were placed on bypass, nine for stabilization before repair. Of the nine, seven (78%) were weaned from ECMO to conventional ventilation. Repair of the diaphragmatic defect was performed an average of 3.8 days later; none of these patients had severe pulmonary hypertension postoperatively, and all survived. Two could not be weaned before repair, one of whom had a complex congenital heart defect. This patient died. The other patient had repair on ECMO because of intrathoracic gastric volvulus. Severe blood loss prompted decannulation, and the patient died. One patient who was placed on bypass was transferred 10 days after having had repair elsewhere (at 4 hours of age). Pulmonary hypertension did not resolve, and the postmortem examination showed alveolar capillary dysplasia, with focal misalignment of the pulmonary vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal Membrane Oxygenation↗

Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome.

BACKGROUND: The purposes of this study were to study congenital central alveolar hypoventilation syndrome (CCAHS), to report the experience at our institution with this disorder, and to describe the surgical technique of diaphragmatic pacing. Three patients are in our diaphragmatic pacing program. They all have CCAHS, documented by means of lack of respiratory drive to hypercapnia and normal peripheral nerve and muscle studies. Two patients have associated Hirschsprung's disease. METHODS: The ages of patients at insertion of bilateral phrenic nerve pacemakers were 1, 2, and 5 years. A bilateral axillary thoracotomy in the third interspace was used in two patients, and anterior thoracotomy was used in one for insertion of electrodes on the phrenic nerves. Counterincisions in both flanks were used in all patients for insertion of the receivers in subcutaneous pockets. RESULTS: In all patients pacing was commenced within 1 week of the surgical procedure, because no primary pacemaker failures occurred. One patient has experienced no failure of the equipment and no infectious complications at 4 years. A second patient has had the pacemakers for 6 months without complications. The third patient underwent placement of bilateral pacemakers in 1984. In this patient a 10-year experience has resulted in subcutaneous implant failure on two separate occasions; also the presence of a staphylococcal empyema necessitated the temporary removal of one phrenic nerve electrode for 6 months, with successful reinsertion. All patients now receive mechanical assisted ventilation when sleeping and phrenic nerve pacing when awake. CONCLUSIONS: Pediatric surgeons should be aware of CCAHS because it may be treated with surgically implanted electrodes that allow for pacing of the diaphragm. The technique has an acceptable complication rate, and it can greatly decrease the impact of the disease on the lifestyle and activity of the patient. CCAHS also may be associated with Hirschsprung's disease.

Artificial Organs↗

Reoperation for cysts of the thyroglossal duct.

OBJECTIVE: To determine the causes of recurrence of thyroglossal duct cysts (TDCs). DESIGN: A case series. SETTING: A university-affiliated children's hospital. PATIENTS: Between 1974 and 1990, 121 children were treated for TDCs. They ranged in age from 1 month to 18 years. Of these 121 children, 23 had recurrent TDC. INTERVENTIONS: Simple aspiration of the cyst, incision and drainage, cystectomy without hyoidectomy and the Sistrunk operation. MAIN OUTCOME MEASURES: Initial procedures that led to recurrence and procedures that resulted in cure. RESULTS: Six children had recurrence of TDCs after simple cystectomy without hyoidectomy; all were cured by the Sistrunk operation. Fourteen children with infected cysts initially underwent a variety of procedures with recurrence and were eventually managed by the Sistrunk operation. TDCs recurred in eight of these. Three children without infected cysts had recurrence after the Sistrunk operation. Of 34 TDCs infected initially, 40% recurred, whereas of 87 TDCs that were not infected initially, only 8% recurred. CONCLUSION: Simple cystectomy without hyoidectomy and pre-existing infection are the main causes of recurrence of TDCs.

Adolescent↗

Progression toward malignancy of hamartomas in a patient with Peutz-Jeghers syndrome: case report and literature review.

Peutz-Jeghers syndrome, inherited in an autosomal dominant fashion, is characterized by hamartomatous polyps of the gastrointestinal tract and by mucocutaneous pigmentation. The frequency of gastrointestinal malignant disease in this syndrome is estimated to be 2% to 3%. The authors review reports associating Peutz-Jeghers syndrome with malignant disease and present a patient who had advanced jejunal adenocarcinoma in association with Peutz-Jeghers syndrome. It has not been determined with certainty whether the malignant lesions arise from hamartomas, from associated adenomatous polyps or from the normal mucosa. Histologic examination of the excised specimen from the patient reported in this paper showed areas typical of a hamartoma as well as areas of hyperplasia, adenoma with mild to severe dysplasia and carcinoma in situ all in the same polyp. These findings suggest that the hamartomatous polyps found in Peutz-Jeghers syndrome have the potential to undergo malignant transformation.

Adenocarcinoma, Mucinous↗

Substance P increases in vitro lymphokine-activated-killer (LAK) cell cytotoxicity against fresh colorectal cancer cells.

Gut-associated lymphoid cells are modulated by several gut hormones. We postulated that lymphokine-associated-killer (LAK) cell cytotoxicity of lymphocytes isolated from the gut mucosa may be increased by substance P (SP). Intestinal lamina propria mononuclear cells (LPMC) and colonic cancer cells were isolated from operative specimens by successive mechanical and enzymatic dissociation methods. Effector LAK cells were induced by culturing LPMC with recombinant interleukin-2 at a concentration of 250 U/ml. Substance P (10(-5) M) was added to the culture medium. Targets consisted of fresh colon cancer cells, HT-29 (cultured human colon cancer cell line), and control cell lines. After 4 days of incubation, cytotoxicity was measured using a 4-h 51Cr release assay. LAK cells alone showed moderate cytotoxicity against HT-29 and none against fresh colon cancer cells. LAK cells generated in the presence of substance P showed moderate cytotoxicity against HT-29 and strong cytotoxicity against fresh colorectal cancer cells. The percentage of cytotoxicity +/- SEM at various effector to target ratios was [(*) denotes P < 0.05 compared with above]: [table: see text] We conclude that substance P significantly increases LAK cell cytotoxicity against fresh colon cancer cells, but not against cultured cells.

Colorectal Neoplasms↗

Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience.

UNLABELLED: Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. METHOD: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. RESULTS: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. CONCLUSION: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

Abortion, Induced↗

Pericardial hemangioma presenting as thoracic mass in utero.

Pericardial hemangiomas are rare lesions. We present the case of an infant who was referred to our fetal diagnosis and treatment group for the presence of a left thoracic mass, pleural effusion, and mediastinal shift on fetal ultrasound. The characteristics of the lesion suggested the presence of a pulmonary sequestration. A chest radiograph done at birth was normal. At 2 weeks of age, an enhancing lesion of the left pericardium was identified on chest CT. A cardiac MRI demonstrated enhancement of the mass on T2-weighted images. The patient underwent thoracoscopic assessment of the mass for diagnostic purposes. Multiple lesions were identified along the left pericardium and diaphragm. A frozen section biopsy revealed a hemangioma. The natural history for hemangiomas is gradual regression; however, they may increase acutely in size and cause symptoms prior to involution. Investigations should be performed to identify the involvement of other organs. This case illustrates the need to closely follow all patients with prenatally diagnosed thoracic masses with CT imaging, even when they are asymptomatic and have a normal chest radiograph at birth.

Adult↗

[Necrotizing enterocolitis of the newborn. Review for the clinician].

With the advent of modern neonatology and the survival of most premature infants, necrotizing enterocolitis of the newborn (NEC) has become a relatively frequent illness. NEC, although affecting mainly premies, may still be found in any infant, even full term ones. We therefore believe that it is important for all physicians to become somewhat familiar with this entity. The pathogenesis of NEC is comprised of several variables: mesenteric ischemia, gastrointestinal immaturity, enteral feedings and even possibly infection. A diagnosis of NEC is based on a combination of clinical and radiological grounds. On radiographs, pneumatosis intestinalis and air in the portal vein are of special significance. NEC is classified in three broad categories: suspected NEC, definite NEC and advanced NEC. The treatment is either medical or surgical, depending on the severity and the evolution of the disease. It is important to emphasize that any infant who is deteriorating deserves very tight clinical and radiological follow-up. This follow-up should take place in a center where pediatric surgeons are ready to intervene rapidly should there be a need. Even if in some cases NEC is very severe, sometimes fatal, approximately 85% of infants suffering from it survive and among them more than 70% do so without any long term sequelae.

Diagnosis, Differential↗