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R M Filler

Publications and source records attributed to R M Filler.

At least 37 records · Page 2Linked to original sources
PubMed1997

[The treatment of the tracheobronchomalacia in pediatric age].

OBJECTIVE: Aortopexy is the more extended treatment for severe tracheomalacia, when it fails, reintervention and other procedures are necessary. We present our experience in the treatment of this pathology. MATERIAL AND METHODS: Tracheomalacia, bronchomalacia and tracheobroncomalacia cases during a twelve year period (1983-1995) were reviewed (type, age, symptoms, surgical procedures and results were collected). RESULTS: Nineteen patients (9 males, 10 females) presented tracheobronchomalacia in our Hospital. Diagnosis was made by bronchoscopy in all of them: nine cases of tracheomalacia, five bronchomalacias and five suprastomal malacias were found. Mean diagnosis age was twenty four months (newborn-twelve years). Ten patients were managed nonoperatively, five with anterior cricoideal suspension and two cases were treated by aortopexy; one of them, after 2 months of respiratory stridor and recurrent respiratory arrest, needed an endoscopically placed intraluminal expandable Palmaz Prothesis. This prothesis was removed two years later. In this moment, the patient is doing well without stridor or respiratory symptoms.

Bronchial Diseases↗
PubMed1997

Laparotomy or drain for perforated necrotizing enterocolitis: who gets what and why?

Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However, for neonates less than 1,000 g survival in the drained group was 69% compared to 22% for the laparotomy group (P <.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

Drainage↗
PubMed1997

Endogenous hyperinsulinism: diagnosis, management, and long-term follow-up.

Persistent hyperinsulinemic hypoglycemia of the neonate (PHHN) usually presents in the neonate or infant. Although rare, the possibility of PHHN should be considered in all neonates who have unremitting hypoglycemia. Untreated hypoglycemia results in severe neurological disability or death. An inappropriately elevated serum insulin level and low serum ketone and free-fatty-acid concentrations in the presence of hypoglycemia confirm the diagnosis. Early diagnosis and aggressive medical management have reduced the morbidity associated with PHHN. Pancreatic resection is necessary for long-term control of hypoglycemia when medical therapy fails. Ninety-five percent pancreatectomy has been the procedure of choice at the author's institution and other pancreatic centers. However, a review of the authors' experience and of in the English-language literature has demonstrated a 33% failure rate, requiring further surgery or medical management. Furthermore, long-term follow-up showed that diabetes develops in most children who undergo 95% pancreatectomy. The high failure rate of 95% pancreatectomy and the ultimate development of diabetes in virtually all children suggest the alternative approaches should be considered.

Combined Modality Therapy↗
PubMed1996

Excess zinc associated with severe progressive cholestasis in Cree and Ojibwa-Cree children.

BACKGROUND: High hepatic copper concentrations have been reported in several liver disorders. We report six Native Canadian children with severe chronic cholestatic liver disease, who had excess hepatic copper and zinc. METHODS: The children, aged 22 months to 8 years, came from northern Ontario, Canada. All were referred for possible liver transplantation because of end-stage liver disease. We examined explanted liver samples (or liver biopsy material in one case) by scanning transmission electronmicroscopic (STEM) X-ray elemental microanalysis and atomic absorption spectrophotometry. Samples from four controls (two with no liver pathology, one with biliary atresia, and one with Wilson's disease) were also analysed by atomic absorption spectrophotometry. FINDINGS: The explanted livers showed similar distinctive signs of advanced biliary cirrhosis, and on electronmicroscopy there were dense deposits in enlarged lysosomes and in cytoplasm. Hepatic copper concentrations were many times higher in the five patients with measurements (47.6-56.9 microgram/g dry weight) than in two samples of normal control liver tissue (2.3 and 2.9 microgram/g). Similarly, hepatic zinc concentrations were many times higher in the patients than in controls (104-128 vs 1.9-3.2 microgram/g dry weight). INTERPRETATION: The excess copper may be due to chronic cholestasis but the excess zinc is unexplained. Since three of the patients are related (shared grandparents), a genetic disorder of metal metabolism is possible, but we cannot exclude environmental factors.

Child↗
PubMed1996

Primary hyperparathyroidism in a paediatric hospital.

We retrospectively reviewed the presentation and management of children with primary hyperparathyroidism (PHPT) from 1973 to 1995 at a paediatric tertiary-care centre. There were 11 patients (6 females), aged 12.3-17.7 years at presentation, with sporadic PHPT confirmed by histopathology (single adenoma). Presentation consisted of renal colic, or non-specific gastrointestinal, musculoskeletal or neurological symptoms. Misdiagnosis was common until hypercalcaemia was identified, 0.5-24 months after onset of symptoms (mean 7.7 months). All patients had hypercalcaemia and low-normal serum phosphate. The parathyroid hormone (PTH) radioimmunoassay used before 1986 was elevated in 1/4 patients; the intact PTH assay used after 1986 was elevated in 7/7 patients. At presentation, six had end-organ damage: band keratopathy, renal lesions, and/or bone disease. Preoperative localization was accurate in 0/4 patients diagnosed before 1986, but 5/7 patients diagnosed after 1986: three by ultrasound or sestamibi scan alone, and two by ultrasound and technetium scan. Surgical outcome was not dependent upon the accuracy of pre-operative localization. PHPT is rare in children but usually associated with end-organ damage, presumably due to delayed diagnosis. It should be considered in the differential diagnosis of unexplained non-specific complaints. The intact PTH assay greatly assists pre-operative diagnosis. The usefulness of pre-operative localization requires further research.

Adolescent↗
PubMed1996

Central venous access catheters in children with haemophilia.

Twenty-five central venous lines (two external 23 subcutaneous ports) were placed in 19 boys with haemophilia A (n = 17) or B (n = 2). The mean age of the boys was 4.9 years (range 0.2-15.3 years). The haemophilia was severe (factor level < 1%) in 18 boys and moderate (factor level 3%) in one. Three boys had circulating inhibitors and three were positive for human immunodeficiency virus (HIV)-1 antibody. Central venous lines were placed to facilitate intermittent factor replacement therapy (n = 6), long-term factor prophylaxis (n = 9), induction of an immune tolerance protocol (n = 2) or therapy for acquired immunodeficiency syndrome (AIDS)-related complications (n = 2). The ports remained in place for 15795 days (mean 687 days, range 11-2059 days). The frequency of port-related sepsis was 48% (11/23 ports in eight boys) or 0.7 port infections per 1000 patient days. Ports were removed from five boys with an unresolved infection (four with Staphylococcus aureus sepsis and one with Pseudomonas sp. sepsis). Other complications requiring port removal included a catheter tip placed too high in the venous system (n = 1), severe persistent pain associated with needle access of the port (n = 1) and a subclavian vein thrombosis (n = 1). Both the benefits and risks of a subcutaneous port should be considered when deciding whether to place this device in a very young child with haemophilia.

Acquired Immunodeficiency Syndrome↗
PubMed1995

The use of expandable metallic airway stents for tracheobronchial obstruction in children.

Expandable metallic angioplasty stents (Palmaz stent) have been implanted in the trachea and/or bronchi of seven children. Three children had severe tracheal stenosis after tracheoplasty for congenital tracheal stenosis repair, and four had tracheomalacia or bronchomalacia with or without vascular compression. The mean age at stenting was 9.7 months (range, 2 to 15 months). Balloon expandable stents were inserted into the trachea or bronchus through a 3.5-mm bronchoscope under fluoroscopic control. Initially a single tracheal stent was used for all patients except for one with obstruction in the trachea and both bronchi, in whom three stents were implanted. Three children had recurrent airway obstruction 1 month later; one was cured with a second stent; one child died 1 year later; and the other is being treated for heart disease. The others have no serious respiratory problems. The stents in all have been in place for 1 to 25 (mean, 11) months. No immediate complications were noted. Early and late bronchoscopy showed incomplete epithelialization of the stent and patches of granulation tissue on it. Two stents were removed bronchoscopically, one at the completion of treatment for tracheomalacia and the other at the time of recurrent airway obstruction. This preliminary experience indicates that expandable metallic stents have a useful role in the treatment of selected lower airway obstructions.

Airway Obstruction↗
PubMed1994

Postpericardiotomy syndrome and chylopericardium: two unusual complications after aortopexy for tracheomalacia.

In two boys (aged 10 years and 7 months), large symptomatic pericardial effusions developed after aortopexy for tracheomalacia. Both patients underwent percutaneous pericardial drainage. The delayed presentation (3 weeks postoperatively) and associated findings in the 10 year old were typical of postpericardiotomy syndrome, and he had an uneventful recovery after a course of aspirin therapy. The 7 month old had chylopericardium and was treated with pericardial drainage and a low-fat, medium-chain triglyceride diet. Both cases represent previously unreported complications of aortopexy.

Aorta, Thoracic↗
PubMed1994

Tracheoplasty without stent, using preshaped cryopreserved cartilage allografts in neonatal pigs.

The management of congenital and acquired laryngotracheal stenosis in children often includes tracheoplasty with different materials. Autologous cartilage is the most commonly described graft for tracheal reconstruction. In 12 1-week old piglets a tracheal defect was created and repaired using cryopreserved allografts sculpted before preservation into a newly designed shape. In half the animals (group 1), a free pericardial autograft was interposed on the luminal surface of the cartilage graft. A stent was not used in any animal. The animals grew normally and were killed after 2 months. The grafted tracheas showed maintenance of their structural support, and no evidence of narrowing of the grafted area in comparison to the trachea above the graft. The lumen was lined by ciliated respiratory epithelium. There was no identifiable pericardium at the time of sacrifice and no differences between the two groups. This study shows that in neonatal pigs, cartilage allografts, sculpted before cryopreservation, are a suitable alternative to autografts to cover tracheal defects and can be used without stenting. No advantages were identified when interposing a free pericardial graft.

Animals↗
PubMed1994

Pediatric malignant pheochromocytoma.

Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.

Adolescent↗
PubMed1994

Tracheomalacia.

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Aorta↗
PubMed1994

Tracheomalacia.

Tracheomalacia describes a generalized or localized weakness of the trachea that results in excessive narrowing of the tracheal lumen during expiration or whenever intrathoracic pressure increases. Severe forms of tracheomalacia are characterized by life-threatening apneic spells, inability to extubate the airways, and episodic pneumonia. Aortopexy has proved to be an effective method of treatment for most of these conditions when seen in children. When aortopexy fails to prevent tracheal obstruction, a support applied to the external circumference of the trachea can be useful to prevent airway collapse.

Child↗
PubMed1993

Value of CT in determining the resectability of hepatoblastoma before and after chemotherapy.

OBJECTIVE: The purpose of this study was to assess the accuracy of CT for determining surgical resectability of hepatoblastoma before and after chemotherapy. MATERIALS AND METHODS: Preoperative and postoperative CT scans of 15 children with surgically confirmed hepatoblastoma were retrospectively reviewed and correlated with surgical findings and pathologic analysis. RESULTS: Before chemotherapy, 12 of the 15 children had unresectable tumors because of bilobar involvement, metastatic disease, or vascular extension. After chemotherapy, tumor volumes decreased by 20-98%, with increased areas of low attenuation and calcification. Periportal areas of low attenuation (four of nine), abdominal adenopathy (four of four), and lung nodules (four of six) disappeared after chemotherapy. On the basis of CT and clinical findings, all tumors were thought to be resectable. Surgical correlation showed that the extent of hepatic tumor had been overestimated on immediate preoperative CT scans in three children (tumor was staged as bilobar, but only single-lobe resection was required). Correlation between areas of low attenuation in the tumor and necrosis in pathologic specimens was poor. Tumor invasion of periportal lymphatics was seen in one of five children with periportal areas of low attenuation. Postoperatively, five of 13 children had CT abnormalities, three at the resection margins with calcification, low attenuation, or both. Hepatic or abdominal disease has not recurred, and 12 of the 14 children who survived surgery are well. One child who had abnormal findings on preoperative chest CT died of pulmonary metastases. Another in whom chemotherapy was limited because of toxic effects died after tumor recurred in the liver. CONCLUSION: Pretreatment CT scans cannot be used to predict ultimate resectability of hepatic tumors; nor are preoperative scans always accurate for judging exact lobar involvement. Absence of disease as shown on CT scans correlated with absence both at surgery and at follow-up. Postoperative hepatic changes are common and do not necessarily reflect recurrent or residual tumor.

Carcinoma, Hepatocellular↗
PubMed1992

Limited pulmonary resections for congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformations (CAMs) are rare lung lesions characterized by the presence of a multicystic mass of pulmonary tissue. To clarify the surgical management of CAM of the lung, we reviewed our institutional experience with 34 patients with histologically documented CAM. Symptoms developed in the first 2 days of life in 20 patients; 14 patients developed symptoms a median of 69 days (range, 22 days to 15 years) after birth. A diagnosis of CAM was confirmed at thoracotomy in all patients. Four patients were treated with a limited pulmonary resection for disease confined to one lobe. Seven patients underwent a composite resection involving a lobectomy plus limited resection of the second lobe. One patient underwent pneumonectomy for a severely malformed lung. The remainder of the patients were treated with anatomic lobectomy. The only recurrence was a patient who had a partial cystectomy performed at the initial operation. We conclude that in the appropriate malformation subtype, limited pulmonary reactions can preserve lung tissue and may prevent subsequent complications of CAM.

Adolescent↗
PubMed1992

Juvenile hemangiomas involving the thoracic trachea in children: report of two cases.

Two 3-month-old girls presented with posterior mediastinal juvenile hemangioma (JH), a benign tumor rarely found in the mediastinum. Incomplete resections were performed. Over 4 and 7 months, respectively, the hemangiomas recurred and grew through the tracheal wall into the tracheal lumina, reducing the airway by 90% in one case and 70% in the other. The recurrences were resected, including five tracheal rings in the first case and four in the second. In case 1 the tumor recurred in the trachea 2 months later. A tracheostomy was performed, and corticosteroids were administered for 6 months. The tumor involuted and the patient was decannulated after 18 months; she is doing well 7 years later. The patient in case 2 has been well in the 18 months since her second operation. Airway obstructions can be treated by tracheal resection when conservative management fails.

Airway Obstruction↗
PubMed1992

A 20-year review of pediatric pancreatic tumors.

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.

Adenoma, Islet Cell↗
PubMed1992

Clinical factors affecting mortality in children with malrotation of the intestine.

In children with symptoms secondary to malrotation of the intestine, a retrospective statistical study was undertaken to identify factors associated with an increased risk of mortality. Between 1964 and 1989, laparatomy was performed on 182 children. For study purposes, the children were divided into three groups. Group I included 71 patients with an obstruction only in the duodenum. The remaining 111 children, all of whom had midgut volvulus, were further divided; those without gut necrosis (79) into group II, and those with necrosis (32) into group III. One child each died in groups I and II, and 15 in group III. In all children we evaluated the relationship between mortality and age at presentation, presence of associated serious abnormalities, time from onset of symptoms to surgery, and the presence of necrotic bowel. For group III, we considered the influence of percentage of bowel resected on mortality. In these 182 children the factors associated with an increased risk of mortality were presence of necrosis (P < .0001), presence of other abnormalities (P = .0008), and younger age (P = .0084). Time from onset of symptoms to surgery was not associated with statistically increased risk of mortality. The 1% mortality noted in children without intestinal necrosis (group I and II) was related to associated abnormality. For group III the estimated probability of survival ranged from .999 for patients with 10% of intestinal necrosis to .351 for whose with 75% of intestinal necrosis, assuming the best prognostic conditions (patient older than 3 months with no associated serious abnormalities).

Abnormalities, Multiple↗