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Biomedical subjects

R J Fitzgerald

Publications and source records attributed to R J Fitzgerald.

At least 37 records · Page 2Linked to original sources
PubMed1993

Tumours of heterotopic salivary tissue in the upper cervical region in children.

Heterotopic salivary tumours in the upper neck are rare. Three children, one with mucoepidermoid carcinoma and two with pleomorphic adenoma in ectopic cervical sites are presented. Local complete excision was successful in the patients with pleomorphic adenoma. More extensive surgery was undertaken for the mucoepidermoid carcinoma. All three patients remain disease free at 3-6 years follow-up. Oncogenesis of heterotopic salivary tissue entrapped in an upper cervical lymph node during embryogenesis is a possible etiological mechanism. In a patient with a cervical malignant salivary tumour, a careful search should be made to find a primary tumour. In the event of a negative search, this should be considered the primary tumour and treated appropriately.

Adenoma, Pleomorphic↗
PubMed1993

The use of a long split rectal tube to aid in pull-through procedures for imperforate anus.

Problems with loss of correct orientation of the pull-through rectal pouch during posterior sagittal anorectoplasty (PSARP) for high imperforate anus can occur during laparotomy after perineal closure is complete. A simple modification of the de Vries-Peña technique is described that facilitates preservation of orientation and precise anatomical neomuscle construction. A slotted rubber tube preserves correct anatomical orientation of the neorectal pouch and allows accurate construction of the muscle complex around the tube.

Anus, Imperforate↗
PubMed1993

Gastrointestinal manifestations of neurofibromatosis in childhood.

Von Recklinghausen's neurofibromatosis (VRN) is a familial disorder characterised by a generalized neuroectodermal dysplasia--symptomatic involvement of the gastrointestinal tract is rare. We report on our experience with two children who had proven gastrointestinal neurofibromas and highlight the need for careful assessment in children with VRN who develop abdominal pain.

Child↗
PubMed1993

Heterotopic gastric cyst of the tongue in a newborn.

Gastrointestinal heterotopia in the tongue is a rare condition. A male newborn was noted to have a cystic swelling in the anterior part of the dorsum of the tongue. This was surgically excised. Histology revealed it to be a heterotopic gastrointestinal cyst. A literature review was undertaken because of the rarity of this lesion. As most of these cysts are present from birth, they are likely to be developmental in origin.

Choristoma↗
PubMed1993

Extrarenal Wilms' tumor.

Extrarenal nephroblastoma (Wilms' tumor) is very rare with to date just 34 cases described. Two such cases out of a total of 61 patients with WT presenting to a single institution over a 10-year period are presented. Both children, a boy aged three years and a girl aged four years, both presented with an abdominal mass which had crossed the midline. Ultrasonography and contrast studies in the two patients revealed retroperitoneal tumors distinct from kidneys, but unilateral hydronephrosis was noted in both patients. Primary surgical excision was performed and both patients grouped as stage III favorable histology. Postoperative treatment included chemotherapy and radiotherapy as per UKCCSG protocols. The patients are clinically well with no evidence of disease 7 years and 20 months respectively from diagnosis. An individualized approach to treatment of extrarenal WT is advocated.

Child, Preschool↗
PubMed1993

Laryngo-tracheo-oesophageal cleft: a plea for early diagnosis.

Congenital laryngeal clefts are rare. This paper reports on the management of 3 patients with Type 2 laryngo-tracheo-oesophageal cleft. One patient died following tracheopexy after successful cleft closure. Prognosis is related to the presence of other major anomalies, the early diagnosis of the lesion and institution of appropriate respiratory and nutritional care prior to correction of the defect. A greater awareness of the condition combined with aggressive diagnostic endoscopy should result in early diagnosis and improved survival.

Abnormalities, Multiple↗
PubMed1993

Biochemical properties of Streptococcus sobrinus reisolates from the gastrointestinal tract of a gnotobiotic rat.

Streptococcus sobrinus strain 6715-13-201 was inoculated into the oral cavity of a gnotobiotic rat and then reisolated from different portions of the gastrointestinal tract. Fourteen isolates, selected on the basis of their colonial morphology, were then screened for their ability to adhere to saliva-coated hydroxyapatite (SHA) in vitro, and their ability to produce extracellular polysaccharide from sucrose, and low pH in glucose broth. Certain isolates were also tested for their cariogenic potential as monoinfectants in gnotobiotic rats. All isolates differed in their abilities to adhere to SHA, with most showing an increased level of adhesion in the presence of sucrose, but this did not correlate with their ability to be aggregated by dextran. Most isolates were capable of producing glucosyltransferases (with only one exception) and dextranases (also one exception). There was more variability in the production of dextranase inhibitor. No isolate was capable of producing dextranase inhibitor in the absence of dextranase production. There were no correlations between the ability of isolates to adhere in vitro or produce/utilize polysaccharides and their ability to produce caries in vivo. Due to the differences between strains in their abilities to adhere, produce polysaccharides, utilize polysaccharides or produce a low pH and the lack of correlation between any of these parameters and cariogenicity, the results suggest that the ability of strains to colonize and produce caries depends on a number of different characteristics, no one of which is essential.

Animals↗
PubMed1993

Neuroblastoma--a surgical perspective.

The role of an aggressive surgical policy in the management of neuroblastoma (NBL) was examined in a retrospective study from a total of 57 patients presenting to a single institution between 1979 and 1989. Surgery consisted of either primary excision of tumour or elective resection following intensive chemotherapy. Two year disease-free survival (DFS) for the entire group was 100% for Stage I patients (n = 2), 86% for Stage II (n = 7), 55% for Stage III (n = 11), 12% for stage IV (n = 33) and 50% for stage IVs (n = 4). In all long-term survivors, surgical excision of primary tumour had been achieved. Elective surgery of primary tumour was not performed in six patients with Stage IV disease; median survival for these patients was eight months compared with 19 months for those other patients with Stage IV disease who did have surgery +/- high dose melphalan and autologous bone marrow rescue. Postoperative complications were documented in 13 of 48 operated patients (27%), emphasising the technical challenges encountered in resection of NBL. Age at presentation and site of primary tumour were major factors affecting prognosis: patients who presented < 1 years of age (n = 15) achieved 80% DFS, > 1 year and < 2 years (n = 12), 33%, and > 2 years (n = 30) 13%; 10 of 11 patients (91%) with primary supradiaphragmatic disease are alive and well compared with 10 out of 46 (22%) with infradiaphragmatic disease (P = 0.01). Based on the experience from this centre, it would appear that surgery can be curative for patients with Stage I, II and III disease but can only, at best, prolong DFS for Stage IV patients. Alternative therapeutic strategies are indicated for this latter group of patients.

Abdominal Neoplasms↗
PubMed1992

Primary hepatic tumours in children: a 26-year review.

Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.

Antineoplastic Agents↗
PubMed1992

Delayed primary anastomosis for esophageal atresia: 18 months' to 11 years' follow-up.

In 1977 we started treating babies with isolated esophageal atresia by delayed primary anastomosis and in 1981 reported our early experience in five cases treated between 1977 and 1979. Since 1979, 11 further consecutive cases have been managed by initial gastrostomy followed by delayed primary esophageal anastomosis. Their mean gestation was 35 weeks (range, 28 to 40 weeks) and mean birth weight was 2,040 g (range, 1,140 to 2,720 g). The esophageal gap between the two ends when assessed initially at fluoroscopy ranged from 2.2 to 4.5 cm (mean, 3.2 cm). Age at delayed primary anastomosis ranged from 6 to 20 weeks. Anastomotic leak occurred in three babies in the immediate postoperative period and all were successfully managed conservatively. Eight of the 11 patients developed anastomotic strictures; seven cases required 1 to 5 esophageal dilatations. One patient who did not respond to multiple esophageal dilatations required resection of an esophageal stricture. One patient died at 15 months of age of unrelated causes. The 10 surviving patients have been followed-up from 18 months to 11 years. At follow-up, seven patients were eating normally. Three patients had swallowing difficulties and all three were found to have esophageal strictures on barium swallow, two of them also had gross esophageal reflux and hiatus hernia. The height and weight in the 10 patients varied from 3rd centile to 75th centile. Delayed primary anastomosis is feasible in cases of isolated esophageal atresia and the patient's own esophagus is the best. A more aggressive approach should be applied to gastroesophageal reflux in these patients.

Anastomosis, Surgical↗
PubMed1992

The changing role of surgery in the management of rhabdomyosarcoma.

A retrospective analysis was performed of 56 patients presenting to Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland, over a 25 year period with a diagnosis of rhabdomyosarcoma, in an effort to assess the changing role of surgery in the management of this disease. There were 38 males and 18 females; the age range at presentation was from birth to 13 years with a median of three years. Head and neck tumors occurred most frequently (23) followed by pelvic (14), paratesticular (6), extremities (6), gastrointestinal (4) and thoracic (3). Prior to 1971 there were no survivors while a doubling of survival has been observed within the past decade (31% to 63%). The minimum period of follow-up was four years. Surgical intervention ranged from total primary excision (20) to biopsy only (21) with total or subtotal resection performed electively in 15. Surgical evaluations, including examinations under anesthesia, staging laparotomy and second look laparotomy were also invaluable in assessing disease status. The incorporation of multiagent chemotherapy together with more selective use of radiotherapy has enabled a more conservative role to be adopted by the surgical oncologist thereby minimizing longterm sequelae.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Renal function following extracorporeal lithotripsy in children.

Although extracorporeal shockwave lithotripsy (ESWL) has revolutionised the management of urinary calculous disease, the long-term effects of ESWL on renal function are not known. This study of 18 children demonstrated a mild, statistically insignificant decrease of 15% in DTPA measured glomerular filtration rate following ESWL. In view of this, we recommend that pretherapy and posttherapy estimates of renal function be obtained in all patients undergoing ESWL. Long-term follow-up is necessary to further quantify the effects of ESWL on the developing kidney.

Adolescent↗
PubMed1991

Are the selection criteria for the conservative management in spina bifida still applicable?

Management of spina bifida is still controversial. At one extreme is the policy of total care in all cases while some form of selection is practised in many centres. We reviewed 104 patients treated over the last five years. Forty-nine patients, who had one or more adverse criteria, were managed conservatively. Twenty-four of these 49 patients required VP shunt insertions subsequently. Four patients had delayed closure of back lesions, three of them were followed by shunts. There was an overall increased (57%) incidence of surgical intervention in conservatively managed patients over the last five years as compared with 34% from our previous report. We found no difference in the timing of insertion of shunts in actively and conservatively managed patients in this study. The mortality in the actively and conservatively managed patients was 7% and 47% respectively. Eighty-five per cent of the patients who had no surgery died at an average age of 23 days. Patients who died without surgical intervention did not survive long enough to warrant surgery, while the mortality was 20% and had among those who survived surgical intervention. Paraplegia alone was not a predictor of high mortality but the presence of two or more adverse criteria were more accurate indicators of poor prognosis. As 78% of all the deaths in conservatively managed patients occurred within three months, reappraisal of management policy is necessary in the infants surviving beyond three months of age as death is less likely after this time.

Humans↗
PubMed1991

Ultrasonography to diagnose and exclude intussusception in Henoch-Schönlein purpura.

Abdominal pain is a frequent symptom in the child with Henoch-Schönlein purpura and raises the suspicion of intussusception or perforation. One hundred and fifty two children with a diagnosis of Henoch-Schönlein purpura over 11 years were reviewed. Of these 60 had abdominal pain, 19 gastrointestinal bleeding, and nine were suspected intussusception. Intussusception was confirmed in two of these cases with ultrasonography. Ultrasound is an important tool in the early diagnosis of intussusception complicating Henoch-Schönlein purpura. Where the intussusception appears loose an expectant policy, with careful monitoring, may allow spontaneous reduction. It may also be used in monitoring patients for postoperative recurrence of intussusception, mural haematoma, and uncomplicated intestinal vasculitis with oedema.

Child↗
PubMed1991

Evaluation of conservative therapy for exomphalos.

Two management patterns were identified in 36 patients with exomphalos--primary surgical closure and initial topical therapy with delayed surgical closure. Primary surgical closure of minor exomphalos was well tolerated in 15 patients, but was associated with a high local and systemic morbidity rate in 14 patients with major defects. In contrast, initial topical therapy with silver sulphadiazine and delayed closure in seven matched patients with a major defect were well tolerated and did not prolong duration of hospitalization. Enteral feeding was more readily established and subsequent fascial closure facilitated in the conservatively treated group. It was suggested that this method should be more often considered in the management of all instances of major exomphalos.

Administration, Topical↗
PubMed1990

An alternative approach to pyogenic hepatic abscess in childhood.

Childhood pyogenic hepatic abscess is rare and remains a difficult diagnostic problem. Seven cases in six children were treated over a 13 year period. There was a recurrence in one patient 7 years after the first presentation. Four patients had identified predisposing factors, namely, chronic granulomatous disease, acute appendicitis, previous abdominal surgery and umbilical vein catheterization, while the other two were cryptogenic. There were no specific symptoms and signs but the combination of unexplained pyrexia, upper abdominal tenderness, hepatomegaly and leucocytosis should raise the suspicion of hepatic abscess. Four cases were diagnosed by ultrasound, one by radionuclide scan and the other two at laparotomy. Treatment in the earlier years was by transabdominal drainage. In the later part of the series, percutaneous catheter drainage using ultrasound guidance was achieved with satisfactory result. Ultrasonography can provide an early diagnosis and effective treatment can be achieved with percutaneous catheter drainage in combination with appropriate antibiotic therapy.

Adolescent↗