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Biomedical subjects

E Kiely

Publications and source records attributed to E Kiely.

At least 19 recordsLinked to original sources
PubMed2008

Familial Currarino syndrome presenting with peripheral primitive neuroectodermal tumour arising with a sacral teratoma.

This report illustrates a rare genetic disorder, Currarino syndrome, in association with an unusual malignant transformation to a peripheral primitive neuroectodermal tumour within a sacral teratoma. The triad of features consists of a presacral mass, partial sacral agenesis and anorectal anomalies. The most common presentation is constipation. In this case there was a history of constipation, teratomas and spinal abnormalities in many of the family members over three generations. Detailed family history taken at time of initial presentation may have prevented delay in diagnosis and averted the need for intensive treatment, which may well cause late sequelae.

Child, Preschool↗
PubMed2005

Short-pouch and low-anastomosis Duhamel procedure results in better fecal control and normal defecation pattern.

PURPOSE: The purpose of this study was to analyze the long-term outcome of the short-pouch and low-anastomosis Duhamel procedure and to evaluate the quality of life after pull-through. We also tried to answer the questions: Does the modified Duhamel procedure produce fecal continence? Is the quality of life correlated to normal bowel function (1-3 per day) without the use of laxatives? MATERIALS AND METHODS: Between January 1993 and January 2002, 53 patients, 44 (83 percent) males and 9 (17 percent) females, who underwent a Duhamel procedure were assessed retrospectively. Of the 53 children, 36 (67.9 percent) underwent a primary Duhamel procedure at our institute (Group A). Seventeen (32.1 percent) patients had a primary Duhamel pull-through and subsequently a stoma before a redo modified Duhamel procedure (Group B). The technical modification was creation of a short rectal pouch of 35 mm, achieving a low colorectal anastomosis 0.5 cm from or on the dentate line. All 53 patients were assessed prospectively by a disease-specific questionnaire. The total score provided a single index of the quality of life associated with fecal continence. RESULTS: For Group A (n = 36), the mean period of follow-up was 71.4 +/- 29 months. Constipation was seen in four (11.1 percent) patients. In two (5.6 percent) patients there was a mild degree of soiling. Sensation and urge to defecate was intact in 34 (94.4 percent) children who could wait to go to the toilet until it was socially convenient. For Group B (n = 17), the mean period of follow-up was 73.9 +/- 31.2 months. Constipation occurred in 17 (100 percent), encopresis in 14 (82.4 percent), and enterocolitis in 15 (88.2 percent) after the primary Duhamel procedure. In comparison, there was complete absence of constipation (100 percent), encopresis (100 percent), and complete resolution of enterocolitis (100 percent) after the redo modified Duhamel (P = 0.001). Continence to solid stools after primary Duhamel was seen in 5 (29.4 percent) vs. 17 (100 percent) after redo modified Duhamel. After the redo modified Duhamel all 17 (100 percent) patients have retained the sensation and urge to defecate and 15 (88.2 percent) can wait to get to the toilet until it is socially convenient. In all 53 patients, the quality of life was good in 86.8 percent (46/53) and correlated directly with fecal continence (r = 0.977). There was no correlation between age of patient and fecal continence (r = 0.597) and rate of fecal incontinence did not decrease with age. Direct correlation was seen with the presence of constipation and the incidence of late onset enterocolitis (r = 0.942). CONCLUSIONS: Short-pouch, low-anastomosis Duhamel pull-through procedure results in fecal continence and complete emptying. The above procedure has a positive impact on the quality of life in these children.

Anastomosis, Surgical↗
PubMed2004

Outcome and complications after resection of hepatoblastoma.

PURPOSE: The aim of this study was to review the outcome and complications after resection of hepatoblastoma treated over 2 decades in our institution. METHODS: Clinical, radiologic, and pathologic data were reviewed retrospectively, focusing on the outcome and complications. RESULTS: Between January 1978 and December 2002, 56 children were treated for hepatoblastoma. The age range was 0.08 to 8.74 years (median, 1 year). The right lobe was involved in 48%, the left lobe in 22%, and in 29% the main bulk of the tumour was centrally located. Surgical procedures included the following: hemihepatectomy in 62%, trisegmentectomy in 18%, extended hemihepatectomy in 16%, and liver transplantation and laparotomy in one patient each. Intraoperative complications occurred in 5(9%)--rupture of the tumour (1), haemorrhage from the contralateral lobe (1), a defect in the left hepatic duct (1), cardiac arrest from tumour embolus (1), and bleeding from the inferior vena cava (1). The mean blood loss was 280 mL (50 to 2,000 mL). Postoperative complications occurred in 12 patients (22%) including subphrenic abscess (3), adhesion obstruction (2), ischaemic stenosis of the bile duct (1), abdominal wound dehiscence (1), pyloric obstruction (1), and pleural effusion (2). Fifteen patients died, 14 as a result of tumour recurrence (mortality rate, 27%).

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Prolonged use of tissue plasminogen activator for bilateral lower limb arterial occlusion in a neonate.

The authors report the case of a 29-week-gestation twin with very severe vascular compromise of both lower limbs secondary to premature twin-twin transfusion. A tissue plasminogen activator (tPA) infusion was used locally for 13 days with complete recovery of perfusion to both legs, demonstrated by serial angiograms, thereby avoiding bilateral amputation. There were no side effects as a result of the continuous administration of tPA. The authors therefore suggest that the benefits of thrombolysis from a local infusion of tPA in neonates may outweigh the potential risks. This case report thus supports the view that under certain circumstances infusion of tPA in neonates may offer significant benefits. J Pediatr Surg 36:654-656.

Angiography↗
PubMed1999

Sacral dysgenesis associated with terminal deletion of chromosome 7q: a report of two families.

UNLABELLED: Most cases of sacral dysgenesis are considered to be sporadic events. We present two families in whom the presence of associated clinical features prompted specific investigation of chromosome 7, leading to the identification of an underlying chromosome 7q deletion causing sacral dysgenesis. All affected individuals had microcephaly and developmental delay. Detailed cytogenetic studies confirmed that all three affected individuals had a deletion of chromosome 7q associated with their sacral dysgenesis, developmental delay and related problems. The three affected patients were studied clinically, radiologically and cytogenetically. Eleven unaffected individuals from the two families were also investigated by genetic studies, specifically evaluating chromosome 7. CONCLUSION: It is important that detailed family history, evaluation of associated malformations and the overall clinical picture be considered in identifying the underlying diagnosis in cases of anal stenosis/sacral agenesis. The cases we present demonstrate the value of detailed chromosome studies in such situations.

Abnormalities, Multiple↗
PubMed1998

Hemisplenectomy for giant splenic cysts in children.

To analyse the clinical features and outcome following surgery of giant splenic cysts in children, all patients presenting to a children's hospital over the years 1987-1996 were reviewed. Variables analysed included presenting symptoms, method of diagnosis, operative procedure, and complications. Six patients (aged 8-16 years) presented with abdominal pain or a large abdominal mass. Ultrasound examination demonstrated large (>5 cm) unilocular splenic cysts. Five of the six children underwent hemisplenectomy without complication or blood transfusion. The remaining patient had an infected cyst, for which total splenectomy was necessary because of major intraoperative haemorrhage. Hemisplenectomy is based on accurate dissection of the splenic hilum with clear knowledge of the intracapsular vascular anatomy of the spleen. Preoperative vaccination is recommended in the event that splenectomy is required. This retrospective review concludes that hemisplenectomy for splenic cysts is a safe procedure in children.

Adolescent↗
PubMed1998

Elective partial splenectomy in childhood.

BACKGROUND & PURPOSE: The indications and results of elective partial splenectomy in children remain uncertain. The aim of this study was to determine (1) the indications for elective partial splenectomy (EPS), (2) the extent of splenic resection, and (3) the complications of EPS. METHODS: Patients who underwent EPS from three centers over a 10-year period (1986 through 1996) were studied. Variables analyzed included clinical presentation and indications for EPS, extent of resection performed, perioperative transfusions, and complications. Postoperatively, splenic viability was determined by regular Doppler ultrasound scan and splenic function on peripheral blood film analysis. RESULTS: EPS was attempted in 12 children for various conditions: giant epithelial cyst (n = 6), hypersplenism and metabolic disease (n = 4), pseudotumor (n = 1), and abscess (n = 1). One procedure was converted to total splenectomy because of thin splenic tissue around an infected giant cyst. No child required transfusion for splenic bleeding. There were no postoperative complications or deaths. Only 7 of the 12 children received prophylactic preoperative immunisation. In the 11 successful cases splenic remnant viability remains confirmed by ultrasound scan and normal blood film. There has been no significant splenic regrowth or recurrent hypersplenism in the four children with metabolic disorders. CONCLUSIONS: EPS is suitable for benign splenic conditions and can be performed without major blood loss. Preoperative vaccination is advisable. Up to 95% of the spleen can be safely removed, basing the blood supply of the residual spleen tissue on peripheral polar vessels, with adequate postoperative function.

Adolescent↗
PubMed1997

Meconium ileus: a ten-year review of thirty-six patients.

Of 36 neonates with meconium ileus secondary to cystic fibrosis treated over a 10-year period, twenty-one (58%) had simple uncomplicated disease while fifteen (42%) had complications which included perforation (5), volvulus (6) and atresia (5). Gastrografin enema was employed in 20 infants with relief of obstruction in 8 (40%). Operative procedures consisted of resection and primary anastomosis in seventeen patients, stomas were fashioned in six, three had an enterotomy with irrigation only and two had Bishop-Koop enterostomy. Post-operative complications developed in 5 (18%) of these 28 patients. The overall survival rate was 97%. The one death occurred in an infant with short bowel syndrome, patent ductus arteriosus, hydrocephalus and pulmonary damage. There were eight additional patients who had meconium obstruction in the absence of cystic fibrosis.

Cystic Fibrosis↗
PubMed1996

Intestinal glycosaminoglycans in neonatal necrotizing enterocolitis.

Advanced necrotizing enterocolitis (NEC) is a common neonatal surgical emergency of unknown aetiology. Despite improvements in the prognosis, the aggressive form of the disease is still associated with significant rates of morbidity and mortality. Recent evidence indicates that the extracellular matrix (ECM) is important in gastrointestinal development and glycosaminoglycans, major constituents of the ECM, are attenuated in inflammatory bowel disease. The hypothesis of this study was that changes in the nature and distribution of intestinal glycosaminoglycans occur in NEC. The distribution and nature of glycosaminoglycans were determined in 31 sections of well preserved resection margins and severely diseased bowel from eight neonates affected by NEC. An established histological method of glycosaminoglycans analysis using cationic gold with silver enhancement was employed in this study. The identity of specific glycosaminoglycans was also elucidated using a combination of cationic gold staining and glycanase digestion. In well preserved tissue, staining was seen throughout the full thickness of the bowel. The epithelial basement membrane and basolateral surfaces, lamina propria and submucosa were particularly prominent. In moderate disease, patchy loss of anionic sites was frequently observed with glycosaminoglycans-deficient areas adjacent to intact sites. In severe NEC, there was extensive loss of glycosaminoglycans in most of the sections examined. Glycanase analysis revealed that the glycosaminoglycans in well preserved tissue were sensitive to chondroitinase ABC and only vascular sites were sensitive to heparinase III. The consequences of glycosaminoglycans loss in NEC as demonstrated in this study are not known but modulation of gastrointestinal glycosaminoglycans could be important in the pathogenesis of NEC and may underlie some of the clinical manifestations of this condition.

Colon↗
PubMed1996

Resection and primary anastomosis in necrotizing enterocolitis.

Necrotizing enterocolitis (NEC) is the most common surgical emergency in the newborn. Up to half of babies with NEC develop advanced disease requiring surgical intervention. Options include peritoneal drainage under local anaesthetic, enterostomy only, resection and enterostomies, and resection with primary anastomosis. Resection with enterostomies is favoured by many paediatric surgeons but management of neonatal enterostomies can be difficult. The outcome of 26 infants undergoing surgery for advanced NEC over a 2-year period is reviewed. Resection and primary anastomosis was possible in 18 infants of whom two (11%) died. Recurrent NEC developed in four (22%) and strictures in three (17%) of these infants. An initial enterostomy was fashioned in eight infants, three following resection of necrotic intestine and five as a proximal diverting stoma in infants with pan-intestinal involvement. Five of these eight infants died (63%), giving an overall mortality of 27%. Primary anastomosis is an effective procedure following resection of grossly involved intestine in infants with NEC. The mortality and morbidity in this series compared well with those reported for staged procedures.

Anastomosis, Surgical↗
PubMed1995

Management of alimentary tract duplication in children.

Duplication of the alimentary tract is rare but potentially dangerous. Five of 72 children with alimentary tract duplication treated between 1973 and 1992 died from postoperative complications; a further ten required more than one operation. Ileal duplications were the commonest, occurring in 16 patients (22 per cent). Thoracoabdominal duplications were the most complicated and responsible for much of the overall morbidity and mortality. Surgical complications were related to the size and location of the duplication, communication with the gastrointestinal tract or vertebral canal, presence of heterotopic gastric mucosa and involvement of mesenteric vessels. Complete excision of the duplication should be possible in most cases.

Adolescent↗
PubMed1995

Scattered radiation in a neonatal surgical unit.

We studied the doses of "scattered" received by neonates in a Neonatal Surgical Unit by placing thermoluminescent dosemeters in various positions expect the direct beam. Doses of "scattered" radiation received by neonates as a results of exposure of their neighbours to X-ray examinations were found to be negligible. Sick neonates need not be moved from their neighbours undergoing exposure to X-rays.

Humans↗
PubMed1994

Manganese in long term paediatric parenteral nutrition.

The current practice of providing manganese supplementation to neonates on long term parenteral nutrition is leading to a high incidence of hypermanganesaemia. Magnetic resonance imaging (MRI) studies in adults on long term manganese parenteral nutrition have shown changes in TI weighted MRI images and similar findings in a neonate receiving trace element supplementation are reported here. Whole blood manganese concentration in the infant was 1740 nmol/l (or 8.3 times upper reference limit). In all neonates on long term parenteral nutrition with evidence of cholestatic liver disease so far investigated, the whole blood manganese concentrations were > 360 nmol/l (reference range 73-210). Manganese supplementation to patients on long term parenteral nutrition requires reappraisal, particularly in those who develop cholestatic liver disease associated with parenteral nutrition.

Cholestasis↗
PubMed1994

Unproven treatment in childhood oncology--how far should paediatricians co-operate?

Parents of children with terminal illness may try many different types of alternative and unproven treatment, not all recognised by the medical establishment. When active participation is requested difficult ethical dilemmas may arise. We present one such case, a child of five years with an inoperable posterior fossa brain tumour.

Airway Obstruction↗