Bairbre Connolly

LYMPHOPROLIFERATIVE DISORDERS AFTER ORGAN TRANSPLANTATION IN CHILDREN

BACKGROUND After organ transplant, patients are at risk of posttransplant lymphoproliferative disorders (PTLD). The purpose of this study was to analyze 26 pediatric cases of PTLD observed at our institution between 1988 and 1996, and to evaluate the validity of the Society for Hematopathology Workshop (SHPW) 1997 classification in our patient population. METHODS Charts were reviewed for analysis of incidence, clinical course, and outcome. Tissue samples were classified by a pathologist according to SHPW recommendations. RESULTS By morphology, 20 were monomorphic, 5 polymorphic, and 1 hyperplastic. Assessment of lineage by morphology, molecular studies, and immunophenotyping did not correlate in six cases. By immunophenotyping, 12 were B cell, 4 T cell, 8 mixed B/T cells, and 2 undetermined. The 20 patients evaluable for treatment efficacy were treated with various therapeutic combinations, including immunosuppressive drug reduction, acyclovir/ganciclovir, interferon-alpha, immunoglobulins, surgery, and local irradiation. No patient received systemic chemotherapy. Thirteen patients achieved complete remission and 3, partial; 1 died 5 days after starting therapy, and 3 of progressive disease. Adverse prognostic factors included low platelet or neutrophil counts; stage III-IV and SHPW morphology were marginally significant. CONCLUSIONS The majority of patients eligible for treatment can be cured with immunosuppressive drug reduction and antiviral drugs, along with surgery and irradiation when indicated. Systemic chemotherapy or innovative approaches may have a role in unresponsive cases. Morphologic SHPW grouping is feasible and seems to have clinical relevance. However, correlation with clonality and immunophenotyping is not always possible, necessitating modifications including segregation of descriptive morphology from clonality and cell origin.

Spontaneous reduction of intussusception: clinical spectrum, management and outcome

Background. To analyze the spectrum of clinical features, management and outcome of children with documented spontaneous reduction of intussusception (SROI).¶Materials and methods. Review of records of 50 children (33 boys, ¶17 girls; age range 11 days–15 years; mean age 4 years) with documented SROI, in whom intussusception was initially diagnosed by sonography (US) in 44, air enema in 2, and computed tomography in 4, in the 6-year period 1992–1998.¶Results. Symptoms suggestive of intussusception were present in 21 (3 of whom had Henoch-Schönlein purpura and 4 had previous ileocolic intussusception reduced by air enema). Intussusception was an incidental finding in the other 29, in 28 of whom the finding was in the small bowel. Intussusception was limited to the small bowel in 43 and was ileocolic in 7. SROI was usually documented on US. Laparotomy performed in only 4 showed no evidence of intussusception or pathologic lead point. Outcome in all patients was favorable.¶Conclusions. SROI may present in symptomatic or asymptomatic children and occurs more commonly than previously reported. These intussusceptions are usually short-segment, small-bowel intussusceptions with no recognizable lead point. In asymptomatic patients, conservative observation is warranted. Intervention should be dictated by the clinical findings in symptomatic patients.

Cumulative effective doses from radiologic procedures for pediatric oncology patients.

OBJECTIVE: Our aim was to estimate the cumulative effective doses (CEDs) from radiologic procedures for a cohort of pediatric oncology patients. METHODS: A retrospective cohort study of the imaging histories of 150 pediatric oncology patients (30 each in 5 subgroups, that is, leukemia, lymphomas, brain tumors, neuroblastomas, and assorted solid tumors) for 5 years after diagnosis was performed. All procedures involving ionizing radiation were recorded, including radiography, computed tomography (CT), nuclear medicine (NM) studies, fluoroscopy, and interventional procedures. CED estimates were calculated. RESULTS: Individual CED estimates ranged from <1 mSv to 642 mSv, with a median of 61 mSv. CT and NM were the greatest contributors; CT constituted 30% of procedures but 52% of the total CED, and NM constituted 20% and 46%, respectively. There was considerable variability between tumor subgroups. CED estimates were highest in the neuroblastoma (median: 213 mSv [range: 36–489 mSv]) and lymphoma (median: 191 mSv [range: 10–642 mSv]) groups and lowest in the leukemia group (median: 5 mSv [range: 0.2–57 mSv]). CONCLUSIONS: CEDs from diagnostic and interventional imaging for pediatric oncology patients vary considerably according to diagnoses, individual clinical courses, and imaging modalities used. Increased awareness may promote strategies to reduce the radiation burden to this population.

Tube feeding and quality of life in children with severe neurological impairment

Objective: To assess the quality of life (QOL) of neurologically impaired children before and after gastrostomy (G) and gastrojejunostomy (GJ) tube insertion. Design: This was a prospective longitudinal study of children with severe neurological impairment who underwent G or GJ tube insertion. At baseline, and at 6 and 12 months after tube insertion, parents rated (1) global QOL and health-related quality of life (HRQOL) using 10 cm visual analogue scales, with 10 representing maximal QOL and (2) HR-QOL using a questionnaire-based measure. Results: Fifty patients, 45 and five of whom underwent G and GJ tube insertion, respectively, were enrolled with a median age of 591 days. Forty-two had a static neurological disorder, and eight had a progressive neurological disorder. The mean weight for age z score increased significantly over time: −2.8 at baseline and −1.8 at 12 months. The mean QOL and HR-QOL scores at baseline were 5.5 and 5.6 out of 10, respectively. There was no significant change in these scores at 6 and 12 months post-tube insertion. Children with a progressive versus a static neurological disorder had a significantly lower QOL over time. Ease of medication administration as well as feeding showed a significant improvement in scores from baseline to 12 months. Parents felt that the G and GJ tube had a positive impact on their child’s health at 6 months (86%) and 12 months (84%). Conclusion: QOL as rated by parents did not increase following insertion of a G or GJ tube in neurologically impaired children. However, parents felt that the tube had a positive impact on their child’s health, particularly with regards to feeding and administration of medications.

Practice of ALARA in the pediatric interventional suite

As interventional procedures have become progressively more sophisticated and lengthy, the potential for high patient radiation dose has increased. Staff exposure arises from patient scatter, so steps to minimize patient dose will in turn reduce operator and staff dose. The practice of ALARA in an interventional radiology (IR) suite, therefore, requires careful attention to technical detail in order to reduce patient dose. The choice of imaging modality should minimize radiation when and where possible. In this paper practical steps are outlined to reduce patient dose. Further details are included that specifically reduce operator exposure. Challenges unique to pediatric intervention are reviewed. Reference is made to experience from modern pediatric interventional suites. Given the potential for high exposures, the practice of ALARA is a team responsibility. Various measures are outlined for consideration when implementing a quality assurance (QA) program for an IR service.

Image Gently, Step Lightly: Increasing Radiation Dose Awareness in Pediatric Interventions through an International Social Marketing Campaign

In the past several decades, advances in imaging and interventional techniques have been accompanied by an increase in medical radiation dose to the public. Radiation exposure is even more important in children, who are more sensitive to radiation and have a longer lifespan during which effects may manifest. To address radiation safety in pediatric computed tomography, in 2008 the Alliance for Radiation Safety in Pediatric Imaging launched an international social marketing campaign entitled Image Gently. This article describes the next phase of the Image Gently campaign, entitled Step Lightly, which focuses on radiation safety in pediatric interventional radiology.

Image-guided percutaneous biopsy of musculoskeletal lesions in children

BackgroundPercutaneous core needle biopsy (PCNB) of musculoskeletal lesions can provide early and definitive diagnosis and guide decisions on management. The technique is less invasive than open biopsy and has a low complication rate.ObjectivesThe purpose of this study was to assess the diagnostic accuracy and safety of image-guided PCNB of musculoskeletal lesions in children.Materials and methodsRetrospective review of the medical records of patients referred for PCNB of musculoskeletal lesions was performed. Data collected included tumor type and complication rates. Lesion “hit” or “missed”, and core adequacy and ability to reach a definitive pathological diagnosis were reviewed and used to determine whether the biopsy was overall successful or unsuccessful.ResultsA total of 127 biopsies were performed in 111 patients. Of the 127 PCNB procedures, 114 “hit” the lesion and 13 “missed,” and 120 of the cores provided for analysis were deemed adequate for pathological interpretation and 7 were deemed inadequate. A definitive pathological diagnosis was possible in 97 of the 127 PCNB preocedures and not possible in 30. Overall 76% of the PCNB procedures were successful. The diagnostic success of biopsy in primary malignant tumors was significantly higher (92%) than in primary benign tumors (65%; P = 0.008). Six minor complications resulted from PCNB.ConclusionThis study showed that PCNB is accurate and safe for the diagnosis of musculoskeletal lesions in pediatric patients, and its results are comparable to those in adult studies.

Further report of small-bowel intussusceptions related to gastrojejunostomy tubes

Background. The use of gastrojejunostomy (GJ) tubes for feeding pediatric patients who have inadequate oral intake, cannot tolerate gastric feeding, or have significant gastroesophageal reflux may present problems.¶Objective. To report our experience of intussusception associated with GJ tubes, with emphasis on risk factors.¶Materials and methods. Clinical histories and imaging studies were reviewed for all patients with GJ tube-associated intussusceptions at our institution from January 1995 to March 1999.¶Results. Of 251 GJ tubes inserted, 40 (16 %) intussusceptions occurred in 30 patients (20 males). They ranged in age from 3 months to 17 years (mean 2.6 years) and in weight from 3 to 90 kg (mean 12.5 kg). The underlying diagnosis varied. The main symptom was bilious vomiting. The initial diagnosis was made by sonography in 19 cases and fluoroscopy in 21. Intussusceptions recurred eight times in patients with a distal pigtail (n = 17) but only twice in patients without the distal pigtail (n = 18).¶Conclusions. GJ-tube related intussusception is a common, easily diagnosed problem. Predisposing factors appear to be male sex, young children, and presence of a distal pigtail on the tube. Awareness of intussusception is imperative for prompt diagnosis to achieve feeding tolerance.

Image Gently, Step Lightly: Promoting Radiation Safety in Pediatric Interventional Radiology

OBJECTIVE The Image Gently, Step Lightly campaign is an education and awareness campaign focusing on radiation safety in pediatric interventional radiology. To promote radiation safety by standardizing workflow and encouraging team responsibility, the campaign Website includes a procedural checklist that the medical team may use to review radiation safety steps before each pediatric interventional procedure. CONCLUSION Use of this checklist can be an effective tool in the ongoing effort to maximize radiation safety during interventional procedures.

Efficacy and safety of radiologically placed gastrostomy tubes in paediatric haematology/oncology patients

BACKGROUND The treatment of malnutrition, which is of great concern in paediatric haematology/oncology patients, is fraught with problems. The goals of our study were to document the complications and assess the weight gain with gastrostomy tubes (G-tubes) in this population. PROCEDURE Patient data were acquired by retrospective review of all haematology, oncology, and bone marrow transplant (BMT) patients (n = 44) who received radiologically placed G-tubes at our institution over a 4-year period. RESULTS Forty-four G-tubes were placed (59% peri-BMT). At tube placement, 55% of patients were malnourished and 45% were nourished. Seventy-five percent of patients had the procedure without general anaesthetic. Localized G-tube-site infection was the most common complication (41%). Major complications occurred in 3 patients; 2 patients experienced localized peritonitis, and 1 patient developed a localized collection of pus in the abdominal wall. There were no G-tube-related deaths. At 1 month after the tube insertion, 39% of patients had gained, 54% maintained, and 7% lost weight. At 3 months, 69% had gained, 28% maintained, and 3% lost weight. There was a statistically significant weight gain from the time of the G-tube placement to both 1 month (P < 0.018) and 3 months (P < 0.0001) after G-tube placement. Patients in all diagnosis categories showed improvement from 1 to 3 months. CONCLUSIONS We conclude that retrograde tube placement is safe and can frequently be done without general anaesthetic and that G-tube feeding effectively reverses malnutrition and prevents weight loss in this patient population.