Persistent mediastinal mass is not indicative of recurrence after chemotherapy only in paediatric Hodgkin's disease.

Most patients with Hodgkin's disease are treated with chemotherapy in conjunction with radiotherapy, but at the end of treatment a residual mass is often present. After combined therapy, it has been assumed that no additional treatment is needed. However, for children treated without radiotherapy, no data exist on the relevance of a residual mediastinal mass to risk of relapse. We report on the findings of follow-up thorax radiographs of a group of 27 children with initial mediastinal involvement, who were treated with chemotherapy only. We conclude that the regression rate of the mediastinal mass was not related to a later recurrence. Regression after chemotherapy without radiotherapy is probably slower than after combined therapy. We consider chest radiograph examinations to be appropriate for the follow-up of tumour regression. When the data were compared with a group of children with Hodgkin's disease without mediastinal involvement, we found that survival was not related to initial mediastinal involvement.

Megarectum in constipation.

BACKGROUND: Faecal impaction is frequently observed in children with chronic constipation. The term megarectum is often used to describe this finding. AIM: To evaluate rectal functioning and rectal measures in constipated children with a filled rectum, in order to define the terms faecal impaction, enlarged rectum, and megarectum. METHODS: All children underwent radiological investigation, colonic transit time study, anorectal manometry, and rectal volume and rectal wall compliance measurements. Patients with faecal impaction were compared with controls, who had an empty rectum on digital rectal examination. RESULTS: A total of 31 patients and six controls were included in the study. The mean duration of complaints was 4.2 years and all had faecal incontinence. The colonic transit times in the patients showed a distinct delay in the rectosigmoid segment. Anorectal manometry was not significantly different between patients and controls. The rectal width in patients was 0.68 and in controls 0.52 with an upper limit of 0.61. The pressure-volume curve in patients showed significant less relaxation at a distension of 50 ml. The slope of the curve (corresponding with rectal wall compliance) was comparable for patients and controls. CONCLUSIONS: We suggest that faecal impaction is a filled rectum found on digital rectal examination; an enlarged rectum is defined by a rectopelvic ratio greater than 0.61; and megarectum is defined in those with significant abnormalities found with anorectal manometry, pressure-volume curves, or rectal compliance investigation. A diminished relaxation of the rectum on rectal distension could be the first sign of megarectum in children with chronic constipation.

Is partial nephrectomy appropriate treatment for unilateral Wilms' tumor?

PURPOSE: After nephrectomy for unilateral Wilms' tumor (WT), two potential hazards threaten the remaining kidney. The development of a metachronous WT (2% to 3%), and decrease in renal function after chemotherapy. Our study aims included: (1) to clarify how many WT patients could benefit from partial nephrectomy (PN) without jeopardizing the high cure rate, (2) to establish the function of the kidney remnant, and (3) to test the radiological criteria for preoperative selection of PN candidates. METHODS: A retrospective analysis of 90 consecutive cases of histologically verified WT (1982 to 1992) was carried out. Data were studied independently by the surgeon, the radiologist, and the pathologist. The preoperative selection criteria for PN were functioning kidney, tumor confined to upper or lower pole leaving two thirds of the kidney tumor free, and no hilar or vascular structures involved. After PN, renal function was estimated by a calculated creatinine clearance (CC) combined with the assessment of the relative function of each kidney by renal scintigraphy. RESULTS: There were 46 boys and 44 girls with median age at diagnosis of 3 years 6 months (range, 3 months to 16 years 7 months). Stage I disease occurred in 40 patients, stage II in 23, stage III in 16, and stage IV occurred in 11 patients. Nearly all patients received preoperative chemotherapy. Radiological analysis of 85 available data sets (5 missing) suggested that PN was possible in 13 patients, and data were inconclusive in two. All 13 patients selected by the radiologist appeared to be in agreement with the pathological criteria for resectability. Of these cases, complete resection by PN was performed in five, could have been possible in retrospect in two, and appeared impossible in six. In two more patients, PN was performed; in both cases the radiologist had predicted PN to be impossible. Median follow-up after PN was 61 months, range, 45 to 167 months. Two patients died of distant metastasis, and no local recurrence occurred. Five are in remission with a median CC level of 110 mL/min, range, 85 to 124. Relative functions of the kidney remnants were 20% to 50%. Contralateral nephrectomy for progressive obstruction was performed in one, but CC level remained normal (85 mL/min). Comparing the preoperative radiological prediction with the combined surgical and pathological findings in a 2 x 2 frequency table, the sensitivity of the radiological findings could be calculated to be 80%, the specificity 97%, and the accuracy 87%. CONCLUSIONS: (1) Partial nephrectomy is safe in approximately 10% of Wilms' tumor patients. (2) The function of the kidney remnant remains well, and even permits contralateral nephrectomy. (3) Preoperative imaging shows an accuracy of 87% to predict the possibility for partial nephrectomy.

A new diagnostic approach to vascular rings and pulmonary slings: the role of MRI.

UNLABELLED: The conventional diagnostic work-up of a patient suspected of having a vascular cause for stridor, or dysphagia, includes esophagography and bronchoscopy to delineate the abnormal structure without imaging the structure itself. Cine-angiography is regarded as the golden standard, but is not routinely performed. Magnetic resonance imaging (MRI) is non-invasive and has the important advantage over cine-angiography of depicting all structures in the field of view. Color Doppler echocardiography depicts the great vessels, but not the esophagus and trachea. In 14 patients with obstructive symptoms and in four patients without obstructive symptoms MRI successfully imaged the abnormal structure, as was the case in two symptomatic patients using computer tomography. In this series, the findings were confirmed at surgery or by cine-angiography. CONCLUSION: we suggest that in patients suspected of having a vascular cause for stridor or dysphagia, MRI should be performed. If there is need for a screening procedure, color Doppler echocardiography should be used and if that is equivocal or non-conclusive, esophagography and bronchoscopy should be used. If MRI is difficult to interpret, it should be augmented by magnetic resonance angiography before considering cine-angiography.

Diagnostic imaging in abdominal neuroblastoma: is there a complementary role of MIBG-scintigraphy and ultrasonography?

UNLABELLED: In a retrospective study we evaluated the agreement between the results of meta-iodo benzylguanidine (MIBG) scintigraphy and abdominal ultrasonography (US) in the diagnosis and follow up of neuroblastoma (NBL) with respect to the abdominal region. Data of 28 consecutive paediatric patients with NBL or suspected NBL were included (16 M/12 F, mean age 2.9 years, range 3 weeks-13.4 years). The results (as judged by the nuclear physician or radiologist, respectively) of 60 MIBG examinations (123I and 131I) including 26 single photon emission computed tomography (SPECT) and US, respectively, performed within a period of 14 days, could be evaluated. Full agreement was reached in 37 comparisons (62%), while partial and no agreement was found in 17 (28%), and 6 (10%) comparisons respectively. In 8 out of 37 comparisons with full agreement, 12 diagnosed lesions were histopathologically proven, while 11 comparisons with negative findings were also negative in other clinical modalities. US diagnosed correctly in 68% of the histopathological proven lesions, while this was 54% for MIBG scintigraphy. In approximately 50% of the MIBG scans in which SPECT was available, SPECT provided significant additional information. CONCLUSION: Congruent results of MIBG scintigraphy and ultrasonography in the detection of abdominal lesions in patients with suspected neuroblastoma indicate a high reliability in the diagnosis and localisation. Due to the favourable results of additional SPECT, it is advisable to perform SPECT routinely in this diagnosis.

Ewing's sarcoma of the pelvis: changes over 25 years in treatment and results.

The pelvic localisations of Ewing's sarcoma have the worst prognosis due to large size at diagnosis, frequent distant metastases, radiosensitive organs next to the tumour and difficult surgery. The purpose of the present study was to analyse treatment results over a period of 25 years and to investigate the impact of newer chemotherapy schedules, improved radiotherapy techniques and newer surgical methods on the prognosis. 35 children and young adults were identified from 1967 to 1994 for whom diagnosis, presentation, performed treatment and outcome were available. Tumour size, as measured from CT scans, response to chemotherapy and radiotherapy target volume, could be reviewed in the later years. Actuarial 5-year survival for the whole group was 31% and for the 24 non-metastatic patients 40%, with a disease-free interval of 19%. Tumour size could be measured in 27 patients and ranged from 36 to 1540 cm3. There were 12 local recurrences, 1 in the 4 patients treated with surgery. After 1983, 9 out of 17 irradiated patients developed local failure. 3 patients had adequate fields and one a close field which did not cover completely the prechemotherapy extent and 3 of these recurred. All 4 patients with stable disease after neoadjuvant CT failed locally, not withstanding high-dose radiotherapy. The mean length of neoadjuvant CT tended to be shorter in patients without local relapse. There was no significant difference in survival before and after 1983.

Variant rhizomelic chondrodysplasia punctata (RCDP) with normal plasma phytanic acid: clinico-biochemical delineation of a subtype and complementation studies.

Rhizomelic chondrodysplasia calcificans punctata (RCDP) is an autosomal recessive peroxisomal disorder which affects phytanic acid oxidation and de novo biosynthesis of plasmalogens in liver and fibroblasts. Peroxisomal thiolase is present in its unprocessed precursor form (44 kDa). We studied a mentally retarded 9-year-old girl with cataracts and atypical bone dysplasia. Neurological findings were mild compared to classic RCDP. Plasma phytanic acid was normal. Results of de novo plasmalogen synthesis and phytanic acid oxidation studied in cultured skin fibroblasts were intermediate between normal controls and classic RCDP. Peroxisomal thiolase was present only as the unprocessed 44 kDa protein. Taken together these results suggest that we are dealing with a variant form of RCDP with clinical and biochemical abnormalities much milder as compared to classic RCDP. In order to establish the genetic relationship between our patient and classic RCDP patients complementation studies were carried out. Earlier studies had already shown that fibroblasts from all RCDP patients studied belong to a single complementation group. Fibroblasts from our patient could also be assigned to this complementation group suggesting that the phenotypic variability results from different mutations within the same gene.

Defaecation disorders in children, colonic transit time versus the Barr-score.

It is still unclear how to evaluate the existence of faecal retention or impaction in children with defaecation disorders. To objectivate the presence and degree of constipation we measured segmental and total colonic transit times (CTT) using radio-opaque markers in 211 constipated children. On clinical grounds, patients (median age 8 years (5-14 years)) could be divided into three groups; constipation, isolated encopresis/soiling and recurrent abdominal pain. Barr-scores, a method for assessment of stool retention using plain abdominal radiographs, were obtained in the first 101 patients, for comparison with CTT measurements as to the clinical outcome. Of the children with constipation, 48% showed significantly prolonged total and segmental CTT. Surprisingly, 91% and 91%, respectively, of the encopresis/soiling and recurrent abdominal pain children had a total CTT within normal limits, suggesting that no motility disorder was present. Prolonged CTT through all segments, known as colonic inertia, was found in the constipation group only. Based on significant differences in clinical presentation, CTT and colonic transit patterns, encopresis/soiling children formed a separate entity among children with defaecation disorders, compared to children with constipation. Recurrent abdominal pain in children was in the great majority, not related to constipation. Barr-scores were poorly reproducible, with low inter- and intra-observer reliability. This is the first study which shows that clinical differences in constipated children are associated with different colonic transit patterns. The usefulness of CTT measurements lies in the objectivation of complaints and the discrimination of certain transit patterns. Conclusion. Abdominal radiographs, even when assessed with the Barr-score proved unreliable in diagnosing constipation.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypertrophic osteoarthropathy in childhood malignancy.

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones. Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coli. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis. A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease. Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neoplastic disease involving the chest, HOA may precede pulmonary symptoms by 1-18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions. The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA.

Ultrasound diagnosis of gastroesophageal reflux and hiatal hernia in infants and young children.

In 30 young children suspected of gastroesophageal reflux (GER), the G-E junction was examined with ultrasonography directly after a feeding while these children were on overnight extended esophageal pH monitoring (EEpHM) (32 simultaneous ultrasound/EEpHM studies). The two tests showed 81% to 84% agreement in the detection of the presence or absence of GER, depending on whether the whole period of EEpHM or only the part of it covering the ultrasound observation period were used as the standard. The discrepancies between the two tests were explained by the much longer monitoring period of EEpHM compared to ultrasonography and the inability of EEpHM to show reflux of neutralized gastric contents directly after milk feedings. The two studies probably measure different aspects of clinically significant reflux and must be correlated with the clinical symptoms. Morphological findings associated with significant reflux were (1) a short intra-abdominal part of the esophagus, (2) a rounded gastroesophageal angle, and (3) a "beak" at the gastroesophageal junction. Barium meal findings confirmed these sonographic signs, indicating a sliding hiatal hernia of the distal esophagus, either fixed or intermittent. Ultrasonography can be recommended as a useful and physiological screening test to demonstrate clinically significant GER and a predisposing hiatal hernia of the esophagus in symptomatic children.

Hypertrophic pyloric stenosis: use of the pyloric volume measurement in early US diagnosis.

The diagnosis of hypertrophic pyloric stenosis (HPS) with ultrasonography (US) is dependent on measurements of pyloric diameter (PD), pyloric length (PL), and muscle thickness. The authors were unable to confidently diagnose the condition with US in 45% of patients who underwent surgery for HPS because all three criteria were not fulfilled. An overall measurement of the "amount" of pyloric hypertrophy was introduced: pyloric volume (PV), which was equated to 1/4 pi x PD2 x PL. No overlap was found between patients with HPS (n = 22; PV = 3.13 mL; range, 1.4-5.1 mL) and asymptomatic control subjects (n = 28; PV = 0.65 mL; range, 0.2-1.3 mL) or symptomatic subjects without HPS (n = 25; PV = 0.86 mL; range, 0.4-1.3 mL). A positive correlation was found between age at diagnosis and PV, a finding reflecting that HPS is an acquired condition. In patients less than 4 weeks of age, the criterion of PV greater than or equal to 1.4 mL proved to aid in the identification of early HPS more accurately than any existing criteria.

[Intussusception. The clinical picture and a retrospective study in 34 patients at the Emma Pediatric Hospital]. / Invaginatie. Het ziektebeeld en een retrospectief onderzoek bij 34 patiënten in het Emma Kinderziekenhuis.

Intussusception is a serious condition. It is necessary to establish the diagnosis as soon as possible. This is of importance for the kind of therapy. We confirmed this statement by studying retrospectively 34 patients in the Emma Kinderziekenhuis in Amsterdam. There are two well known forms of therapy: hydrostatic reduction and surgery. Besides we like to pay attention to the method of air-pressure enema reduction which has been used in China for over 23 years.

Hypertrophic osteoarthropathy in childhood malignancy.

A case of hypertrophic osteoarthropathy associated with carcinoma of the nasopharynx in a child is presented. Peculiar findings were the minor changes on the chest film, and the absence of periosteal reactions in the radii.

[Type I tricho-rhino-phalangeal syndrome]. / Het tricho-rhino-falangeaal syndroom, type I.

A two-year-old boy and his mother with the Tricho-Rhino-Phalangeal syndrome type I were studied. The mother showed the complete triad of the syndrome with fine, sparse, slow-growing hair, pear-shaped nose and abnormal fingers. Cone-shaped epiphyses of the phalanges of the hands and shortening of the mesophalanges and of the first and fifth metacarpals were observed radiographically. With the exception of the extreme shortening of several phalanges and metacarpals the above mentioned symptoms were also present in the son.

Mucolipidosis I. Roentgenographic follow-up.

A patient with mucolipidosis I (ML I) is presented. The roentgenographic findings in the skull, hands, ribs, vertebral column, pelvis, and tubular bones are described. Special emphasis is laid on the evaluation of the skeletal alterations during a 13-years follow-up. The similarities to and the differences from the so-called dysostosis multiplex (DM) are outlined. Some peculiarities which may be specific to ML I are discussed. Attention is given to an exceptional feature in our case of this very rare condition, namely, the marked thickening which developed on the frontal portions of the base of the skull, including the sellar region.