Unilateral multicystic dysplastic kidney: experience in children.

OBJECTIVES: To report a retrospective study of unilateral multicystic dysplastic kidneys (MCDK) in children, assessing the contralateral kidneys and urinary tract, the functional consequences, and the urological and nephrological management and outcome, as unilateral MCDK is the most common cause of renal cystic disease in children, and malformations of the contralateral urinary tract and kidney (pelvi-ureteric obstruction, megaureter, reflux, renal dysplasia) have been reported. PATIENTS AND METHODS: The study included 97 patients (60 boys, 37 girls) with MCDK seen between 1985 and 1998; 82 were diagnosed in utero by ultrasonography (US). After birth, the diagnosis was verified by US, renal scanning (in 93) or intravenous urography (in four), and 89 (92%) had voiding cysto-urethrography (VCUG). Of the 97 children, 87 (90% had a mean (range) follow-up of 44.3 (15-115) months. RESULTS: The MCDK was removed in 17 children; the follow-up of 75 children (five lost to follow-up) showed total involution of the MCDK in 25%, shrinkage in 60% and a stable size in 15%. None had any sign of malignancy. The contralateral kidney showed anomalies in 19 of 97 children (20%); 12 had a dilated renal pelvis (two with megaureter), six had a high echogenicity of the contralateral kidney (one had reflux, and two also pelvic dilatation). In only four of the 89 children was reflux found by VCUG; 16 of the 19 anomalies were detected by US. Five children needed surgery on the contralateral urinary tract (three a pyeloplasty, and one each a pyeloplasty plus ureteroneocystostomy, and an antireflux procedure). Of the contralateral kidneys 43% showed compensatory hypertrophy. There was mild renal insufficiency in three children; renal function seemed to be slightly impaired in many. Five infants had hypertension (four with spontaneous resolution) caused by renal scarring after pyelonephritis or inborn dysplasia of the contralateral kidney. There were symptomatic urinary tract infections in seven children. CONCLUSION: US can be used safely to diagnose unilateral MCDKs and malformations of the contralateral urinary tract and kidney. In cases where US of the dysplastic kidney remains uncertain renal scintigraphy is necessary to detect the lack of renal function. The low rate of reflux makes routine VCUG unnecessary if the contralateral upper urinary tract and kidney appear to be normal on US. Nephrectomy of the dysplastic kidney in typical cases is also unnecessary. A long-term nephro-urological follow-up of children with MCDK is recommended.

Abdominal sonography in the evaluation of heterotaxy in children.

Heterotaxy is a rare congenital disorder characterised by abnormal viscero-vascular situs with either left or right isomerism that usually coincides with complex cardiac malformation. A precise diagnosis must be made for the selection of the most appropriate treatment. The diagnosis and evaluation of these conditions have been dependent on invasive studies such as angiocardiography, although more recently magnetic resonance imaging (MRI) has been advocated. In paediatric patients MRI is far from ideal because of the need for sedation and monitoring during the examination. The authors reviewed the modalities used in the evaluation of heterotaxy with emphasis on the use of ultrasonography, including pulse and colour flow Doppler imaging. A series of 14 children with heterotaxy is presented; ten with left abdominal isomerism (polysplenia) and four with right isomerism (asplenia). Results of abdominal sonography were compared with those of echo- and angiocardiography, surgery and autopsy. Of the ten children with left abdominal isomerism, two had no cardiac abnormalities. All those with right isomerism had a complex vitium. One patient with left abdominal isomerism proved to have right atrial isomerism. Some new reproducible patterns in viscero-vascular derangement are reported. The predictable patterns of left and right isomerism are outlined and atypical features discussed.

Neuroimaging in lissencephalies.

Based on the published literature and on our own experiences in the imaging of lissencephalies with ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) we propose a strategy for the use of the different methods depending on the clinical symptoms and the age of the patient. In newborns and babies with suspected lissencephaly ultrasound should be used as the first method. If there is a cortical malformation and a more thorough examination seems necessary, CT can be used in type I lissencephaly. However, due to its excellent grey-white matter contrast MRI is the best method for imaging of lissencephalies. Especially in the diagnosis of type II lissencephaly, MRI is definitely superior to CT and US, and so it should be used in all patients with Walker-Warburg syndrome and other congenital muscular dystrophies as well as in all doubtful cases. It must always be remembered that the extent of the cortical dysplasias is quite variable, as is the presence of further malformations.

[Management of children with prenatally diagnosed urinary tract abnormalities]. / Betreuung von Kindern mit pränatal diagnostizierten Harnwegfehlbildungen.

Between 1984 and 1991, antenatal ultrasound scanning detected urinary tract malformations in 126 infants, who were investigated and treated postnatally in the childrens' hospital of the Westfälische Wilhelms-University Münster. 10 out of 126 children with urogenital changes, died in the first hours after birth, due to pulmonary hypoplasia (Potter's sequence), 1 further infant died later after cardiac operation, and another died of megacystic-megaureter-hypoperistaltic-syndrome. In the first months after birth 71 (61%) of 116 infants underwent urological surgery; 12/116 infants (10.3%) had severe bilateral kidney changes, some of them with severe deficiency of amniotic fluid before birth. 6/116 infants (5.2%) had chronic renal insufficiency, 2 of them will have to be dialyzed in early childhood and longterm, 14 patients (12%) are threatened by chronic renal failure. 14 patients (12%) developed severe arterial hypertension, all had to be treated with antihypertensive drugs, in 5 of them hypertension subsided after unilateral nephrectomy, another five had transient hypertension, but four require continued medical treatment. We describe the prenatal ultrasound findings, compared them with diagnosis after birth, illustrate diagnostics, plans of therapy, urological surgical interventions and nephrological consequences. Benefits and limitations of antenatal ultrasonography for the detection of urinary tract malformations and the treatment of those malformations before and after birth are discussed. In utero diagnosis of severe urinary tract abnormalities allows treatment of these infants immediately after birth, furthermore the prevention of severe infections, additional damage of renal tissue, and early diagnosis and treatment of arterial hypertension and metabolic imbalances caused by chronic renal insufficiency in early childhood.

Sonographic findings in infants with suspected necrotizing enterocolitis.

During a three-year period, 27 infants with clinical signs of necrotizing enterocolitis (NEC) underwent ultrasonography; 22 of these infants also had abdominal radiographs within a few hours. Sonographically, portal venous gas (PVG) was seen in 10 and intestinal pneumatosis without PVG in 8 infants. Six children underwent laparotomy within 24 hours after ultrasonography and showed evidence of recent NEC. Three other children, laparotomized weeks later were found to have intestinal strictures as signs of previous NEC. None of the patients died from NEC. Nine infants without abnormal gas distribution did not develop overt NEC. With respect to PVG, sonography is able to support the tentative diagnosis of NEC prior to radiography. However, absence of PVG does not exclude NEC.

Invasive aspergillosis complicating induction chemotherapy of childhood leukaemia.

Two children with acute leukaemia developed histologically confirmed invasive aspergillosis within 2 weeks after onset of polychemotherapy. One child had received only prednisone and one pulse of vincristine and daunorubicin before. This child showed classical roentgenographic signs of aspergilloma following an upper pulmonary lobe infiltration. The second patient developed caecal aspergillosis obscured by clinical signs of appendicitis. He died of disseminated aspergillosis several weeks later in spite of systemic antifungal therapy. Both case reports illustrate that the possibility of invasive aspergillosis must also be expected in young patients soon after onset of induction chemotherapy.

[Arterial hypertension. Prevention in infants with congenital urinary tract malformations]. / Arterielle Hypertonie. Vorkommen bei Säuglingen mit angeborener Fehlbildung der ableitenden Harnwege.

Eighty-nine newborns and infants with congenital urinary tract malformations were treated in the childrens' hospital of the Westfälische Wilhelms-University from 1986 to 1989. Twenty patients of this group (22.5%) developed severe hypertension requiring treatment within the first year of life. Mean age of diagnosis of hypertension was 5 months (range 0.5-12 months). Median values for blood pressure at time of diagnosis were 138 mmHg (range 120-170) for systolic and 92 mmHg (range 80-110) for diastolic values. Six patients showed characteristic symptoms for hypertension such as restlessness, sweating and sleep disorders. Plasma levels of renin were obtained in 12 of 20 patients. Five patients had raised plasma renin levels. All patients with a severe hypertension were treated with one to several antihypertensive drugs. Risk factors for the development of severe renal hypertension in early infancy are cystic renal malformation, vesico-ureteral reflux, obstructive uropathy and to our experience also short term percutaneous nephrostomy in obstructive uropathy in particular in connection with pyelonephritis. Hypertension can still appear after the successful surgical correction of urinary obstruction. We describe the group of patients with severe hypertension in our study group; diagnostic principles and our therapeutic approach are explained. We conclude that early diagnosis of severe hypertension and consecutive treatment are important in infants with congenital urinary malformations.

Intrahepatic and portal venous gas detected by ultrasonography.

During a 3-year period, sonographic evidence of portal venous gas (PVG) was found in 11 patients. Of these, 10 patients were examined for clinically suspected necrotizing enterocolitis (NEC). In the 11th patient, suffering from nephroblastoma, PVG was detected by routine sonography. Radiographic examination, performed in nine of 11 patients did not show any PVG. Intestinal pneumatosis was radiographically identifiable in only four of these children, whereas eight of 11 patients with sonographically detectable PVG also had sonographic evidence of intramural gas. Follow-up examinations in five patients showed cessation of PVG soon after onset of adequate therapy, indicating that ultrasonography is also a reliable method for monitoring NEC. Sonographic evidence of PVG, however, may be limited to the time before onset of therapy.

Intestinal ultrasonographic findings in Schönlein-Henoch syndrome.

Thirteen children with Schönlein-Henoch syndrome were evaluated for ultrasonographic changes of the gastro-intestinal tract. One patient was examined twice because of a relapse 1 year later. Out of 14 sonographic scans 10 showed abnormal findings, consisting of circumscribed hypo-echoic asymmetrical enlargement of the intestinal wall, the sonographic result of intramural effusions. One patient also had ileocoecal intussusception. Our results suggest that sonography of the intestine is useful to detect changes of the intestinal wall in Schönlein-Henoch syndrome.

Adult height in boys and girls with untreated short stature and constitutional delay of growth and puberty: accuracy of five different methods of height prediction.

To determine how accurately several methods of height prediction estimate adult height, we compared height predictions calculated by the Bayley-Pinneau, Roche-Wainer-Thissen (RWT), target height, and Tanner-Whitehouse Mark I (TW-MI), and Mark II (TW-MII) methods with final adult height in 37 boys and 32 girls with short stature and constitutional delay of growth and puberty. They were first seen at a chronologic age (mean +/- SD) of 14.80 +/- 1.70 years (boys) and 12.87 +/- 2.56 years (girls). Adult height at 23.14 +/- 1.95 years and 21.05 +/- 2.02 years was 170.4 +/- 5.4 cm (boys) and 157.8 +/- 4.2 cm (girls), respectively, and thus within the lower range of normal. Height predictions were calculated for the total group and for patients with parents of normal (group 1) as well as short stature (group 2). For boys, the RWT method gave very accurate results, underestimating adult height by -0.6 cm for the total group. The prediction errors for the other methods were -7.3 cm (TW-MI), -4.2 cm (TW-MII), and +3.1 cm (Bayley-Pinneau method) or +1.7 cm (target height). For girls, no method was superior in estimating adult height. The mean prediction error was -0.8 cm, -2.1 cm, and -1.8 cm with the Bayley-Pinneau, TW-MI, and TW-MII methods, respectively. In contrast, adult height was overpredicted by +2.3 cm and +1.2 cm with the RWT and target height methods. We conclude that patients with short stature and constitutional delay of growth and puberty reach an adult height in the lower range of normal. Height prediction methods differ with respect to their accuracy and their tendency to overestimate or underestimate adult height.

[Changes in the lungs in mucoviscidosis. Feasibility and advantages of different imaging techniques]. / Lungenveränderungen bei der Mukoviszidose. Möglichkeiten und Nutzen der verschiedenen bildgebenden Verfahren.

Pulmonary abnormalities in cystic fibrosis result from the obstruction of small bronchi by highly viscous mucus. Chronic obstructive lung disease and recurrent pulmonary infections result in a typical radiographic pattern later in the disease. Most patients can now be expected to survive into adulthood. The radiologist must make a careful comparison of serial films in order to detect complications early. By far the most important imaging modality is the conventional chest radiograph. CT is more sensitive for detection of structural abnormalities of the lung. Bronchography is a dangerous procedure and can lead to rapid deterioration of lung function. Lung scanning is a very sensitive method for demonstrating regional disturbances of ventilation and may reveal abnormalities earlier than conventional radiographs. In severe hemoptysis, selective bronchial arteriography with embolization of the bleeding vessel can be a life-saving procedure.

[Tuberculous spondylitis following BCG vaccination]. / Tuberkulöse Spondylitis nach BCG-Impfung.

A case of a rare form of BCG osteomyelitis in the spine is presented. After vaccination, the disease started with a lymphadenitis. Later an abscess extended from the pelvic along the psoas muscles into the retroperitoneum. The soft tissue mass extended paraspinally and epidural involvement was also apparent. The vertebral involvement was detected by CT. The radiological findings are discussed with reference to the literature.