Congenital chylothorax: Current evidence-based prenatal and post-natal diagnosis and management.

Congenital chylothorax is an uncommon condition but represents the main cause of congenital pleural effusion during the neonatal period. It usually appears before birth, both as an isolated disorder or in association with hydrops fetalis, negatively affecting the subsequent neonatal outcome. Prenatal treatment is usually considered to ensure a satisfactory lung development in case of moderate to severe pleural effusion or in the presence of hydrops, although consensus on treatment timing and modalities has not been reached to date. Both medical and surgical therapeutic strategies are available to treat this condition and novel treatment options have been recently attempted with acceptable results in both prenatal and post-natal setting. The heterogeneous clinical presentation of congenital chylothorax together with its rarity, its numerous etiologies and the absence of a highly effective treatment renders the diagnostic and therapeutic approach difficult to standardize. In addition, adequate visualization of the lymphatic system is complex, especially in small neonates, although new promising techniques have been developed lately and may contribute to improved management of this serious but infrequent condition. This review focuses on the current evidence base for the diagnosis and treatment options for congenital chylothorax, suggesting a rational diagnostic and therapeutic approach both in the prenatal and in the neonatal period.

Surgical validation of functional magnetic resonance urography in the study of ureteropelvic junction obstruction in a pediatric cohort.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is one of the most common urological diseases in children. The etiology can be intrinsic, extrinsic (crossing vessel [CV] or adhesions), or mixed. To date, ultrasonography and scintigraphy are considered gold-standard imaging techniques for the study of UPJO. Functional magnetic resonance urography (fMRU) combines anatomical and functional information and has been recently evaluated for the detection of CVs in UPJO. OBJECTIVE: The objective of the study was to evaluate the concordance between fMRU and surgery in determining the etiology of UPJO and the presence of obstructing/non-obstructing CVs. STUDY DESIGN: Patients with unilateral hydronephrosis who underwent surgery after an fMRU were included in the sample. Surgical data regarding the etiology of UPJO were compared with radiological results. The etiology was divided into intrinsic, extrinsic due to CV, extrinsic due to adhesions, and mixed or cicatricial (postoperative). The concordance was calculated by means of the Cohen's kappa coefficient. RESULTS: The observed agreement between fMRU and surgical findings regarding the etiology and the presence of CV were 83.2% and 89.4%, respectively (with substantial Cohen's kappa coefficient). The sensitivity and specificity of fMRU were 0.84 and 0.93, respectively; the positive predictive value (PPV) and negative predictive value (NPV) were 0.889 and 0.897, respectively. The observed agreement regarding the type of vessel was 88.3% with a Cohen's kappa coefficient of 0.787 (substantial). DISCUSSION: In children with hydronephrosis, it is very important for the surgeon to quantify the extent of dilation, define the etiology of the obstruction, and the presence or absence of CVs. fMRU is a 'one-stop-shop' technique which provides both anatomical and functional information showing a high concordance with surgical findings, avoiding radiation exposure. CONCLUSIONS: fMRU should be considered a valid imaging technique in the study of pediatric UPJO, as it provides the surgeon with important information regarding the etiology of the obstruction for the preoperative planning.

MRI versus conventional measures of disease activity and structural damage in evaluating treatment efficacy in juvenile idiopathic arthritis.

OBJECTIVE: To compare the American College of Rheumatology paediatric (ACRp) response criteria and conventional radiography with MRI findings in a cohort of patients with juvenile idiopathic arthritis. METHODS: Forty consecutive patients (30 girls, 10 boys; median age 10.8 years) with arthritis of the wrist starting treatment with disease-modifying antirheumatic drugs or biological agents were recruited. At 1-year follow-up the treatment response was assessed by ACRp criteria and radiographic progression using the adapted Sharp/van der Heijde method. Wrist MRIs were evaluated using both the paediatric-MRI and the OMERACT rheumatoid arthritis MRI scores. Sensitivity to change of clinical and imaging variables was assessed by standardised response mean (SRM) and relative efficiency (RE) was used to compare SRMs. RESULTS: ACRp90 responders showed a significantly higher decrease in MRI synovitis score (median change -4) than non-responders (median change 0), ACRp30-50 responders (median change 0) and ACRp70 responders (median change -1) (p=0.0006, Kruskal-Wallis test). Non-responders showed significantly higher radiographic progression than ACRp90 responders (pB=0.016). The MRI synovitis score showed a greater responsiveness to change (SRM 1.69) compared with the majority of ACR core set of variables. MRI erosion scores were less responsive than conventional radiography in detecting destructive changes (RE <1). MRI follow-up revealed no signs of inflammation in four out of 24 wrists with clinically inactive disease. CONCLUSION: Only ACRp90 responders showed a significant decrease in synovitis and the halting of structural damage, suggesting that levels of response higher than ACRp30 are more appropriate for assessing drug efficacy. The excellent responsiveness of MRI and its ability to detect subclinical synovitis make it a promising outcome measure.

Novel automated system for magnetic resonance imaging quantification of the inflamed synovial membrane volume in patients with juvenile idiopathic arthritis.

OBJECTIVE: To introduce a novel automated method for the quantification of the inflamed synovial membrane volume (SV) using magnetic resonance imaging (MRI), and to investigate its feasibility and validity in patients with juvenile idiopathic arthritis (JIA). METHODS: The tool was tested on 58 patients with JIA and wrist involvement. Thirty-six patients had a 1-year MRI followup. MRI of the clinically more affected wrist was performed using a 1.5T scanner and a Flex small coil. An algorithmic approach, based on supervised voxel classification for automatic estimation of SV in a 3-dimensional MRI, was developed. The SV was estimated as the number of positively classified voxels and then normalized by the patient's body surface (NSV). Validation procedures included the analysis of reliability, construct validity, responsiveness to change, discriminant validity, and the predictive value. RESULTS: The agreement between the automated estimation of NSV and the manual measurements was excellent (intraclass correlation coefficient 0.93, 95% confidence interval 0.79-0.98). The automatic NSV demonstrated good construct validity by yielding strong correlations with local signs of disease activity and a moderate correlation with global physician assessment of disease activity and with the Rheumatoid Arthritis Magnetic Resonance Imaging Scoring system synovitis score. NSV showed a strong responsiveness to clinical change (standardized response mean values >1) and satisfactory discriminant validity. High baseline NSV (>4.6) had high predictive value (100%) with respect to erosive progression. CONCLUSION: The proposed automated method allowed reliable quantification of NSV, which represents a promising imaging biomarker of disease activity in JIA. The automated system has the potential to improve the longitudinal assessment of JIA and to predict progressive joint destruction.

Traumatic lesions of adrenal glands in paediatrics: about three cases.

Pediatric adrenal injuries, in blunt thoracoabdominal trauma, are rare and usually associated with traumatic liver and kidney lesions. This paper aims to present imaging findings and possible adrenal involvement in blunt abdominal traumas in childhood. We report three cases of thoracoabdominal trauma with adrenal involvement. Two patients were polytraumatised in car accidents. The third case was unusual because of the mild trauma. The adrenals lesions were right-sided in all cases. Post-traumatic adrenal contusion/haematoma may arise not only because of a direct trauma but also as a consequence of a sudden increase in the pressure in the inferior vena cava system-adrenal veins. This is why adrenal haemorrhage is not directly proportional to the trauma: compression of the inferior vena cava leads to increased pressure in the adrenal venous circulation, which supports the parenchymal lesion. The right adrenal gland is more frequently injured than the left gland: it can be easily compressed between the liver, spine and kidney, and its venous drainage flows directly into the inferior vena cava.

Pancreatitis caused by duodenal duplication.

Two cases of recurrent pancreatitis, due to duodenal duplication, are reported. The aim of this paper is to emphasise the role of endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC) in the detection of associated pancreaticobiliary anomalies and in the planning of the correct surgical approach. The order of imaging in a child with recurrent pancreatitis should be US, barium meal and PTC. ERCP is often difficult to perform in children.

Diagnostic imaging of the early slipped capital femoral epiphysis.

INTRODUCTION: Early slipped capital femoral epiphysis (ESCFE) can be treated surgically, with excellent results, if it is diagnosed in its initial stage; however, the rate of late or missed diagnoses remains surprisingly high. PURPOSE: We compared radiography, US and MR sensitivity in ESCFE diagnosis. MATERIAL AND METHODS: We examined 21 symptomatic overweight patients (15 boys and 6 girls) aged 9 to 15 years with anteroposterior radiographs; frog leg images were not acquired in 3 cases only. US was performed in 19 cases and the images acquired with 5-7.5 MHz probes on the sagittal plane parallel to the femoral neck. MRI was performed in 9 cases, with coronal and sagittal T1 SE and T2* GE images. RESULTS: Our sensitivity rates were 66% for anteroposterior radiography (6 false negatives), 80% for combined anteroposterior and frog leg images (3 false negatives), 95% for US (1 false negative) and 88% for MRI (1 false negative). DISCUSSION AND CONCLUSION: We believe that US is the method of choice in ESCFE diagnosis; if it is negative, but pain persists, MRI should be performed.

High-resolution US of non-traumatic recurrent dislocation of the peroneal tendons: a case report.

We report the case of a young girl with recurrent sharp pain in the outer aspect of the ankle. She had no previous trauma. High-resolution US (HRUS) showed a complete intermittent dislocation of the peroneal tendons. Post-traumatic chronic peroneal instability is quite common, whereas complete dislocation is rare. HRUS is an important adjunct to clinical examination and radiographic evaluation of patients with musculoskeletal disorders of the foot and ankle [1]. This paper reports its value in non-traumatic recurrent dislocation of the peroneal tendons.

[Diagnostic imaging of chronic inflammatory diseases of the intestine in children]. / Diagnostica per immagini nelle malattie infiammatorie croniche dell'intestino nell'età pediatrica.

Chronic inflammatory bowel disease (CIBD) in children is represented mainly by ulcerative colitis (UC) and Crohn's disease (CD). Infectious forms, Behçet's disease and eosinophilic gastroenteritis are not included in this study. UC and CD are difficult to diagnose and to treat, and require a multispecialistic approach including pediatric gastroenterologists, surgeons, radiologists, endoscopists and pathologists. Progress in diagnostic techniques--i.e., nuclear medicine procedures, conventional, duplex and color-Doppler US, and endoscopy--has increased our knowledge of these diseases and it has made their diagnosis easier, opening new therapeutic perspectives. In our paper, after mentioning the major clinical and radiologic features of CIBD, we report on the patients hospitalized in the Gaslini Institute from 1984 to 1994. Thirty-six patients (age range: 3,9 to 14 years) were examined with several imaging techniques--i.e., plain radiography, US, contrast studies and CT--according to the clinical presentation and behavior of the disease. X-ray studies played the leading role in imaging mucosal disease spread (double contrast studies) and monitoring "surgical" complications (plain radiography). These techniques, which were always combined with US and, in some cases, CT, are also a major tool to study extraluminal CIBD spread--i.e., fistulas, pseudotumors, lymph nodes, abscesses and mesenteric changes. Color-Doppler US may play a critical role in the differential diagnosis of these and other small and large bowel pathologic conditions. The most typical CD patterns in bowel disease are listed in the paper, to give a contribution to the differential diagnosis of the chronic abdominal pain syndrome which is a very common condition in children.

[Role of echo-Doppler in the screening of renal artery stenosis in children with kidney transplants]. / Ruolo dell'eco-Doppler nello screening della stenosi dell'arteria renale nei reni trapiantati nell'età pediatrica.

Renal artery stenosis (RAS) in renal transplanted pediatric patients is a long-term complication. The clinical suspicion must be considered when patients exhibit signs of impaired renal function or refractory hypertension, not associated with other complications of renal transplantation -i.e., acute or chronic rejection, glomerulonephritis, cyclosporine toxicity. The intermediate step between clinical suspicion and angiography is represented by Doppler US. The authors report their experience with Doppler US in the screening of RAS in a pediatric series of transplanted patients. The incidence of RAS in our series (54 transplanted kidneys, 46 of them included in the study) was 4.3%. A severe stenosis was demonstrated by both Doppler US and angiography in 2 patients, with 100% Doppler sensitivity. In both stenoses, Doppler US showed high systolic peaks (blood flow velocity > or = 2.5 m/s) and post-stenotic turbulence. Thanks to its high sensitivity, Doppler US is considered to be very useful in the screening of vascular complications in renal transplanted children.

[Computed tomography vs. magnetic resonance in the diagnosis of anorectal anomalies]. / Tomografia Computerizzata vs Risonanza Magnetica nella diagnostica delle anomalie ano-rettali.

Anorectal malformations include a variety of anatomical anomalies which are often difficult to assess. Thirty-four patients (age range: 1 month-19 years) were studied over a 4-year period, 14 of them preoperatively and 20 postoperatively. In addition to inverted lateral plain films, a barred Iopamiro enema was performed in 13 of 14 cases by a Foley's catheter via colostomy. Endocolic pressure could thus be increased, which allowed the detection of rectal fistulas in 11 of 13 cases. Preoperative CT was performed in 9 cases, and MRI in 5. Both CT and axial MR scans provided accurate images of sphincteric muscles: they were well developed in 7 patients and poorly developed in 6; in 1 case the muscles were not identified. Sagittal MR scans only demonstrated the distance between blind pouch and perineum. Sagittal MRI also provided images of the spine (1 patient exhibited tethered cord). In the postoperative management, except for severe sacral anomalies, miscentered puborectal muscle and hypoplastic musculature were observed as the most frequent causes of fecal incontinence in patients treated with abdomino-perineal pull-through of the colon. To select the cases who needed reoperation, most frequently secondary PSARP, 17 patients were studied by CT and 7 by MRI; both techniques were employed in 4 cases. In 6 patients good development of sphincteric musculature and neo-anorectum correctly positioned within were observed. In 4 cases the neo-anorectum was correctly positioned, but sphincteric muscles were too hypoplastic to allow good continence; in 10 patients with incontinence, the neo-anorectum was seen outside sphincteric musculature: in 2 cases the latter was well developed (1 patient had had severe surgical damage to levator ani), while in 8 it appeared to be poorly developed. In our experience, postoperative CT allowed a correct evaluation to be made, whereas MRI failed to yield additional information.

Holoprosencephaly: prenatal diagnosis by sonography and magnetic resonance imaging.

Magnetic resonance (MR) imaging was performed on two women at the 33rd and 34th pregnancy week, respectively, after ultrasonographic detection of a brain malformation. Fetal neuromuscular blockade was induced by pancuronium bromide injected into the umbilical vein under continuous ultrasound (US) guidance. MR images supported the echotomographic diagnosis of holoprosencephaly, improving the image quality and offering additional information in such cases of difficult differential fetal diagnosis. Holoprosencephaly was finally confirmed by neonatal US and autopsy (case 1), US, CT and MR (case 2).

[The relationship between ligation of the ductus arteriosus and intracranial hemorrhage in preterm infants]. / Relazione tra legatura del dotto arterioso ed emorragia intracranica nel pretermine.

Surgical closure of patent ductus arteriosus (PDA) and perioperative time have been proposed as conditions of increased risk of peri-intraventricular hemorrhage (PVH-IVH) in preterm infants. We examined by pre- and postoperative ultrasound (US) scan 15 low birth-weight neonates who underwent PDA ligation within the first two weeks of life. They were assessed with regard to clinical state, perioperative management and development of PVH-IVH. Fourteen did not show onset or extension of intracranial hemorrhage in the immediate postoperative period. One patient developed a wide III grade IVH in the 24 perioperative hours. His conditions were particularly severe, suggesting that many factors such as acidosis, hypoxia, hypercapnia and hypotension might have combined to lead to hemorrhage. We conclude that PDA ligation is not likely to increase the risk of PVH-IVH per se.

[Ultrasonic diagnosis of cerebellar hemorrhage in newborn infants. Apropos of 2 cases]. / Diagnosi ecotomografica di emorragia cerebellare nel neonato. A proposito di 2 casi.

The sonographic diagnosis of cerebral hemorrhage, in preterm infants is quite easy. On the contrary the prematurity degree of the patient and the anatomic location of the intracerebellar hemorrhage makes of this important lesion a difficult diagnosis to achieve. We present our results of 248 preterm infants scanned by ultrasound, in our Institute, to detect intraventricular or subependymal hemorrhage. Out of the 248 patients the incidence of SEH or IVH was 21%, only 1 case of intracerebellar hemorrhage, as complication of IVH-SHE, was found. Another intracerebellar hemorrhage was diagnosed in a full term newborn without other signs of hemorrhage.

In utero ultrasonographic features of campomelic dysplasia.

A prenatal diagnosis of campomelic dysplasia in a primigravida is described. First level fetal ultrasonography demonstrated bowing and shortening of lower limbs. Second level examination allowed the correct diagnosis by demonstrating several skeletal anomalies pathognomonic of campomelic dysplasia.

[Ultrasonics in the study of periencephalic post-traumatic effusions in the newborn and suckling infants]. / L'ecotomografia nello studio delle raccolte periencefaliche post-traumatiche nel neonato-lattante.

The authors report their experience in 8 cases of cranial post-traumatic superficial collections, studied by US. Traumatic cranial injuries with superficial collections are not rare in newborns; their early and complete diagnosis is necessary for a correct therapeutic approach. Sonography could be used as a first choice procedure but presents some limitations in the study of the posterior fossa. Therefore CT remains the technique of choice.

Schizencephaly: prenatal diagnosis by computed sonography and magnetic resonance imaging.

Magnetic resonance (MR) imaging was performed at 29 weeks of pregnancy after ultrasonographic detection of an abnormal cleft in the fetal brain. Fetal neuromuscular blockade was induced by pancuronium bromide injected into the umbilical vein under continuous ultrasound (US) guidance. MR images supported the echotomographic diagnosis of schizencephaly improving the visualization of symmetrical broad clefts connecting the lateral ventricles with the subarachnoid space. Schizencephaly was finally confirmed by neonatal US, computed tomography, and MR.

[Echotomography diagnosis of congenital hip dysplasia. Experience with 1120 cases]. / Diagnosi ecotomografica di displasia congenita dell'anca. Esperienza su 1120 casi.

The congenital hip dysplasia is based on an abnormal acetabular rim, with pathologic capsula and ligaments. These pathologic structures can lead to the subtotal or total dislocation of the hips. In newborns the joint cartilaginous structures, can be visualized by sonography and it is possible to diagnose the different grade of dysplasia. Our series, in part previously selected, consist in 1120 joints. The results in newborns up to 3 months of age are: normal joints 79.5%, immature 10.7%, pathologic 9.8%. After 3 months of age: normal joints 93%, dysplastic 5.2%, subdislocated 0.6%, dislocated 1.2%. In all the cases we used the morphologic Graf technique and in newborns also the dynamic examination according to Novick to precise the minimal degree of the instability of the hips. Sonography permits an early diagnosis of hip dysplasia without false positive or negative results.