3DUS as an alternative to MRI for measuring renal volume in children with autosomal dominant polycystic kidney disease.

BACKGROUND: Total kidney volume, measured by magnetic resonance imaging (MRI), is a validated disease progression marker in adults with autosomal dominant polycystic kidney disease (ADPKD). However, in childhood, MRI is burdensome, explaining the need for alternatives. METHODS: Kidney volume (KV) was evaluated in 30 children with ADPKD, using three-dimensional ultrasound (3DUS), applying the ellipsoid method and manual contouring (KV3DUS-ellipsoid, KV3DUS-contour respectively); manual contouring on MRI (KVMRI), and the ellipsoid method on two-dimensional ultrasound (2DUS, KV2DUS). Correlations and differences were evaluated using Pearson's r and Wilcoxon signed-rank tests, and variability using Bland-Altman plots. RESULTS: All ultrasound volumetry methods showed significantly lower mean (± SD) KV (mL), compared with MRI-KV2DUS: 159 (±101); K3DUS-ellipsoid: 169 (±105); KV3DUS-contour: 185 (±110); KVMRI: 206 (±130); all p < 0.001. All had a strong correlation with KVMRI: 2DUS: r = 0.96; 3DUS-ellipsoid: r = 0.89 and 3DUS-contour: r = 0.94. Both before and after correction factor application, Bland-Altman plots showed lower variability and absolute error for KV3DUS-contour vs KV2DUS and KV3DUS-ellipsoid. CONCLUSIONS: Compared with MRI, ultrasound volumetry was prone to underestimation. However, KV3DUS-contour represents a valuable alternative for MRI in early ADPKD. Although more time-consuming, KV3DUS-contour is recommended over KV2DUS for estimation and follow-up of KV in ADPKD children, given its smaller error.

An Unusual Presentation of Congenital Esophageal Stenosis Due to Tracheobronchial Remnants in a Newborn Prenatally Diagnosed with Duodenal Atresia.

Congenital esophageal stenosis due to tracheobronchial remnants is defined as an intrinsic stenosis of the esophagus caused by congenital architectural abnormalities of the esophageal wall. Although CES is present at birth, it remains asymptomatic till at the age of 4-10 months, when solid food is introduced. Here we present a case diagnosed in the neonatal period after urgent cesarean for an associated duodenal atresia complicated with perforation. There is a mutual association between duodenal atresia and congenital esophageal stenosis. When duodenal atresia is diagnosed, think of possible associated esophageal abnormalities, especially when duodenal atresia is complicated by gastric perforation prenatally.

Multidetector CT of right-sided congenital diaphragmatic hernia associated with hepatopulmonary fusion in a newborn.

We present a neonate with a complex congenital cardiopathy and a right-sided diaphragmatic hernia complicated with hepatopulmonary fusion. Radiography, abdominal US and multidetector CT (MDCT) demonstrated right-sided lung hypoplasia and liver herniation. In addition, MDCT angiography showed abnormal pulmonary vascular anatomy. At surgery, a right-sided diaphragmatic hernia with a partially herniated liver and hepatopulmonary fusion was confirmed. There was no aberrant systemic vascular supply towards the lower lobe, as seen in extralobar sequestration. MDCT angiography of the chest and upper abdomen with optimal enhancement and reconstruction of the pulmonary and hepatic vasculature can demonstrate associated anomalies in cases of suspected primary or secondary right lung hypoplasia.

Cross-sectional study of tracheomegaly in children after fetal tracheal occlusion for severe congenital diaphragmatic hernia.

PURPOSE: To measure tracheal dimensions in children with congenital diaphragmatic hernia (CDH) who had undergone fetoscopic endoluminal tracheal occlusion (FETO) or were treated expectantly during gestation. MATERIALS AND METHODS: The study was approved by the local ethics committee. Computed tomography was performed in 23 patients (14 boys and nine girls) aged 1 month to 6.5 years, and the anteroposterior diameter, width, area, and perimeter of the trachea were determined. Seven of the 23 patients had undergone FETO and 16 had been treated expectantly. The relative difference of each parameter between the two most proximal concentric sections of the trachea, just below the larynx, and the two sections on which the trachea was the largest was compared between both groups (Mann-Whitney U test). Regression statistics were applied to maximum and mean tracheal areas as a function of age. Each trachea was divided into quartiles, and mean areas normalized to 3 years of age were analyzed for each quartile as a function of its relative position on the trachea (Student t test). RESULTS: Tracheal width, area, and perimeter were significantly different between both groups. A linear relationship was observed between the maximum and mean tracheal areas and age for both the FETO group (maximum tracheal area: R(2) = 0.83, P = .0045; mean tracheal area: R(2) = 0.92, P = .0005) and the non-FETO group (maximum tracheal area: R(2) = 0.66, P = .0001; mean trachea area: R(2) = 0.66, P = .0001). The maximum tracheal area in both groups tended to decrease toward the age of 5 years. Significantly different mean tracheal areas per tracheal quartile (P < .05) were found for all quartiles except for the proximal one-fourth. CONCLUSION: The relative difference between proximal and largest tracheal width, area, and perimeter was significantly larger in patients who underwent FETO than in those treated expectantly, demonstrating tracheal dilatation in the former. Measurements of tracheal dimensions at different levels indicate a maximum dilatation in the lower half of the trachea, which tends to level off toward the age of 5 years.

T2 quantifications of lungs in the fetal lamb with experimentally-induced congenital diaphragmatic hernia.

OBJECTIVE: As an aid to evaluate lung hypoplasia, we investigated the difference between T2 value in fetal lungs of lambs with surgically-induced left-sided diaphragmatic hernia (DH) and gestational age (GA)-matched control littermates (normal [NL]). METHODS: Lungs were divided into two groups: DH (n = 4) and NL (n = 6). DH was induced at 65-75 days GA (term = 145 days). Fetal MRI was performed (mean GA: 120 days) with half-Fourier acquisition single-shot turbo spin-echo (TE: 60 ms) and rapid acquisition with relaxation enhancement (TE: 350 ms) in the same location (1.5-T Philips, Gyroscan, Best, The Netherlands). T2 of each lung was calculated for multiple regions of interest by taking natural logarithm of signal-to-noise ratio. Mean T2 was compared between DH and NL (unpaired analysis for entire group). Paired comparison between left/right lung was made within DH and NL. RESULTS: Unpaired analysis showed significantly lower T2 of left respectively right lungs in DH (p = 0.02 [respectively] 0.05]) compared to NL (n = 6), as well as between the T2 of all DH versus NL (p = 0.001). In DH, calculated T2 appeared to be lower in left than in right lungs (difference ranged from -2 to +49%). In NL, left and right lungs showed comparable T2. CONCLUSION: Measurement of T2 signal intensity in DH lungs is feasible and show lower T2 in comparison to NL lungs. Left lungs from lambs with DH show lower T2 than right lungs.

Fetal magnetic resonance imaging of an intracranial venous thrombosis. Case report.

OBJECTIVE: To describe the magnetic resonance imaging findings of an antenatal intracranial venous thrombosis. METHODS: Prenatal ultrasound (US) at 22 weeks' gestational age (GA) in a 27-year-old patient (G2 P1) revealed a nonspecific avascular echogenic mass above the cerebellum evaluated with MRI at 23 weeks' GA. RESULTS: With MRI an enlarged occipital pericerebral space with a layered ovoid mass at the torcular herophili was demonstrated and MRI characteristics suggested a subacute cloth. In follow-up, an important enlargement of the mass was noticed on US examinations. After multidisciplinary consult and parental consent, a prostaglandin induction was performed at 25 weeks' GA. The baby died immediately after birth. Pathology confirmed the diagnosis of a venous thrombosis at the torcular herophili with extension into the superior sagittal sinus. CONCLUSION: In our case, MRI findings were helpful in diagnosing an intracranial fetal venous thrombosis.

Massive lung collapse with partial resolution after several years: a case report.

BACKGROUND: Bronchitis obliterans is a severe and extremely rare complication of respiratory tract infections in children and is characterized by massive atelectasis and collapse of the affected lung. Of the rare cases reported in the literature all surviving children underwent surgical resection of the collapsed lung. CASE PRESENTATION: We report an infant with bronchitis obliterans that was treated conservatively. 5 years after the initial event, partial lung re-expansion was documented. CONCLUSION: This case therefore supports a conservative treatment whenever possible with pneumonectomy only as a last treatment option.

Ultrasound diagnosis of infantile scrotal emphysema.

We report a 4-month-old boy who developed scrotal emphysema following removal of a chest drain. The initial diagnosis was made by ultrasonography. This report describes the sonographic findings, pathophysiological mechanisms and outcome of this rare entity.

High-resolution real-time compound ultrasound imaging of transient protein-losing gastropathy of childhood.

We report the case of a 1.5-year-old girl with transient protein-losing gastropathy with hypertrophic gastric folds (PLGH). The diagnosis of PLGH was made by abdominal ultrasound (US) and not by an upper gastrointestinal (UGI) study as reported in many previous publications. Real-time compound ultrasound imaging showed in high detail the echogenic thickening of the mucosal gastric layer and associated hyperaemia on colour Doppler US. These ultrasonic findings highly correlated with the endoscopic US findings and microscopic changes of the gastric wall in PLGH. An awareness of the high-resolution abdominal ultrasound appearances of PLGH may facilitate earlier diagnosis and obviate the need for an upper GI contrast series.

Postnatal cranial ultrasonographic findings in feto-fetal transfusion syndrome.

Our objective was a retrospective evaluation of cranial US in survivors of twin pregnancy with feto-fetal transfusion syndrome (FFTS), with knowledge of prenatal treatment and neonatal/postnatal clinical data. In 18 pregnancies with FFTS (January 1996 to May 2000), pregnancy management and outcome, and neonatal clinical/neurological data and follow-up (age of 3-7 months) were documented when available. Postnatal cranial US abnormalities were differentiated in prenatal and peri/postnatal lesions, respectively, in "donor" and "recipient." The statistical analysis used was Mann Whitney U test and Fisher's exact test. Overall pregnancy survival rate was 19 of 36 (53%); mors in utero occurred in five twin members. Gestational age at birth was significantly lower in FFTS after laser coagulation (13 of 18; p<0.05). Initial (

Tuberous sclerosis with cystic renal disease and multifocal renal cell carcinoma in a baby girl.

An infant, in whom the prenatal diagnosis of tuberous sclerosis complex was made, presented with extreme bilateral nephromegaly owing to diffuse cystic changes. Histology of the resected non-functioning left kidney revealed, in addition to the characteristic cysts, two foci of renal cell carcinoma not visible on US or MRI. This infant is exceptional given the extensive cystic transformation of both kidneys and the presence of malignant lesions at this young age.