Development and validation of a prognostic prediction model including the minor lymphatic pathway for distant metastases in cervical cancer patients.

To develop and validate a prognostic model, including the minor lymphatic pathway (internal iliac and presacral nodes). STUDY DESIGN: Retrospective cohort. PARTICIPANTS: Locally advanced cervical cancer underwent concurrent chemoradiotherapy. SAMPLE SIZE: 397 and 384 patients in the development and validation data set. PREDICTORS: Our new nodal staging system with the minor lymphatic pathway. OUTCOME: Distant metastases. STATISTICAL ANALYSIS: Cox regression; net reclassification improvement (NRI) and decision curve analysis (DCA). Our new nodal system was the strongest predictor. The predictors in the final model were new nodal system, tumor stage, adenocarcinoma, initial hemoglobin, tumor size and age. The nodal system and the pretreatment model had concordance indices of 0.661 and 0.708, respectively, with good calibration curves. Compared to the OUTBACK eligibility criteria, the nodal system showed NRI for both cases (22%) and controls (16%). The pretreatment model showed NRI for cases (31%) and controls (18%). DCA in both models showed threshold probability of 15% and 12%, respectively, when compared with 24% in OUTBACK eligibility criteria. Our new nodal staging system and the pretreatment model could differentiate between high-risk and low-risk patients, thus facilitating decisions to provide more aggressive treatment to prevent distant metastases.

Evaluation and external validation of minor lymphatic pelvic pathway for distant metastases in cervical cancer patients treated with concurrent chemoradiotherapy.

To evaluate and validate the minor lymphatic pathway for distant metastases in cervical cancer. This is a retrospective cohort of cervical cancer patients underwent curative concurrent chemoradiotherapy. We used original dataset from 1 university hospital and validation dataset from 3 university hospitals. Lymphadenopathy status in CT imaging was reviewed by radiologist in either the obturator and external iliac nodes (major pathway) or the internal iliac and presacral nodes (minor pathway). We then used Cox regression to adjust for all potential confounders, including paraaortic nodes, T stage, histology, age, total treatment time, total number of nodes, total short axis of nodes. 397 and 384 patients were in the original and validation datasets (median follow-up period, 59.5 month's). The minor pathway was independent prognostic factor in multivariable analysis [HR=2.64; 95%CI=1.07-6.55; P = 0.036] and [HR=14.84; 95%CI=3.15-70.01; P= 0.001] in original and validation datasets, respectively. Whereas, the major pathway was statistically non-significant. Further validation showed that the minor pathway had the highest HR for distant metastases with both the EMBRACE (HR=6.05; 95% CI=1.30-28.08; P = 0.022) and the FIGO 2018 (HR=7.43; 95% CI=2.94-18.78; P<0.001) in the original dataset. A similar result was found with the validation dataset: EMBRACE, HR=30.91; 95% CI=2.78-343.62; P = 0.005; and FIGO 2018, HR=42.41; 95% CI=8.83-203.60; P<0.001.This is the first clinical study to validate that the minor lymphatic pathway was predominantly associated with distant metastases in cervical cancer. This finding should be validated in larger cohort to further integrate in standard staging for prediction of distant metastases.

Effect of a 12-week home-based exercise training program on aerobic capacity, muscle mass, liver and spleen stiffness, and quality of life in cirrhotic patients: a randomized controlled clinical trial.

BACKGROUND: Physical inactivity and sarcopenia are two important predictors associated with increased morbidity and mortality in patients with cirrhosis. At present, the benefit of a home-based exercise training program is not well established in cirrhotic patients. The main objective of this study was to evaluate the effect of a 12-week home-based exercise training program on aerobic capacity in cirrhotic patients. METHODS: This is a randomized controlled study. Patients with compensated cirrhosis were randomized by a block of 4 with concealed allocation to the home-based exercise training (n = 20) or control (n = 20). Both groups received protein supplementation (9 g/day) for 12 weeks. The home-based exercise training program included several aerobic/isotonic moderate-intensity continuous training exercises for 40 min per session, at least four times a week, with a total duration of 12 weeks. The heart rate was continuously monitored using a Garmin® watch. In the control group, patients received exercise instruction without active encouragement and continuous monitoring. The primary outcome was a change in the 6-min walk test from baseline. Secondary outcomes were the difference in thigh muscle thickness, liver stiffness, spleen stiffness, and quality of life. RESULTS: A total of 40 patients were enrolled prospectively. The mean age was 56.3 ± 7.8 years, with a male predominance of 65%. The mean body mass index was 25.23 ± 3.0 kg/m2, and all were Child-Pugh A. Chronic hepatitis B or C was the primary cause of cirrhosis. The baseline values were a 6-min walk test of 475 ± 70 m, liver stiffness of 15.3 ± 9.3 kPa, spleen stiffness of 29.8 ± 21.7 kPa, and thigh muscle thickness (average compression index) of 0.64 ± 0.2 cm/m2. All baseline characteristics between the two groups were not different except the mean muscle mass which was significantly higher in the home-based exercise training group (p = 0.03, 95% CI 0.01 to 0.17). At the end of the study, no significant difference in the 6-min walk test was observed (p = 0.36, 95% CI -15.5 to 41.7). Liver stiffness measurement significantly improved in both groups, but no significant difference between groups was demonstrated (p = 0.77, 95% CI -1.3 to 1.8). Thigh muscle thickness was not different between groups. The fatigue domain of the quality of life index was significantly improved in the home-based exercise training group compared with the control group (p = 0.05, 95% CI 0.00 to 0.67). No adverse events occurred in a home-based exercise training program. CONCLUSIONS: A 12-week moderate-intensity home-based exercise training program in compensated cirrhotic patients significantly improved the fatigue domain of the quality of life index without an increase in adverse events. However, no benefit in terms of aerobic capacity, thigh muscle mass, liver stiffness, and spleen stiffness was demonstrated. TRIAL REGISTRATION: Thai Clinical Trials Registry number TCTR20190926002, 26/09/2019 (Retrospectively registered).

Diagnostic performance of qualitative and quantitative shear wave elastography in differentiating malignant from benign breast masses, and association with the histological prognostic factors.

BACKGROUND: To determine the diagnostic performance of qualitative and quantitative shear wave elastography (SWE) and the optimal cutoff values of the quantitative SWE parameters in differentiating malignant from benign breast masses, and to evaluate the association between the quantitative SWE parameters and histological prognostic factors. METHODS: A gray scale ultrasound and SWE were prospectively performed on a total of 244 breast masses (148 benign, and 96 malignant) in 228 consecutive patients before an ultrasound-guided needle biopsy. The qualitative SWE and quantitative SWE parameters (the mean elasticity, maximum elasticity, and elasticity ratio) were measured in each mass. The diagnostic performance of SWE and the optimal cutoff values of the quantitative SWE parameters were obtained. An association analysis of the parameters and histological prognostic factors was performed. RESULTS: The malignant masses had a more heterogeneous pattern on the qualitative SWE than benign masses (P<0.001). The quantitative SWE parameters of the malignant masses were higher than those of the benign masses (P<0.001); the mean elasticity, maximum elasticity, and elasticity ratio of the benign masses were 19.73 kPa, 23.98 kPa, and 2.78, respectively; and the mean elasticity, maximum elasticity, and elasticity ratio of the malignant masses were 88.13 kPa, 98.48 kPa, and 10.64, respectively. The optimal cutoff value of the mean elasticity was 30 kPa, of the maximum elasticity was 36 kPa, and of the elasticity ratio was 4.5. The maximum elasticity had the highest AUC. Combining the three SWE parameters to differentiate between the malignant and benign masses increased the negative predictive value (NPV), which correctly downgraded 72.73% of BI-RADS category 4A masses to BI-RADS category 3. No statistically significant association was found between the quantitative SWE parameters and the tumor grading, tumor types, axillary lymph node statuses, or molecular subtypes of the breast cancers (P>0.05). CONCLUSIONS: The qualitative and quantitative SWE provided good diagnostic performance in differentiating malignant and benign masses. The maximum elasticity of the quantitative SWE parameters had the best diagnostic performance. Adding the three combined quantitative SWE parameters to the BI-RADS category 4A masses potentially downgraded them to BI-RADS category 3 and avoided unnecessary biopsies. No statistically significant association was found between the quantitative SWE parameters and the histological prognostic factors.

Clinical, mammographic, and ultrasonographic characteristics of diabetic mastopathy: A case series.

PURPOSE: Diabetic mastopathy (DMP) is a rare benign breast lesion that mimics breast cancer on ultrasound. Our aims were to identify patient characteristics and imaging features of the disease. METHODS: We conducted retrospective searches of our database for DMP lesions that were pathologically confirmed between January 2004 and November 2015. Mammographic and ultrasound features were reviewed by two experienced radiologists. RESULTS: Twelve women were identified with 16 lesions. Most patients (83%) had type 2 diabetes mellitus (DM) and over half were insulin-dependent (58.3%), with a mean time of 16.9 years between the diagnosis of DM and that of DMP. There were negative findings on mammography for 46.7% of the lesions, including larger-sized lesions. Ultrasound revealed various features, including irregular shape (81.3%), indistinct margins (100%), parallel orientation to the chest wall (93.8%), marked hypoechogenicity (87.5%), and posterior shadowing (62.5%). CONCLUSIONS: DMP was more common in patients with longstanding DM; in particular, type 2 DM and insulin-dependent patients. DMP lesions were usually occult on mammography, despite the relatively large size of DMP, which may help distinguish DMP from invasive cancer. Ultrasound detected several features that are also present in invasive cancer, making tissue sampling necessary to distinguish these.

A Comparison of Accuracy of Planar and Evolution SPECT/CT Bone Imaging in Differentiating Benign from Metastatic Bone Lesions.

Objective: To compare sensitivity, specificity, accuracy and diagnostic confidence in the differentiation between benign and metastatic bone lesions on whole body planar bone scintigraphy and Evolution SPECT/CT. Material and Method: Eighty diagnosed or suspected cancer patients with indeterminate lesions on planar scintigraphy were recruited in the present prospective study. Additional whole body Evolution SPECT/CT was performed after whole body planar scintigraphy. All lesions on both imagings were categorized into 5 categories; definitely metastasis, probably metastasis, indeterminate, probably benign and definitely benign. The diagnosis of each lesion was confirmed by follow-up imaging, pathological findings or clinical follow-up for at least 6 months. Results: Detected lesions on planar scintigraphy and Evolution SPECT/CT imaging were 442 and 477 lesions, respectively.The sensitivity, specificity and accuracy of planar scintigraphy and Evolution SPECT/CT imaging in the diagnosis of metastatic lesions were 27% (95% CI: 13.8, 44.1), 63.2% (95% CI: 58.5, 67.7), 60%, and 97.3% (95% CI: 85.8, 99.9), 100% (95% CI: 96.4, 100) and 99.8%, respectively. Indeterminate lesions on planar scintigraphy were 34.2% (151 lesions from total 442 lesions, which 135 of these 151 indeterminate lesions or 89.4% were located in axial skeleton). Evolution SPECT/CT images were able to characterize all indeterminate lesions. Conclusion: Differentiation of benign and metastatic lesions by Evolution SPECT/CT images has superior diagnostic performance and diagnostic confidence over the planar scintigraphy. Thus, Evolution SPECT/CT images should be considered in characterization of indeterminate lesions on planar scintigraphy, especially in the axial skeleton.

MRI of placenta percreta: differentiation from other entities of placental adhesive disorder.

OBJECTIVES: To retrospectively review the MRI findings of placenta percreta and identify those helpful for differentiation from non-placenta percreta. MATERIALS AND METHODS: The MRI images of 21 patients with a preliminary diagnosis of placental adhesive disorder scanned between 2005 and 2014 were evaluated. Radiologists blinded to the final diagnosis evaluated six previously described MRI findings of placenta adhesive disorder. The sensitivity, specificity, accuracy, negative predictive value (NPV), and positive predictive value (PPV) of MRI for the diagnosis of placenta percreta were also calculated. RESULTS: The study included 12 cases of placenta percreta and 9 cases of non-placenta percreta. Invasion of placental tissue outside the uterus was found only in placenta percreta (p = 0.045; sensitivity 41.7 %; specificity 100 %). All placenta percreta cases also had a moderate to marked degree of heterogeneous placental signal intensity (p = 0.063; sensitivity 100 %; specificity 33.3 %). The size of the dark bands on T2-weighted imaging, and the presence of disorganized intra-placental vessels, showed no statistically significant difference between placenta percreta and non-placenta percreta. The sensitivity, specificity, NPV, PPV, and accuracy of MRI for detection of placenta percreta were 91.7, 44, 80, 68, and 71.4 %, respectively. CONCLUSIONS: MRI is recommended for the evaluation of placenta percreta, with the most specific signs including the invasion of placental tissue outside the uterus on B-FFE sequences, and consideration of the degree of placental signal heterogeneity. The size of the T2 dark band alone, or bizarre disorganized intra-placental vessels, did not correlate with the severity of invasion.

Correlation between the severity of hepatitis B cirrhosis and CT volumetry-based hepatic segmental anatomic changes.

OBJECTIVE: To study the correlation between the changes of hepatic segmental volumes and the severity of hepatitis B cirrhosis, classified by Child-Pugh score. MATERIAL AND METHOD: The study cohort comprised viral hepatitis B cirrhotic patients with different severity (Child-Pugh score grade A to C) and control subjects who underwent hepatic computed tomographic (CT) scan between February 2006 and May 2012. The volumes of total liver (TLV), right lobe (RV), left medial segment (LMV), left lateral segment (LLV), and caudate lobe (CV) were measured and compared between the control group and Child-Pugh A, B, and C groups. RESULTS: Among 120 hepatitis B cirrhotic subjects, there were 85 males (70.8%) with the mean age of 59.2 years (range 36-86 years). Sixty-two subjects were Child-Pugh A, 39 were Child-Pugh B, and 19 were Child-Pugh C. Among 62 control subjects, there were 28 males (45.2%) with the mean age of54.5 years (range 19-82 years). The TLVand R Vwere significantly decreased in Child-Pugh B and C subjects when compared with control subjects. LMV was significantly decreased in Child-Pugh A and continually decreased along with the severity of the disease. LLV was significantly increased in Child-Pugh A and B, but relative in Child-Pugh C subjects. CV was not significantly different in these four groups. CONCLUSION: Hepatitis B cirrhotic patients had progressive hepatic atrophy, initially by left medial segment, followed by right lobe and the whole liver Hypertrophy of left lateral segment was evident in early cirrhosis but not significantly shown in severe cirrhosis. Hypertrophy of caudate lobe was not evident in any degrees of hepatitis B cirrhosis.

Renal vascular variants in living related renal donors: evaluation with CT angiography.

BACKGROUND: Renal vascular variants may complicate the surgical techniques of living related renal transplantation. Renal computed tomographic (CT) angiography is now well accepted for preoperative renal vascular mapping in living related renal donors. OBJECTIVE: To study the prevalence of renal vascular variants in living related renal donors using CT angiography. MATERIAL AND METHOD: Preoperative renal CT angiography of 65 consecutive living related renal donors were retrospectively reviewed by two abdominal radiologists on a 3-D workstation. The number and branching patterns of bilateral renal arteries and veins, as well as the presence of renal arterial and venous variants were described. RESULTS: Supernumerary renal arteries and early branching were present in 18.5% and 12.8% respectively on the right kidneys and 27.7% and 22.4% respectively on the left kidneys. The prevalence of precaval right renal artery was 4.6%. Supernumerary renal veins were present in 35.4% and 1.5% on the right and left kidneys, respectively. Late confluences of left renal veins were identified in 1.5% of left kidneys. Other venous anomalies included 1.5% duplicated inferior vena cava (IVC), 1.5% circumaortic left renal vein, 1.5% retroaortic left renal vein, 1.5% outsized left gonadal veins drained into the left renal vein, and 6.2% right gonadal vein drained into the right renal vein. CONCLUSION: Renal vascular anatomical variants were common. The surgeons and the radiologists should be aware of these variants to prevent postoperative complications.

CT appearances of post-radiation livers in patients with unresectable cholangiocarcinoma.

OBJECTIVE: To characterize the computed tomographic (CT) findings of post-radiation livers and the interval changes in patients with unresectable cholangiocarcinoma. MATERIAL AND METHOD: Thirteen patients with unresectable cholangiocarcinoma who received concurrent chemoradiation with conformal radiotherapy technique (50 to 66 Gy, 2 Gy/fraction) were included in the present study. CT at pre-radiation and sequential follow-up at 1, 3, 6, 9 and 12 months were retrospectively reviewed by two abdominal radiologists to identify CT characteristics of post-radiation liver and the interval changes. RESULTS: CT at pre-radiation and sequential follow-up at 1, 3, 6, 9 and 12 months were available in 92.3%, 100%, 76.9%, 53.8%, 30.8% and 23.1%, respectively. Post-radiation livers showed sharply-delineated, hypodense radiation areas, which were well related with the isodose line of 35 to 56 Gy (mean = 44.4 +/- 6.55 Gy). These radiation areas were mostly appreciated on portal venous phase at 1-month follow-up study in 12 of 13 (92.3%) patients and these were gradually less defined in subsequent studies. Progressive decrease size of radiation areas with persistent enhancement on delayed phase images were recognized. Progression of hepatic cortical irregularity was seen in four (30.8%) patients, as well as pulmonary fibrosis of lung bases. CONCLUSION: Post-radiation liver in patients with unresectable cholangiocarcinoma showed a sharply-defined, hypodense radiation area, which was mostly appreciated in 1-month follow-up CT and was gradually less defined in subsequent studies with evidence of progressive atrophic change.

Computed tomography characteristics of hepatic and splenic abscesses associated with melioidosis: a 7-year study.

PURPOSE: This study aimed to characterise the CT findings associated with hepatic and splenic melioid abscesses. METHODS: Patients with CT evidence of hepatic and/or splenic abscesses were retrospectively evaluated for clinical evidence of melioidosis over a 7-year period. After blinded review of the CT characteristics of intra-abdominal abscesses (IAA), we conducted a stratified analysis of patients with and without melioid IAA. RESULTS: Among 49 patients with CT evidence of hepatic and/or splenic IAA, the mean age was 50.2 years, 22 (44.9%) were women and eight (16.3%) had laboratory confirmation of melioidosis. For the 113 IAA, 33 were melioid abscesses (15 liver and 18 spleen) and 80 were non-melioid abscesses (69 liver and 11 spleen). Splenic IAA were more common in the melioid group (P = 0.001) and smaller in diameter than the hepatic IAA (P < 0.001). Melioid IAA were smaller than non-melioid IAA (P < 0.001) and the CT necklace sign was the strongest predictor for melioid IAA (odds ratio = 24.6, P = 0.006) with 100% specificity. Other significant predictors for melioidosis were concurrent hepatic and splenic involvement (P = 0.009), multiple abscesses (P = 0.015) and residence in an endemic area (P = 0.047). By multivariate analysis, concurrent hepatic and splenic involvement was the sole predictor of melioidosis (adjusted odds ratio = 11.3, 95% confidence interval = 1.6-77.5, P = 0.014). CONCLUSION: The CT necklace sign, along with concurrent hepatic and splenic IAA, were highly suggestive of melioidosis in persons from Central Thailand.

Renal artery stenosis: diagnostic performance of balanced fast field gradient echo MRA.

OBJECTIVE: To compare the diagnostic performance of new B-FFE MR angiography with contrast-enhance MR angiography in diagnosis of renal artery stenosis. MATERIAL AND METHOD: One hundred and fourteen patients suspected of having renal artery stenosi underwent MR angiography with 2D B-FFE technique and 3D post contrast enhancement. RESULTS: Compared with contrast enhanced MR angiography, B-FFE MR angiography correctly depicte, significant stenotic renal arteries (> or = 50% stenosis) in 13 and 12 renal arteries from 21 renal arteries reviewed by first and second radiologists, respectively. The overall sensitivity, specificity, negative an, positive predictive values of B-FFE MR angiography in diagnosis of significant renal artery stenosis (> or =50% stenosis) reviewed by first and second radiologists, were 57.1-61.9%, 91.9-93.6%, 96.1-96 4%, and 81.3 92.3%, respectively. CONCLUSION: B-FFE MR angiography of renal arteries can be promising technique for screening patient. who are suspected of having renovascular disease without requirement of intravenous contrast injection especially the kidney disease patients.

Surgically relevant normal and variant renal parenchymal and vascular anatomy in preoperative 16-MDCT evaluation of potential laparoscopic renal donors.

OBJECTIVE: Using 16-MDCT, we describe and quantify the frequency and types of renal anatomic variants and findings relevant for preoperative evaluation and surgical planning for potential laparoscopic renal donors. MATERIALS AND METHODS: On 16-MDCT, 126 consecutive potential donors underwent scanning before contrast administration and after i.v. power injection of nonionic contrast material during the arterial, nephrographic, and excretory phases. On a 3D workstation, CT images were evaluated retrospectively in consensus by three abdominal imagers. The number and branching pattern of bilateral renal arteries and veins, including anomalies of the inferior vena cava and lumbar-gonadal axis, were categorized along with the frequency of incidental findings of the renal parenchyma and collecting system. RESULTS: Major arterial variants including supernumerary and early branching arteries were present in 16% and 21%, respectively, of left kidneys and 22% and 15%, respectively, of right kidneys. Major and minor venous variants were detected in 11% and 58% of left kidneys and 24% and 3% of right kidneys. Late confluence of the venous trunk was identified in 17% of left kidneys and 10% of right kidneys. Incidental parenchymal and urothelial abnormalities, most commonly cysts and calyceal calcifications, were identified in 30% of the kidneys. Other relevant incidental findings included focal infarcts, cortical scars, atrophic scarred kidney, and bilateral papillary necrosis. Urothelial variants included bilateral simple ureteroceles and rightsided complete duplicated collecting system. CONCLUSION: 16-MDCT angiography and urography allow confident detection and classification of a variety of anatomic and incidental anomalies relevant to the preoperative selection of potential laparoscopic renal donors and to surgical planning.

Utility of 16-MDCT angiography for comprehensive preoperative vascular evaluation of laparoscopic renal donors.

OBJECTIVE: Our objective was to determine the efficacy of 16-MDCT angiography in preoperative evaluation of vascular anatomy of laparoscopic renal donors. METHODS AND MATERIALS: Fifty-five consecutive renal donors (25 men and 30 women) underwent 16-MDCT angiography followed by donor nephrectomy. In the arterial and nephrographic phases, images were acquired with 60% overlap and 0.6-mm reconstruction in both phases after 120 mL of iohexol was injected at 4 mL/sec. On a 3D workstation, images were evaluated retrospectively by two abdominal imagers blinded to surgical results with respect to number and branching pattern of renal arteries and major and minor renal veins. These CT angiography results were compared with surgical findings. RESULTS: The surgically confirmed sensitivity of both reviewers (1 and 2) using the MDCT data for detection of renal arteries was 98.5% (65 of 66), and accuracies were 97.0% for reviewer 1 and 95.5% for reviewer 2. Sensitivity and accuracy detection of renal veins was 97% (61 of 63) and 98% (62 of 63) for reviewer 1 and reviewer 2, respectively. Sensitivity and accuracy detection of early arterial bifurcation (< 2 cm from aorta) was 100% (14 of 14), and sensitivity in detection of late venous confluence (< 1.5 cm from aorta) was 100% (8 of 8). All major renal venous variants were identified; reviewer 1 identified 78% (18 of 23) minor venous variants, and reviewer 2 identified 83% (19 of 23) minor venous variants. There were no hemorrhagic complications at surgery. Excellent agreement between reviewers (kappa = 0.92-0.97) was achieved for detection of normal and variant anatomy. CONCLUSION: 16-MDCT angiography enabled excellent preoperative detection of arterial anatomy and venous laparoscopic donor nephrectomy.

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome presenting with a large nephrogenic cyst, severe oligohydramnios and hydrops fetalis: a case report and review of the literature.

OBJECTIVES: To report a case of EEC syndrome with a large nephrogenic cyst detected by prenatal ultrasonography. METHODS: Prenatal ultrasonographic detection, genetic counselling, termination of pregnancy, radiographic study, autopsy and ultrastructural study of scalp hair. The literature on EEC syndrome with genitourinary anomalies and prenatal diagnosis was also reviewed. RESULTS: A 6-cm cyst in the right side of abdominal cavity was detected in a fetus of 24 weeks' gestational age with severe oligohydramnios and hydrops fetalis. The autopsy revealed bilateral renal dysplasia with a large nephrogenic cyst at the right side and markedly hypoplastic urinary bladder and pulmonary hypoplasia. The fetus also had ectrodactyly and syndactyly of hands and feet and ectodermal dysplasia and left cleft lip and palate. Ultrastructure of hair from scanning electron microscopy revealed no obvious abnormality. CONCLUSION: This is a rare case of fetal malformation partly detected prenatally. In order to detect this syndrome, careful ultrasound search for acral anomalies should be performed in cases with renal malformation or obstructive uropathy.