13 results
Case 26 - Scimitar syndrome
- from Section 3 - Cardiac imaging
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 107-110
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Summary
Imaging description
A full-term neonate developed respiratory distress at birth. There was a history of in utero diagnosis of complex heart disease including hypoplastic left heart as well as a hypoplastic right lung. The chest radiograph at birth (Fig. 26.1a) demonstrated decreased volume of the right lung with dextroposition of the heart. Echocardiography demonstrated a large atrial septal defect (ASD), hypoplastic left heart, and large patent ductus arteriosus (PDA). CT angiography of the chest on day 2 of life demonstrated findings consistent with scimitar syndrome, including abnormal pulmonary venous drainage of the right lung into the inferior vena cava (IVC) via a scimitar vein in the right lower lung (Fig. 26.1b). In addition a small right pulmonary artery was noted with the hypoplastic right lung (Fig. 26.1c). There was also evidence of a right lower lobe horseshoe lung extending posteriorly across the midline behind the IVC to interface with the left lung (Fig. 26.1b). This received arterial supply from an aberrant proximal right pulmonary artery branch (Fig. 26.1c). Venous drainage and airway bronchial branches of this segment were also on the right side (not shown). Dextroposition of the heart was noted along with a hypoplastic left ventricle and large ASD (not shown). Also seen was a large PDA and juxtaductal coarctation of the aorta as well as a left-sided superior vena cava (SVC) draining to the coronary sinus (Fig. 26.1d, e).
Case 63 - Urachal mass
- from Section 6 - Urinary imaging
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 264-268
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Summary
Imaging description
A 35-year-old lady in her first trimester of pregnancy presented with abdominal pain and a palpable anterior abdominopelvic mass. Ultrasound (US) of the pelvis (Fig. 63.1a, b) demonstrated a gravid uterus with multiple uterine fibroids (not shown). In addition, a hypoechoic solid mass with moderately prominent vascularity was noted superior to the uterus, extending up to the umbilicus, raising the suspicion of a pedunculated fibroid. An MRI of the pelvis (Fig. 63.1c, d) also revealed a large heterogeneously enhancing mass beneath the anterior pelvic wall, extending up to the umbilicus. The mass was resected and histopathological analysis revealed a squamous cell carcinoma of a urachal remnant. The pregnancy continued successfully to term.
Importance
The urachus is a three-layered remnant of the embryonic allantois (innermost layer of transitional epithelium, an intermediate layer of fibroconnective tissue, and outer smooth muscle layer). The urachus extends from the dome of the bladder to the umbilicus and gets progressively obliterated by four to five months of gestation, leaving behind a fibrous cord called the median umbilical ligament. The complete obliteration of the urachus was originally thought to be a prenatal process; however, recent literature has indicated that the involution may continue in the first few months of life. Failure of this obliteration may result in different types of urachal remnant anomalies, including a completely patent urachus (vesicoumbilical fistula, 48%), vesicourachal diverticulum (3%), umbilical-urachal sinus (18%), and urachal cyst (31%). A normal urachal remnant is quite commonly seen as an incidental finding on pelvic US in children as a small hypoechoic structure, superior to the urinary bladder (Fig. 63.2). A patent urachus can be seen as a tubular connection between the bladder and the umbilicus on sagittal us (Fig. 63.3). An umbilical-urachal sinus can be seen as a blind-ending midline tubular structure below the umbilicus, and may occasionally become infected, resulting in umbilical drainage. A vesicourachal diverticulum is generally asymptomatic and can be incidentally detected on imaging as a midline cystic structure at the superior aspect of the bladder. Occasionally, a large diverticulum may result in stasis, aberrant micturition, infection, or stone formation. Urachal diverticulum as well as other urachal anomalies are particularly associated with prune belly syndrome (see Case 58). A urachal cyst is formed when the urachus closes at both the umbilical and bladder ends and remains patent in between.
Case 64 - Wilms’ tumor
- from Section 6 - Urinary imaging
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- By Rakhee Gawande, Stanford University, Kriengkrai Iemsawatdikul, Siraraj Hospital, Mahidol University, Heike E. Daldrup-Link, Lucile Packard Children's Hospital, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 269-275
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Summary
Imaging description
A 22-month-old female presented with a history of vomiting and a palpable right abdominal mass. An abdominal ultrasound (not shown) and an MRI of the abdomen (Fig. 64.1) demonstrated a large mass involving the right kidney, with distortion of the pelvicalyceal system. In addition a large tumor thrombus was noted in the right renal vein, extending into the inferior vena cava (IVC) to the level of the mid liver with marked dilatation of these vessels. The tumor thrombus also extended into the left renal vein, up to the renal hilum (Fig. 64.1). A renal biopsy confirmed the diagnosis of a Wilms’ tumor (WT).
Importance
WT is the most common renal neoplasm in children, with 95% of cases occurring between 2 and 5 years of age. Five to seven percent of children have bilateral disease with 7% of cases being multicentric (Fig. 64.2). Nephrogenic rests, which are thought to be precursors of WT, are seen in 1% of neonates and it is believed that only 1% of these transform into WT. Histologically, WTs are of triphasic cell lineage comprising blastemal, stromal, and epithelial cells and about 7% are anaplastic. Favorable histology WT have absence of anaplasia, have a better prognosis, and are more responsive to chemotherapy. Unfavorable histology WTs exhibit anaplasia, are chemotherapy resistant, and are associated with an increased risk of recurrence.
Case 42 - Umbilical venous catheter malposition
- from Section 4 - Vascular and interventional
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 188-192
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Summary
Imaging description
A seven-day-old premature neonate presented with elevated liver enzymes and concern for a liver mass. Review of the outside CT scan of the abdomen showed a hypodense non-enhancing lesion in the right lobe of the liver (Fig. 42.1a). The outside ultrasound of the abdomen demonstrated a large thick-walled multilobulated cystic lesion with multiple septations (Fig. 42.1b). Repeat ultrasound (US) after transfer included color Doppler imaging which demonstrated the lesion to be cystic and avascular with mild perilesional hyperemia (Fig. 42.1c). Review of the outside chest radiographs revealed an abnormal anterior course of the umbilical venous line on the lateral view (Fig. 42.1d, e). The pediatric radiologist therefore suggested that rather than a neoplasm the liver “mass” represented a fluid collection/hematoma secondary to malposition of the umbilical venous line into the liver parenchyma with hemorrhage and fluid extravasation. A follow-up US at five months demonstrated complete resolution of the lesion with residual calcification in the liver.
Case 73 - Torsion of the appendix testis
- from Section 7 - Endocrine - reproductive imaging
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- By Rakhee Gawande, Stanford University, Heike E. Daldrup-Link, Lucile Packard Children's Hospital, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 303-306
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Summary
Imaging description
A longitudinal ultrasound of the right scrotum (Fig. 73.1) in a 13-year-old boy with gradual onset of testicular pain demonstrates a well-defined oval hypoechoic avascular structure located in the groove between the testis and epididymis. There was mild hyperemia of the testis and epididymis, which were otherwise normal in appearance. A moderate hydrocele and scrotal wall edema was also noted. These findings were suggestive of torsion of the appendix testis.
Importance
Testicular appendages are remnants of embryonic mesonephric and paramesonephric ducts and consist of vascularized connective tissue. Five types of appendages have been identified. The appendix testis is a tiny structure, 1–7 mm in size, located at the upper pole of the testis, in the groove between the testis and epididymis. It has similar echogenicity to the testis and may be oval or sessile and less commonly pedunculated. It is present in 92% of patients and is the commonest appendage to undergo torsion. The appendix epididymis is located at the head of the epididymis, is commonly pedunculated, and is less frequently seen on ultrasound. The other appendages are difficult to identify on ultrasound imaging.
Case 96 - Osteochondritis dissecans of the capitellum
- from Section 9 - Musculoskeletal imaging
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- By Rakhee Gawande, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
- Published online:
- 05 June 2014
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- 24 April 2014, pp 387-388
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Summary
Imaging description
AP and lateral elbow radiographs (Fig. 96.1) of an 11-year-old girl with elbow pain demonstrated a small subarticular lucency in the lateral capitellum with surrounding sclerosis and subtle flattening of the capitellum. Based on these imaging findings a diagnosis of osteochondritis dissecans (OCD) of the capitellum was made.
Radiographs are the most common initial modality for screening patients with suspected OCD. In the elbow, supplemental views such as 45 degrees flexion or oblique views may help to demonstrate the lesion. Elbow radiographs may be normal in the early stages of OCD of the capitellum. With disease progression, flattening of the capitellum is noted with focal rarefaction and non-displaced subchondral fragmentation. In late stages, a focal defect of the articular surface may be noted, with presence of a loose body.
A radiographic classification of OCD has been described based on AP radiographs. A grade I lesion demonstrates cystic change in the lateral or middle capitellum, a grade II lesion shows a gap between the lesion and the underlying subchondral bone, and a grade III lesion is characterized by the presence of a loose body.
Case 62 - Nephroblastomatosis
- from Section 6 - Urinary imaging
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- By Gregory Cheeney, Stanford University, Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University, Heike E. Daldrup-Link, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
- Published online:
- 05 June 2014
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- 24 April 2014, pp 260-263
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Summary
Imaging description
A four-year-old boy presented with large palpable flank masses bilaterally. An ultrasound (not shown) revealed bilaterally enlarged kidneys with homogeneous low echogenicity, resembling renal cortex. A contrast-enhanced CT was performed which showed a thick rim of non-enhancing, homogeneous soft tissue, surrounding centrally enhancing renal parenchyma (Fig. 62.1). Bilateral homogeneous non-enhancing soft tissue surrounding the renal parenchyma is a typical finding in diffuse bilateral nephroblastomatosis. Another patient with a similar presentation is shown in Figure 62.2. In this case, axial contrast-enhanced CT images demonstrate multiple lenticular, uniform, non-enhancing lesions in the renal cortex (Fig. 62.2), consistent with the more common multifocal type of nephroblastomatosis. On CT, focal nephroblastomatosis appears as low attenuation, subcapsular, or intraparenchymal lesions with poor enhancement relative to that of adjacent normal renal parenchyma. On MRI, focal nephroblastomatosis demonstrates low to intermediate signal intensity foci on both T1- and T2-weighted sequences. Both types of nephroblastomatosis typically resemble the normal renal cortex with regard to their echogenicity on ultrasound, and density and signal intensity on unenhanced CT and MRI. After contrast media injection, however, the lesions typically show no or only minimal enhancement. Contrast administration is helpful in differentiating nephroblastomatosis from Wilms’ tumor. The latter is typically inhomogeneous and enhances after contrast media administration.
Case 17 - Lymphoma: pulmonary manifestations
- from Section 2 - Thoracic imaging
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 56-61
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Summary
Imaging description
A 16-year-old girl presented with a two-month history of night sweats, weight loss, and cough. A radiograph of the chest revealed nodules in the right lung and mediastinal widening suggestive of lymphadenopathy (Fig. 17.1a, b). CT scan of the chest (Fig. 17.1c, d) demonstrated multiple nodules of varying sizes in both lungs. Most of the nodules demonstrated air bronchograms. In addition a pleural-based nodule was noted in the right upper lobe. Multiple enlarged mediastinal and hilar lymph nodes were present. The lung nodules and the mediastinal and hilar lymph nodes demonstrated increased FDG uptake on PET/CT (Fig. 17.1e, f). A lymph node biopsy confirmed the diagnosis of Hodgkin’s lymphoma, nodular sclerosis.
Importance
Pulmonary parenchymal involvement in children is slightly more common with Hodgkin’s disease (HD) than non-Hodgkin’s lymphoma (NHL). It is seen in HD in approximately 12% of patients, most at the time of diagnosis and usually associated with mediastinal or hilar lymphadenopathy. Pulmonary involvement in NHL is seen in around 10% of cases and may occur without the presence of associated lymphadenopathy. The mechanism of spread of the disease to the lungs is typically by hematogenous or lymphangitic channels and less commonly by direct or endobronchial spread. On CT imaging, different patterns of involvement can be seen. A nodular pattern is characterized by the presence of single or multiple nodules, which may have irregular borders, air bronchograms, or cavitation (Figs. 17.1 and 17.2). Subpleural nodules or masses can also occur (Fig. 17.1). Lymphoma may also produce a pattern of lobar or segmental consolidation that can be confused with pneumonia (Fig. 17.3).
Case 16 - Opsoclonus–myoclonus due to underlying ganglioneuroblastoma
- from Section 2 - Thoracic imaging
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 53-55
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Summary
Imaging description
A 19-month-old child presented with symptoms of difficulty in walking, abnormal eye and limb movements, and irritability. MRI of the brain for suspected cerebellar ataxia was unremarkable (Fig. 16.1a, b). CT scan of the neck and chest revealed a small left paraspinal soft tissue lesion at the level of T1 and T2 (Fig. 16.1c–e) and a diagnosis of neuroblastoma-associated opsoclonus–myoclonus syndrome (OMS) was suggested. Metaiodobenzylguanidine scintigraphy (MIBG) scan was negative (Fig. 16.1f). Excision biopsy of the mass confirmed the diagnosis of a ganglioneuroblastoma.
Importance
OMS, also known as dancing eye syndrome, is a rare disorder that presents in early childhood with involuntary rapid eye movements, myoclonic limb jerking, ataxia, and behavioral changes. It can be idiopathic, postinfectious, or a paraneoplastic manifestation of neuroblastoma. OMS is associated with neuroblastoma in 40% of patients and occurs between the ages of six months and three years in these patients. Conversely, only about 2% of patients with neuroblastoma present with OMS. In children, OMS may occur as a paraneoplastic manifestation of all neural crest tumors, including neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The pathogenesis of OMS is thought to be immune-mediated, with a cross-reactive autoimmunity between neuroblastoma cells and the central nervous system. Neuroblastomas associated with OMS tend to be low grade, thoracic in location, and have a more favorable outcome than non-OMS neuroblastomas. Neuroblastomas associated with OMS are usually very small in size and present a diagnostic challenge as they are less metabolically active and urinary catecholamines or MIBG scans can be negative. CT and/or MRI of the neck, chest, abdomen, and pelvis are the most sensitive studies to detect these occult neuroblastomas (Fig. 16.1).
Case 44 - Ruptured appendicitis mimicking an intussusception
- from Section 5 - Gastrointestinal imaging
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- By Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
- Published online:
- 05 June 2014
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- 24 April 2014, pp 196-200
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Summary
Imaging description
A six-year-old child presented to the ER with abdominal pain. A ultrasound (US) study (Fig. 44.1a, b) demonstrated a lesion in the right lower quadrant with a pattern of alternating sonolucent and hyperechoic layers, giving an appearance of a bowel loop-within-loop, suggesting intussusception.
A plain radiograph of the abdomen (Fig. 44.1c) revealed blurring of the right flank fat plane, medial displacement of ascending colon gas, and subtle scoliosis of the spine, indicating a right-sided inflammatory process or mass. A contrast enema was obtained to reduce the intussusception diagnosed on US (Fig. 44.1d). The study revealed a possible filling defect at the hepatic flexure which rapidly disappeared (? reduced intussusception) and normal reflux of contrast was noted into the ileum (Fig. 44.1d). The child continued to have abdominal pain, fever, and high white blood cell count. Repeat US the following morning demonstrated a similar, slightly more complex layered pattern and marked surrounding echogenic inflammation was noted (Fig. 44.1e). Taking into account the clinical and laboratory findings as well as the child’s age (old for typical idiopathic intussusception), perforated appendicitis with phlegmon/abscess was suggested as a more likely diagnosis. CT examination confirmed ruptured acute appendicitis with an appendicolith and periappendiceal fluid collection/abscess (Fig. 44.1f, g).
Case 12 - Pleuropulmonary blastoma
- from Section 2 - Thoracic imaging
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- By Jordan Caplan, Stanford University, Rakhee Gawande, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 36-39
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Summary
Imaging description
A three-year-old previously healthy male presented with 1 week of progressive respiratory distress, fever, and lethargy. He was being treated for a viral upper respiratory tract infection.
The frontal chest radiograph (Fig. 12.1a) shows complete opacification of the left hemithorax with marked tracheal and mediastinal shift to the right and compressive atelectasis of the right lung. The differential diagnosis includes a large left pleural effusion, which could be related to infection/pneumonia/empyema, versus a large mediastinal or intrapulmonary mass such as lymphangioma, teratoma, neuroblastoma, pleuropulmonary blastoma, or lymphoma.
Axial contrast-enhanced CT (Fig. 12.1b) and coronal reformat (Fig. 12.1c) show a large hypodense heterogeneous largely intrapulmonary mass with irregular enhancing areas, causing mediastinal shift and inversion of the left hemidiaphragm. It was uncertain whether the mass was partially cystic or if there was some component of pleural effusion. The mass did not extend behind the aorta or encase the mediastinal vessels, making a diagnosis of a primary mediastinal mass such as neuroblastoma or lymphoma less likely.
Ultrasound of the chest (longitudinal view) revealed a largely solid lesion with heterogeneous echogenicity with some cystic components (Fig. 12.1d). There was no evidence of pleural effusion.
Based on imaging this was thought to be a large cystic/solid intrapulmonary neoplasm with pleuropulmonary blastoma the most likely diagnosis in a child of this age. Subsequent biopsy of the lesion confirmed a type II pleuropulmonary blastoma.
Case 65 - Ureteropelvic junction obstruction
- from Section 6 - Urinary imaging
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- By Rakhee Gawande, Stanford University, Heike E. Daldrup-Link, Lucile Packard Children's Hospital, Stanford University, Beverley Newman, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 276-280
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Summary
Imaging description
A nine-year-old boy presented with intermittent episodic left flank pain consistent with Dietl’s crisis (=sudden attack of acute lumbar and abdominal pain accompanied by nausea and vomiting, caused by distension of the renal pelvis due to kinking of the ureter or other obstruction of urine flow from the kidney to the ureter). An initial ultrasound showed moderate left renal pelvocaliectasis (Fig. 65.1a). A follow-up study one month later showed mild dilatation of the left renal pelvis (Fig. 65.1b) and a follow-up ultrasound six months later when the child was again acutely symptomatic showed moderate hydronephrosis with marked dilatation of the renal pelvis (Fig. 65.1c). A review of serial ultrasounds of the kidneys revealed fluctuating mild to moderate hydronephrosis of the left kidney over several years. The imaging appearance was strongly suggestive of intermittent ureteropelvic junction (UPJ) obstruction.
Importance
UPJ obstruction is the most common cause of hydronephrosis in children and accounts for approximately 64% of cases. It is defined as a partial or total obstruction at the point where the renal pelvis narrows to form the ureter. It is found more commonly in boys and frequently involves the left kidney. It can be bilateral in 10–40% of cases. Ipsilateral vesicoureteral reflux is seen in about 10% of patients with UPJ obstruction. Various congenital renal anomalies may be associated with UPJ obstruction, including contralateral multicystic dysplastic kidney (MCDK), renal agenesis, duplicated renal collecting system, horseshoe kidney (Fig. 65.2), and ectopic kidney. Other associated congenital anomalies include imperforate anus, congenital heart disease, esophageal atresia, and VATER syndrome.
Case 70 - Adrenal hemorrhage
- from Section 7 - Endocrine - reproductive imaging
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- By Rakhee Gawande, Stanford University, Rosalinda Castaneda, Stanford University Molecular Imaging, Heike E. Daldrup-Link, Lucile Packard Children's Hospital, Stanford University
- Edited by Heike E. Daldrup-Link, Beverley Newman
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- Book:
- Pearls and Pitfalls in Pediatric Imaging
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- 05 June 2014
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- 24 April 2014, pp 291-293
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Summary
Imaging description
A seven-day-old neonate presented with a history of birth asphyxia and abdominal distension. A routine abdominal-renal ultrasound demonstrated incidental heterogeneous lesions in the bilateral suprarenal regions (Fig. 70.1). The lesions were relatively well defined, with mild compression of the upper poles of both kidneys. A review of prenatal ultrasound images (not shown) did not reveal any evidence for adrenal masses. The finding is most consistent with bilateral adrenal hemorrhages. A neuroblastoma is much less likely due to the bilateral nature of the lesions and the history of an acute occurrence.
Importance
The adrenal gland in neonates is large and susceptible to hemorrhage due to rapid regression of the fetal cortex during the neonatal period and markedly engorged vascular channels in the primitive cortex. Adrenal hemorrhage is postulated to occur either due to ischemic hemorrhagic infarction because of reflex vascular redistribution in response to asphyxia or rupture of engorged veins related to increased abdominal pressure and inferior vena cava (IVC) compression. A significant hemorrhage can result in hypovolemic shock and may present as a life-threatening emergency. In neonates, the development of adrenal insufficiency is rare.