computed tomographic evaluation of the retroperitoneum in infants and children

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JOURNAL OF COMPUTED TOMOGRAPHY 1983;7:63-75 COMPUTED TOMOGRAPHIC EVALUATION OF THE RETROPERITONEUM IN INFANTS AND CHILDNEN PHILIP STANLEY With many retroperitoneal diseases in infancy and childhood, computed tomography will give infor- mation unavailable by any other single imaging technique. This maxim applies particularly to the adrenal, where the bercolating nature of neuroblas- toma with its propensity toward spinal involvement is exquisitely shown with computed tomography. Renal neoplasms and trauma can be illustrated ef- fectively with computed tomography, as can pan- creatic disease and spinal and paraspinal inflam- matory and neoplastic disease. In addition to diagnostic studies, computed tomography may be used as guidance for invasive procedures. KEY WORDS: Computed tomography; Adrenal; Kidney; Spine; Tumor; Inflammation; Trauma INTRODUCTION It is for suspected disease in the retroperitoneum that abdominal computed tomography is most fre- quently performed at Childrens Hospital of Los An- geles. The retroperitoneum can be conveniently de- fined as the space lying between the posterior parietal peritoneum and the fascia transversalis. This area can be further divided into three spaces whose anatomical boundaries are often visible on axial tomography. The anterior pararenal space lies between the posterior parietal peritoneum and the anterior renal fascia (Zuckerkandl’s fascia]. It con- The Department of Radiology, Childrens Hospital of Los An- geles, Los Angeles, California Address reprint requests to: Philip Stanley, M.D., Department of Radiology, Childrens Hospital of Los Angeles, P. 0. Box 54700, Los Angeles, California 90054. Received August 5, 1982; accepted August 5, 1982. C 1983 by Elsevier Science Publishing Co., Inc. 52 Vanderbilt Ave., New York, NY 10017 0149-936x/83/010063-13$3.00 tains the pancreas and retroperitoneal portions of the descending duodenum and asuending and de- scending colon. The perirenal space is between the anterior and posterior renal fascia, containing as it does the kidneys and adrenals. If the space on ei- ther side is extended to the midline, the major vas- cular structures are included. The posterior para- renal space is between the posterior renal fascia (Gerota’s fascia] and the fascia transversalis. It con- tains no important organs. On account of the anatomic orientation of the ret- roperitoneal organs and separating fascial planes and vascular structures, this region is particularly suitable for display by axial computed tomography (CT) - Neoplastic disease of the kidney or adrenal is the most common indication for CT, fiollowed by sus- pected malignant lymphadenopatby. Trauma and inflammatory disease account for a lesser number of examinations. The following sections describe the technique for demonstrating retroperitoneal disease, along with various examples of pathology contained therein. SEDATION Patient movement causes severe degradation of the computed tomographic image even with an ultrafast machine. In children under 6 years of age, sedation is given unless there is a proven higtory of quietness and cooperation during the examination. Nothing is given orally for 4 hours before the examination ex- cept for essential medicines which may be taken with a small amount of water. For children under 2 years of age, chloral hydrate is given orally 30-45 minutes before the examination; this can be fol- lowed by orally administered contrast medium. (The dose is 50 mg/kg of chloral hydrate with a maximum of 2 g. For a particularly anxious child, this may be increased to 75 mg/kg.) We have had no

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Page 1: Computed tomographic evaluation of the retroperitoneum in infants and children

JOURNAL OF COMPUTED TOMOGRAPHY 1983;7:63-75

COMPUTED TOMOGRAPHIC EVALUATION OF THE RETROPERITONEUM IN INFANTS AND CHILDNEN

PHILIP STANLEY

With many retroperitoneal diseases in infancy and childhood, computed tomography will give infor- mation unavailable by any other single imaging technique. This maxim applies particularly to the adrenal, where the bercolating nature of neuroblas- toma with its propensity toward spinal involvement is exquisitely shown with computed tomography. Renal neoplasms and trauma can be illustrated ef- fectively with computed tomography, as can pan- creatic disease and spinal and paraspinal inflam- matory and neoplastic disease. In addition to diagnostic studies, computed tomography may be used as guidance for invasive procedures.

KEY WORDS:

Computed tomography; Adrenal; Kidney; Spine; Tumor; Inflammation; Trauma

INTRODUCTION

It is for suspected disease in the retroperitoneum that abdominal computed tomography is most fre- quently performed at Childrens Hospital of Los An- geles. The retroperitoneum can be conveniently de- fined as the space lying between the posterior parietal peritoneum and the fascia transversalis. This area can be further divided into three spaces whose anatomical boundaries are often visible on axial tomography. The anterior pararenal space lies between the posterior parietal peritoneum and the anterior renal fascia (Zuckerkandl’s fascia]. It con-

The Department of Radiology, Childrens Hospital of Los An- geles, Los Angeles, California

Address reprint requests to: Philip Stanley, M.D., Department of Radiology, Childrens Hospital of Los Angeles, P. 0. Box 54700, Los Angeles, California 90054.

Received August 5, 1982; accepted August 5, 1982. C 1983 by Elsevier Science Publishing Co., Inc. 52 Vanderbilt Ave., New York, NY 10017 0149-936x/83/010063-13$3.00

tains the pancreas and retroperitoneal portions of the descending duodenum and asuending and de- scending colon. The perirenal space is between the anterior and posterior renal fascia, containing as it does the kidneys and adrenals. If the space on ei- ther side is extended to the midline, the major vas- cular structures are included. The posterior para- renal space is between the posterior renal fascia (Gerota’s fascia] and the fascia transversalis. It con- tains no important organs.

On account of the anatomic orientation of the ret- roperitoneal organs and separating fascial planes and vascular structures, this region is particularly suitable for display by axial computed tomography (CT) -

Neoplastic disease of the kidney or adrenal is the most common indication for CT, fiollowed by sus- pected malignant lymphadenopatby. Trauma and inflammatory disease account for a lesser number of examinations.

The following sections describe the technique for demonstrating retroperitoneal disease, along with various examples of pathology contained therein.

SEDATION

Patient movement causes severe degradation of the computed tomographic image even with an ultrafast machine. In children under 6 years of age, sedation is given unless there is a proven higtory of quietness and cooperation during the examination. Nothing is given orally for 4 hours before the examination ex- cept for essential medicines which may be taken with a small amount of water. For children under 2 years of age, chloral hydrate is given orally 30-45 minutes before the examination; this can be fol- lowed by orally administered contrast medium. (The dose is 50 mg/kg of chloral hydrate with a maximum of 2 g. For a particularly anxious child, this may be increased to 75 mg/kg.) We have had no

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64 STANLEY THE JOURNAL OF COMPUTED TOMOGRAPHY VOL. 7. NO. 1

problems with aspiration of regurgitated gastric contents with this dosage. In older children, be- tween 2 and 6 years of age, demerol-phenergan- thorazine mixture (DPT) is given by intramuscular injection. The dose of the mixture (demerol 25 mg/ ml, phenergan 6.25 mg/ml, and thorazine 6.25 mg/ ml) is 1 ml/lOkg with a maximum of 2 ml. This should not be repeated. If the child becomes very drowsy and swallowing is impaired, then oral con- trast medium is not given. When a child is under heavy sedation, an electrocardiographic monitor is used, and the child is continuously observed either directly through a lead glass observation panel or through closed circuit television. For a very active child on whom it is essential to obtain a scan, intra- venous Valium (0.2 mg/kg maximum dosage) is carefully and patiently titrated intravenously. The dosage of sedation and its effectiveness is recorded for each patient in a book so that it may be modified if necessary on subsequent visits. It should be re- membered that this is a diagnostic examination that should have low morbidity and no mortality. Do not add to the morbidity by overenthusiastic or hurried sedation. Everyone who works in the CT room should have experience in basic resuscitative pro- cedures.

hour before the examination. If a pelvic mass is sus- petted and the oral contrast medium has not reached the rectum, then dilute contrast solution, of the same strength as the oral agent, can be given rectally in the form of a small enema.

INTRAVENOUS CONTRAST MEDIUM

Unless the patient is allergic to the agent, intrave- nous contrast medium is always given; for some ex- aminations, particularly if liver metastases are not suspected, only a postenhancement scan is re- quired. This is especially so with follow-up exami- nations, e.g., neuroblastomas, lymphadenopathies, and abscesses. Intravenous contrast medium will opacify the kidneys, ureters, and bladder. If given as a bolus, the major vascular structures (e.g., aorta and main branches and inferior vena cava) will be opacified and enhancement of hypervascular tu- mors will be apparent. In addition, contrast me- dium will increase accentuation between the tumor and host tissue (e.g., Wilms’ tumor). It should be noted that hepatic metastases may be hypodense on preenhancement studies but isodense after the use of contrast medium. It is for this reason that both pre- and postenhancement studies are performed in patients with suspected liver metastatic disease. Two milliliters of 60% contrast per kilogram of body weight given intravenously with a maximum of 100 ml. If the child is an inpatient, an intrave- nous line with a 1% or 21-gauge butterfly needle is placed on the ward before coming to the CT area so that the agitation of venepuncture does not disturb the child during scanning. For outpatients, the pro- cedure nurse sets up the intravenous line before the scout film is taken. If vascular structures and tumor vascularity are to be evaluated, then half the con- trast medium is given as a bolus, the scanning is started, and the remainder is infused slowly. It is inadvisable to give contrast medium into a lower limb vein, as the high level of medium in the infe- rior vena cava causes excessive scatter, which de- grades the image.

ORAL CONTRAST AGENTS

If at all possible, oral contrast medium is always given before abdominal scanning. Nonopacified loops of bowel may be confused with masses, lymphadenopathy, and normal structures. The se- cret to satisfactory bowel opacification is to give plenty of dilute contrast medium well ahead of the examination. The contrast medium is a 3% solution of Gastrografin or Hypaque diluted and made more palatable with orange juice or punch. It is an error to make the solution too strong. High-density scatter streaking will obscure the adjacent structures. For this reason also it is important that the bowel is clear of barium residue before the scan is per- formed. (Surgical clips can be equally as destructive to the scan.) If there is excessive scatter from a con- trast fluid level-air interface in the stomach, then this artifact can be reduced by scanning in a right lateral decubitus position (see below).

For children under 1 year of age, 50-60 ml of contrast medium is given, increasing to 250 ml by 6 years, and up to 600 ml by 12 years of age and over. For pathology within the upper abdomen, the con- trast medium is given 1 hour before the scan; for the lower abdomen, 2-3 hours beforehand, and for sus- pected pelvic disease, the contrast medium is given 3-4 hours before the study with a repeat dose an

INTRATHECAL CONTRAST

In those patients in whom the plain films or other imaging studies suggest paravertebral pathology, or when the disease has a known propensity towards vertebral or intraspinal spread, the examination is performed with intrathecal metrizamide. This is of- ten combined with examination of the cerebrospi- nal fluid for malignant cells, which is part of the protocol before starting chemotherapy with many tumors, especially neuroblastomas. As a premedi-

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FEBRUARY 1983 RETROPERITONEUM 65

cation not only to sedate the patient, but also to pre- vent seizures that have occasionally been reported with metrizamide, phenobarbitone is given before the study (5 mg/kg to a maximum of 130 mg). In older children, the phenobarbitone is given intra- venously. For infants, it may be given as an elixir. A limited myelogram is performed through the level of suspected involvement by using metrizamide at a concentration of 220 mg I/ml. In the newborn, l- 1.5 ml of metrizamide is given, increasing to 12 ml for children 10 years old or older. The patient is scanned 2-3 hours later when the contrast medium is more dilute. An alternative is to use a smaller volume of more dilute metrizamide (180 mg I/ml) with immediate scanning without a preceding rou- tine myelogram.

SCANNING TECHNIQUE The transverse or axial plane is used routinely and, if necessary, sagittal and coronal reconstruction can be performed later from the computed data. With a small child, it is possible to perform direct sagittal scans with a wide gantry; if the child can sit up in the gantry; direct coronal scans may be performed. Images in the sagittal and coronal planes, although not routinely obtained for diagnosis, are most useful to the radiation therapist in portal planning and iso- dose determination.

The patient is restricted if necessary by a wrap- around blanket of a ‘Velcro’ tape harness and placed in the scanner. Most patients are scanned in a su- pine position, but if there is suspected lymphade- nopathy, the prone position may be used to com- press the bowel against the spine, thus accentuating the retroperitoneal nodes. A right lateral decubitus position is occasionally used to minimize the con- trast flare from the stomach. The operator selects the field of view and radiographic factors. If coop- erative, most children can suspend respiration for the duration of the scan, which varies between 2 and 9.6 seconds, depending on the scanner. With sedation, quiet respiration during the scan causes very little image degradation.

The normal technique at this institution is to per- form l-cm thickness slices at l-cm intervals in chil- dren below 10 years of age and 1.5-cm intervals above this age, depending upon the nature of the underlying pathology. With suspected adrenal hy- perplasia, 5-mm sections are taken at 5-mm inter- vals. Not only is the retroperitoneal area examined, but with tumors, the liver and chest are included.

Window dimensions and display format are im- portant when examining the retroperitoneum. The usual viewing window width is 300-500 units, the

lower value being used when there are limited con- trast differences. At this level, however, the image tends to be grainy. With more inherent contrast, high values are used; for spinal viewing, widths of 1000 units are preferred, particularly if metrizamide has been introduced. Often it is visually more ac- ceptable to reverse the black and white display with spinal viewing. The window level is adjusted on the monitor to give the right degree of penetration.

PATHOLOGY Kidney Computed tomography is used extensively in the diagnosis of pediatric renal disease. The most fre- quent indication is demonstration of renal tumors, followed by trauma, inflammatory disease, and oc- casionally, with renal calcification, renal obstruc- tion, and cystic disease. The complications associ- ated with renal transplantation may also be visual- ized by this modality. The resolution of the scanner is insufficient at the present time ‘to allow demon- stration of discrete vascular lesions by computed to- mography.

Tumors

wnMs’.-The most frequent renal tumor seen in the pediatric population is Wilms’ tumor. Com- puted tomography confirms the presence of the tu- mor and shows its extent within the kidney, as well as any involvement of adjacent structures. Spread into the renal vein and inferior vena cava is dem- onstrated along with nodal and hgpatic metastases. A second mass within the opposite kidney will be apparent, and inclusion of the chest is the best method to demonstrate pulmonary metastases. An- atomic variants, e.g., horseshoe kidney-which may not be visualized by other imaging techniques but have an important bearing on therapy-will also be shown by computed tomography:. Not only is CT the best way initially to demonstrate the size and extent of a Wilms’ tumor, but it is most useful for follow-up studies, demonstrating shrinkage by che- motherapy of an initially unresectable mass, and for following up the renal fossa to detect recurrence af- ter surgery.

The introduction of CT has altered the approach to the diagnosis of renal masses. If a solid renal mass is discovered on ultrasonography, a computed tomogram is performed, both before and after intra- venous contrast medium enhancement. At the end of the examination, a single film of the abdomen is taken, the equivalent of an intravenous urogram,

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66 STANLEY 1 ‘HE JOURNAL OF COMPUTED TOMOGRAPHY VOL. 7, NO. 1

FIGURE 1. There is a large intrarenal tumor of dimin- ished density on the right side compared with the en- hancing kidney remnant, which is displaced medially. Histology revealed a Wilms’ tumor.

which has now been almost completely replaced by computed tomography in the initial diagnosis of renal tumors.

On a CT scan, a Wilms’ tumor is of lower density compared with normal kidney, and the difference is accentuated by intravenous contrast medium en- hancement (Figure 1). Large tumors, especially if treated with chemotherapy or radiation, often con- tain areas of further diminished density within the tumor indicative of tumor necrosis [l] (Figure 2). Calcification within the tumor may occur, but it is rare [2], The tumor is usually well defined, having a pseudocapsule. As to be expected, there is defor-

FIGURE 2. There are rounded areas of diminished den- sity within a large lower polar left Wilms’ tumor. These areas were found to represent necrosis at surgery.

mity and obstruction of the underlying collecting system. Blood clots may be visualized within the renal pelvis. There may be no visualization of the collecting system of the kidney because the venous drainage is blocked by a tumor. In this situation, there is enlargement of the renal vein and inferior vena cava with a large filling defect of the same density as the primary renal tumor (Figure 3). Oc- casionally, this filling defect will be surrounded by contrast medium if the cava is incompletely blocked. With complete obstruction paravertebral and abdominal wall collaterals may be visible. A

FIGURE 3. [A) There is enlargement of the inferior vena cava (I) with tumor completely replacing the lumen. A Wilms’ tumor is occupying much of the right kidney, with a large anterior extension (T). (B) After chemother- apy, the inferior vena cava is of normal caliber and does not contain tumor. There is a small residual tumor “scar” on the anterior margin of the right kidney. The lower pole of the spleen is partially obscuring the left kidney. Fur- ther sections showed no abnormality in this kidney.

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bolus of intravenous contrast medium will often vi- sualize the renal, celiac, and superior mesenteric ar- teries and the aorta. These may all be displaced by an extensive tumor.

Nodal involvement is difficult to assess in chil- dren. If extensive, it should be visible on a CT scan, but subtle enlargement can be missed. A very large tumor will displace the stomach and bowel away if arising from the left kidney; on the right side, a large tumor will extend into the right lobe of the liver. Several patients have been referred to this in- stitution with an incorrect diagnosis of a primary hepatic tumor in the right lobe whereas lower sec- tions have demonstrated that the tumor arose from the kidney (Figure 4). Also, a Wilms’ tumor may ex-

FIGURE 4. (A) This section through the upper liver dis- closes a large mass with a necrotic center initially diag- nosed as a primary liver tumor at an outside institution. (B) Lower sections with intravenous contrast show defor- mity of the right collecting system with an intrarenal mass extending anteriorly with a large extrarenal compo- nent. This was a large Wilms’ tumor extending high into the right lobe of the liver.

tend into the chest via the retroperitoneal space. The origin of these extensive tumors may be diffi- cult to establish at times, although there is often lo- calized calyceal obstruction or displacement typical of an intrarenal mass (Figure 5). A tumor this exten- sive cannot be completely removed: by surgery, and it may be difficult with other large but not so exten- sive tumors to assess operability. A’ large tumor ex- tending into the liver may have a thin, but surgi- cally important plane of separation; a large left- sided tumor, even crossing over the midline, may also be successfully removed. Involvement of the inferior vena cava and right atrium’calls for special surgical techniques for complete removal of the tu- mor. Very rarely, the primary tumor may extend into the spinal canal destroying bone, obliterating the epidural fat, and displacing the spinal cord. In our institution, this is more common with meta- static Wilms’ tumor in the thoracic spine.

A tumor in the opposite kidney usually has the same density characteristics as thesother kidney tu- mor, but not always (Figure 6). Artieriography is of- ten employed in this situation if l~ocal surgical ex- cision of the tumors is to be undertaken.

As part of the initial evaluation, the chest and liver are scanned although in our experience pul- monary metastases are uncommon, and liver metas- tases very rare at the time of presentation.

LYMPHOMA. Some experience has been gained with CT in pediatric renal lymphoma [l]. The dis- ease is usually bilateral with diffuse involvement and ill-defined margins (Figure 7). In addition, there may be enlargement of adjacent lymph nodes and hilar lymphadenopathy. It should be noted that, however, based on CT alone it may be difficult to separate a discrete lymphomatous mass from a Wilms’ tumor.

ANGIOMYOLIPOMA. Renal involvement by tuberous sclerosis may be manifested in two ways: the pres- ence of discrete tumors (angiomyolipomas) and cys- tic disease, both of which may be detected by CT. Angiomyolipomas are seen as masses with lucent centers of fatty density with multiple flecks of cal- cium [3]; the cysts are often small; lying within the renal cortex or adjacent to it. It should be noted that small angiomyolipomas may be missed on CT. In a patient with apparent unilateral disease and hema- turia, in whom surgery is contemplated, the other kidney should be studied arteriographically.

OTHER RENAL TUMORS AND TUMdRLIKE CONDITIONS.

Metastatic disease to the kidney is rare but may

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FIGURE 5. (A) There is a large pleural based mass en- croaching on the right lung displacing the mediastinum to the left. (B) A high nonenhanced abdominal scan shows right sided hydronephrosis with an anterior intra- renal mass (M). (C) A lower section reveals a lower pole mass with some areas of tumor necrosis. Surgery con- firmed the presence of an extensive Wilms’ tumor.

FIGURE 6. There are bilateral Wilms’ tumors seen on this scan performed after contrast medium enhancement. The tumor on the right side is occupying the midsection and that on the left, the posterior half. There are areas of necrosis on the left after chemotherapy.

simulate a primary renal tumor although, in one pa- tient with metastatic osteogenic sarcoma, the meta- static deposit was of greater density than the host kidney (Figure 8). Nephroblastomatosis will show bilaterally enlarged kidneys on CT scans with slightly lobulated outlines and a nonenhancing pe- ripheral rind of tissue.

Trauma. Computed tomography has an impor- tant place in the diagnosis of renal trauma. For mi- nor trauma with microscopic hematuria, a limited intravenous urogram is sufficient. However, with major and nonpenetrating trauma-especially if there is suspected liver and spleen damage-CT is the method of choice for initial evaluation once the patient’s condition has been stabilized. In this situ- ation it is unnecessary to perform a preenhance- ment study. A single plain film of the abdomen after the CT scan with contrast-medium enhancement is helpful not only in complementing the scan findings, but also in demonstrating the ureters in full length with a view of the bladder. (With improved “scout” view, this latter film may not be necessary.)

The most serious consequences of trauma are avulsion of the renal pedicle or complete thrombo- sis of the renal artery. When either of these catastro- phes occur, the kidney will be visible, but it will not enhance. If there is very poor contrast-medium enhancement with filling of spidery calyces but no other evidence of renal damage, then a dissection of the renal artery is suspected and arteriography is undertaken.

The important renal trauma lesion of parenchy-

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FIGURE 7. (A) The scan performed after contrast-me- dium enhancement reveals a large tumor mass of dimin- ished density replacing and expanding the upper pole of the right kidney. In addition, there is a diffuse involve- ment of the left kidney. This is an example of bilateral renal lymphoma. (B) A scan after contrast-medium en- hancement at the level of the renal hilus showing an ill- defined mass of reduced density within the left kidney. The patient has disseminated lymphoma. (Not the same patient as in A.)

ma1 damage, which may involve the collecting sys- tem, can easily be shown with CT [4]. A break in the renal substance is readily demonstrated along with a subcapsular collection (Figure 9). Maceration of the kidney will be shown as fragments of kidney split apart with blood and contrast-medium-laden urine interspersed. A urinoma will be obvious as a collection of fluid outside the collecting system the density of which will increase with subsequent scans.

In addition to showing the extent of the trauma to the affected kidney, CT will demonstrate the other kidney as well as the liver and spleen.

Inflammatory disease. Computed tomography is an excellent modality for demonstrating renal car- buncles and perirenal inflammatory disease, but it has little or no part in the diagnosis of chronic pye- lonephritis although characteristic images have been reported. [5] With a renal abslcess, there is an area of diminished density that may be moderately well defined, and there may be some displacement of the collecting system. Peripheral enhancement with an irregular inner margin may be seen after a bolus of intravenous contrast medium. Usually there are characteristic clinical feaitures to support the radiologic diagnosis of a renal abscess. Com- puted tomography may be used ati a guideline for percutaneous drainage of an abscess.

FIGURE 8. (A) On this scan without contrast-medium enhancement, there is a tumor of increased density oc- cupying the posterolateral aspect of the right kidney. (B) This subscapular mass is now highlighted by the ad- jacent, enhanced kidney. This tumor was metastatic os- teogenic sarcoma.

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Adrenal

Computed tomography is the imaging method par excellence for visualizing adrenal disease. Com- puted tomography is used most frequently to diag- nose adrenal tumors, but hyperplasia, abscesses, and hematomas may all be evaluated with this mo- dality.

FIGURE 9. A scan after contrast-medium enhancement in this trauma victim reveals a renal parenchymal break anteriorly (asterisk) with a perirenal collection of fluid (arrows). Conservative therapy was successfully pursued.

Renal Calcification. The presence of calcifica- tion and its exact site within or adjacent to the kid- ney can be easily demonstrated by a few judiciously placed CT sections [5].

Renal Obstruction and Cystic Disease. Com- puted tomography has a very limited role in the pri- mary diagnosis of obstructive hydronephrosis, al- though obstruction may be seen on scans performed for another purpose, e.g., a large pelvic mass. Ultra- sonography and radionuclide renograms are the most helpful studies with uncomplicated severe renal obstruction.

Although characteristic features of infantile poly- cystic disease have been described with CT [5], in- travenous urography and ultrasound are equally as efficacious, and they are easier and cheaper to per- form. With multicystic disease, ultrasonography and renal radionuclide scans are simpler and do not require an injection of intravenous contrast medium to make the diagnosis.

Kidney Transplant. Computerized tomography has no part in the routine investigation of live, re- lated, potential renal donors, but may be useful in demonstrating the nature and extent of fluid collec- tions around a transplanted kidney. Usually the first investigation in the latter situation is ultrasonogra- phy. Computed tomography is not useful in di- agnosing rejection or renal artery stenosis, the former requiring isotope scans and the latter, angi- ography.

Tumors

NEUROBLASTOMA. The very origin and method of spread of this tumor lends itself to exquisitely di- agnostic studies with CT. The examination is mod- ified slightly, depending upon the patient’s age and findings on other imaging modalities. Below 6 months of age, metastatic liver involvement is to be expected along with the primary tumor. If the plain film or other imaging modalities show evidence of paravertebral disease, then metrizamide is intro- duced into the lumbar space before a scan is per- formed (see above].

Neuroblastomas have certain characteristic fea- tures on CT scans. Calcification-which is usually nodular and may be in multiple sites within the tu- mor-is visible in about 95% of patients (Figure 10). The tumor tends to invade and permeate through adjacent structures. The ipsilateral kidney is dis- placed downward and laterally, and the hilus is of- ten invaded. The aorta is frequently elevated from the spine, being surrounded by tumor and separated from the inferior vena cava. (These vascular struc-

FIGURE 10. A scan without contrast-medium enhance- ment revealing a large neuroblastoma compressing the liver and displacing the stomach anteriorly. There are areas of nodular calcification within the tumor.

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FEBRUARY 1983 RETROPERITONEUM 71

FIGURE 11. A scan, performed after a bolus injection of contrast medium shows a large neuroblastoma with a var- iegated appearance due to areas of increased vascularity intermingled with necrosis within the tumor. The aorta [arrow) is elevated by retrocrural disease. Metrizamide is present in the spinal canal.

tures will be visible on bolus contrast studies [Fig- ure 111). In addition, the celiac axis and superior mesenteric arteries may be encased by tumor. The diaphragmatic crura are often thickened and dis- placed anteriorly by retrocrural nodes (Figure 12). Neuroblastomas tend to invade the spinal canal through the intervertebral foramen. Computed to- mography with intrathecal metrizamide is the only established method of demonstrating bony destruc- tion along with extra- and intraspinal involvement in a single examination [6]. In those patients in

FIGURE 12. Partially calcified neuroblastoma is present beneath the elevated, thickened crura.

whom metrizamide has not been used, it may still be possible to detect intraspinal involvement by showing displacement or obliteration of the fat sur- rounding the spinal cord [7,8].

On preenhancement films, the noncalcified ele- ments of the tumor are less dense than the adjacent kidney. After bolus injection of intr@venous contrast medium, large tumors often have 1a variegated ap- pearance with areas of increased vascularity inter- spersed with islands of diminished density (Fig- ure 11).

Local spread to paraaortic nodeis will be shown on CT as well as invasion of the liver either by ad- jacent tumor or by multiple metastases in the youn- ger patients (Figure 13).

After removal of the primary tumor, or with treat- ment by radiation and chemotherapy, CT is an ex- cellent modality for subsequent examinations. In addition to scanning the abdomen, the chest is also included if preliminary scout f&s indicate the possibility of intrathoracic paravertebral disease.

PHEOCHROMOCYTOMA. This is a rare tumor in childhood, but it is an important, treatable cause of hypertension. At Childrens Hospit of Los Angeles, CT has changed the radiologic evaluation of chil- dren with urinary and peripheral blood evidence of a pheochromocytoma, which has yet to be localized anatomically. If the biochemical studies suggest an adrenal location, then CT is first performed. If an extraadrenal site is suggested (by high norepineph- rine levels], venous sampling is first performed to localize the tumor more closely, hollowed by a CT

FIGURE 13. Multiple hepatic metastaees are present from a calcified neuroblastoma on the left side in this s-month- old girl. In addition, there are two enlarged retrocrural nodes.

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scan of the area indicated by venous blood analysis. Arteriography is most unlikely to detect a tumor missed by CT, but is useful before surgery in dem- onstrating important arterial anatomy with large masses.

About 5% of pheochromocytomas will show areas of calcification due to old hemorrhages or necrosis [9]. There is frequently an enhancing cir- cumferential shell about the tumor [lo] (Figure 14). The tumor is most frequently in the adrenal area, but it may arise anywhere along the sympathetic chain. With multiple endocrine syndromes, type II and III, bilateral tumors are found and may be ma- lignant [9]. Venous invasion is unusual with malig- nant pheochromocytomas, but it can be shown by CT which will also demonstrate hepatic, lymph node, and lung metastases [ll].

ADRENAL CORTICAL HYPERFUNCTION. With hyper- cortisolism, CT will distinguish between a primary adrenal cause and pituitary-dependent disease. With normal or symmetric hyperplasia of the adre- nals, the hypercortisolism is due to overproduction of adrenocorticotrophic hormone by the pituitary, usually a microadenoma [9]. The pituitary should also be examined by CT in preparation for a trans- sphenoidal surgical approach. Rarely, macronodu- lar hyperplasia may be present due to pituitary hy- perfunction. A unilateral adrenal mass indicates a primary adrenal cause. A small tumor (less than 5 cm) is compatible with an adenoma; a larger mass suggests a carcinoma. Venous invasion with a car- cinoma may be seen on a CT scan.

FIGURE 14. A study, performed after contrast-medium enhancement, which discloses a rounded mass lying in- ferior to the lower pole of the left kidney. There is cir- cumferential enhancement in this surgically proven pheochromocytoma.

Hyperaldosteronism (Conn’s syndrome) is rare in children. Because an adenoma may be small and below the resolving power of CT, venous sampling and venography may be required to distinguish be- tween a solitary tumor and hyperplasia [9].

ADRENAL HRMATOMA AND ABSCESS. Adrenal hema- toma in the newborn, which may later become sec- ondarily infected, can easily be shown by CT. How- ever, it is usually investigated with intravenous urography and ultrasound, which together with the typical clinical features and normal urinary vanilyl mandelic acid levels enable the correct diagnosis to be made. The natural regression of the hema- toma can be satisfactorily documented with ultra- sound.

Pancreas Pancreatic disease, relatively common in adults, is rare in children. Even so, CT will show primary tu- mors and metastatic disease around the pancreatic head, calcific pancreatitis, fat and iron replacement, and the effects of trauma-particularly pseudocyst formation.

Primary pancreatic tumors that may be hormon- ally active are unusual in children. If large enough, the tumor will be visible on a CT scan and may con- tain calcium (Figure 15). If the tumor is hormonally active, the route of investigation follows that estab- lished for adults by Doppman et al. [9]. Metastatic disease to the lymph nodes around the pancreatic

FIGURE 15. There is a large mass in the head of the pan- creas with an area of central lucency and an adjacent nodule of calcification. This proved to be a non-p-cell ad- enoma.

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FEBRUARY 1983 RETROPERITONEIJM 73

FIGURE 16. A large mass of nodes in the region of the pancreatic head is present in this teenager with multifo- cal hepatocellular carcinoma.

head is easily demonstrated by CT (Figure 16). Dila- tation of the biliary system secondary to obstruction should be obvious on the scan.

Primary colonic masses may invade the pan- creatic head. If they are mucous secreting, floccu- lent calcification may be detected by CT (Figure 17).

Fatty replacement or infiltration of the pancreas is seen in cystic fibrosis [12] and in metaphyseal chondrodysplasia with pancreatic insufficiency and neutropenia (Schwachman syndrome). This can be beautifully shown on CT, as can deposits of calcium in calcific pancreatitis and iron in thalassemic chil-

FIGURE 17. A large mass with speckled calcification lies anterior to the right kidney. This is an extensive mucus- secreting carcinoma of the colon with hepatic metastases.

FIGURE 18. This 18-month-old chilcl with a histologi- cally proven rhabdomyosarcoma has + large retroperito- neal mass with pathological calcification and invasion of the hilus of the left kidney. This appearance is identical to that seen in neuroblastoma.

dren receiving multiple blood transfusions [ 131. Pseudocyst formation usually seen after trau-

matic pancreatitis can be demonstlrated by CT, but it is probably best diagnosed and followed up by ultrasound [ 11.

Other Retroperitoneal Tumors

Retroperitoneal rhabdomyosarcomas can have iden- tical features to neuroblastomas exicept that involve- ment of the spine and contents occurs less fre-

FIGURE 19. There is a large cystic qass adjacent to the enhanced left kidney with a more solid component ex- tending anteriorly. There are enlarg4d preaortic nodes. This rhabdomyosarcoma extended intb the chest.

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74 STANLEY THE JOURNAL OF COMPUTED TOMOGRAPHY VOL. 7, NO. 1

Blood Vessels

FIGURE 20. A scan after injection of contrast medium shows a partially enhancing mass surrounding the right colon. This was found to be a hemangioma at surgery.

Aneurysms of the abdominal aorta and complica- tions thereof are readily detected by CT in adults, but there have been few descriptions of CT of pe- diatric aortic disease, admittedly a rare occurrence [l&15]. However, abnormalities of the venous system are more frequent. A tumor or blood clot within the inferior vena cava can be easily demonstrated with CT, particularly if calcified. Anatomic variants, with azygous or hemiazygous continuation of the inferior vena cava and duplication of the inferior vena cava, may be detected and can simulate lymphadenopa- thy unless contrast-medium-enhancement studies are performed. With portal hypertension, dilatation and tortuosity of the splenic, superior mesenteric, and portal vein, along with retroperitoneal collater- als may be visible.

quently. The tumor will encircle and displace major blood vessels, invade the renal hilus, and displace the kidneys laterally (Figure 18). Calcification may be present along with heterogeneous enhancement after administration of intravenous contrast me- dium. Occasionally, rhabdomyosarcomas may have a low-density cystic component that can be easily evacuated at surgery (Figure 19).

There are rare benign retroperitoneal tumors which can be demonstrated by CT. Lymphangiomas and hemangiomas may surround the bowel and have large contrast-medium-enhancing spaces with- in them (Figure 20).

Lymphadenopathy

In adults, CT is an extremely efficient method of de- tecting lymphadenopathy, and measurements have been established giving the upper dimensions of normal lymph nodes. This diagnostic ease is not seen with pediatric patients because of the lack of fat that separates individual nodes in the adult. Also, loops of unopacified bowel may simulate lymphadenopathy [ 71. Even so, lymphadenopathy may be confidently diagnosed in some patients (Fig- ure 19). Prone as well as supine scanning is some- times helpful. Because many treatment protocols require a staging laparotomy for lymphomas, inde- pendent of the results of abdominal imaging, only limited attention has been paid to the initial detec- tion of lymph node disease. However, it is a field that requires further investigation.

FIGURE 21. (A) A scan without contrast-medium en- hancement show swelling of a poorly defined right psoas muscle. (B) Section through the pelvis revealing swelling of the iliacus muscle with a moderately well defined lu- cency [asterisk] compatible with an abscess. Blood cul- tures revealed staphylococcus aureus.

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FEBRUARY 1983 RETROPERITONEUM 75

Vertebral and Paraspinal Disease

Tumors and inflammatory disease arising from the vertebral bodies or paraspinal musculature may project into the retroperitoneal space. The axial plane of CT is ideally suited to demonstrate the ori- gin, nature, and scope of the disease. Psoas ab- scesses will show as muscle swelling with ill-de- fined margins (Figure Zl), and will later have lucent centers with circumferential areas of contrast-me- dium enhancement [16]; in long-standing cases, cal- cification will be present. Malignant primary bone tumors arising from the spine are rare in children, but at Childrens Hospital of Los Angeles we have experience with a sacral Ewing’s sarcoma invading the retroperitoneal space.

Undescended Testis

Computed tomography has been employed in the localization of the testis in children with cryptor- chism [17]. In view of the irradiation involved in CT with scans at l-cm intervals from the kidneys to the groin, ultrasound is the first investigation, fol- lowed by a retrograde gonadal venogram (single film technique). Only if these two investigations are noncontributory would CT be performed.

INTERVENTIONAL COMPUTED TOMOGRAPHY WITH RETROPERITONEAL DISEASE

Over the past Z-3 years, there has been an explo- sion of interventional procedures performed on ret- roperitoneal structures. The procedures may be di- vided into (a] biopsies, and (b) drainage procedures. The latter may be for abscesses, hematomas, or urine from an obstructed renal pelvis (percutaneous nephrostomy). Many of the masses and fluid collec- tions are visible ultrasonically, and the initial nee- dling can be guided by portable ultrasound in the special procedures radiographic suite. In those sit- uations in which the area cannot be localized by ultrasound, CT is employed. With tissue biopsies, the examination is performed completely in the CT area. With drainage procedures, the patient is trans- ferred to a fluoroscopy suite for a catheter-guide wire interchange after the initial needling unless a trocar is used that does not require fluoroscopy.

REFERENCES 1. Berger PE. Chapter title. In computerized tomography in gas-

trointestinal imaging in pediatrics. Franken EA (ed): Phila- delphia; Harper and Row; 1982.

2. Kuhn JP, Berger PE. Computed tomographic imaging of ab- dominal abnormalities in infancy and childhood. Pediatr Ann 1980;9:200-9.

3. Hansen GC, Hoffman RB, Sample WF, Becker R. Computed tomography diagnosis of renal angiomyolipoma. Radiology 1978;128:789-791,

4. Kuhn JP. Berger PE. Computed tomography in the evaluation of blunt abdominal trauma in children. Radio1 Clin North Am 1981;19:503-26.

5. Kuhn JP, Berger PE. Computed tomogtaphy of the kidney in infancy and childhood. Radio1 Clin North Am 1981; 19:445-61.

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Resjo IM, Harwood Nash DC, Fitz CR, et al. C.T. metrizamide myelography for intraspinal and paraspinal neoplasms in in- fants and children. Am J Roentgen01 1979;132:367-72.

Kuhns LR. CT. of the retroperitoneum in children. Radio1 Clin North Am 1981;19:495-501.

Brasch RC, Korobkin M, Gooding CA. Computed body to- mography in children: evaluation of 45 patients. Am J Roent- genol 1978;131:2i-5. Doppman JL. Computed tomography of the endocrine glands. Categorical course on computed tomography. The American Roentgen Ray Society Meeting, May 1982. Laursen K, Damgaard-Pederson K. CT. for pheochromocy- toma diagnosis. Am J Roentgen01 1980;134:277-80. Dunnick NR, Doppman JL, Geelhoed GW. Intravenous ex- tension of endocrine tumors. Am J Roentgen01 1980;135: 471-6.

Cunningham DG, Churchill RJ, Reynes CJ. Computed tomog- raphy in the evaluation of liver disease in cystic fibrosis pa- tients. J Comput Assist Tomogr 1980;4:151-4.

I”. Long JA, Doppman JL, Nienhuis AW, et al.: Computed to- mographic analysis of P-thalassemic syndromes with hemo- chromatosis. J Comput Assist Tomogr 1980;4:159-65.

14. Boldt DW, Reilly BJ: Computed tomolgraphy of abdominal mass lesions in children. Radiology 1977;124:371-8.

15. Siegel MJ, Balfe DM, McClennan BL, Levitt RG: Clinical util- ity of CT. in pediatric retroperitoneal disease. Am J Roent- genol i982;i3a:loli-7.

16. Afshani E. Computed tomography of abdominal abscess in children. Radio1 Clin North Am 1981;19:515-26.

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CONTINUING MEDICAL EDUCATION QUESTIONS

True or false:

1. Computed tomography is the methlod of choice for the evaluation of renal transplant complications.

2. Computed tomography is the best method to investi- gate retroperitoneal neuroblastoma.

3. Unopacified loops of bowel may simulate lymphade- nopathy.