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dominal masses. For example, an- tion obstruction, and ectopic uretero- Solid renal lesions occur much less
giography may be indicated for pa- celes are other causes of hydrone- frequently than cystic lesions. Most
tients with a hepatic tumor in whom phrosis but, unlike ureteropelvic junc- of these masses are benign and in-
assessment of resectability is being tion obstruction, are associated with dude renal vein thrombosis and me-
determined. ureteral dilation (hydroureter), a find- soblastic nephroma. Renal vein
ing frequently detectable by ultra- thrombosis in the newborn usually
WORKUP BY ANATOMIC sound. Reflux may occur alone or in produces no symptoms and may be
association with posterior urethral associated with hematuria or protein-
LOCATION
valves. The initial workup for hydro- uria. In addition, thrombocytopenia
nephrosis should be with ultrasound and/or disseminated intravascular
Retroperitoneal Masses
and voiding cystourethrography. coagulation may be initial signs. De-
An algorithm has been devised (Fig Using these studies, one will be able hydration and hemoconcentration are
2) to aid in the evaluation of a neo- to determine the level of obstruction usually predisposing factors. Infants
natal abdominal mass. This has been and whether ureterovesical reflux is of diabetic mothers are particularly
made as simple as possible. A more present. No further imaging proce- susceptible. Diagnosis is usually pos-
comprehensive discussion follows. dures may be indicated, depending sible with ultrasound alone, which
Kidney. Since the majority of neo- on the results of these studies. Renal shows absence of blood flow in the
natal abdominal masses are renal in scintigraphy to evaluate renal func- renal vein and an enlarged kidney. CT
origin, the flank is the most common tion and intravenous pyelography (1 or MRI can demonstrate thrombus in
site of presentation. A list of possible to 2 weeks postnatally) to better de- the renal vein and/or inferior vena
masses arising from the kidney or fine the anatomy may be useful under cava. A diethylenetriamine renal scan
adrenal gland can be found in Table specific circumstances. CT and MRI has been suggested as another
1 Defining the mass as either solid
. are not necessary. Obstructing le- method of diagnosing renal vein
or cystic during physical examination sions involving the ureteropelvic junc- thrombosis. However, these proce-
and ultrasound is useful in narrowing tion or ureterovesical junction are dures are usually not necessary.
the differential diagnosis. Cystic treated surgically. Temporary de- Treatment is nonoperative and con-
masses are more common, with mul- compression by ultrasound-guided sists of rehydration and correction of
ticystic, dysplastic kidney, and hydro- percutaneous nephrostomy can be electrolyte disturbances. Hepariniza-
nephrosis making up most of the neo- useful in preserving renal function tion is only indicated in cases of pul-
natal flank masses and accounting and assessing whether renal function monary embolus (rare). Recovery of
for 40% of all neonatal abdominal might improve in a severely compro- renal function is variable but may be
masses. A multicystic, dysplastic kid- mised kidney. This would provide in- good.
ney consists of multiple noncommun- formation concerning whether to re- Mesoblastic nephroma (fetal renal
icating cysts thought to be the result pair or resect the involved kidney. hamartoma) is benign and the most
of atresia of the ureteropelvlc system Reflux severe enough to produce sig- common neonatal renal tumor. More
and is often palpable as an irregular nificant hydronephrosis is usually the than 90% of these tumors are pal-
feeling, compressible flank mass. The result of urethral obstruction (poste- pable and 1 8% of the infants have
ultrasound findings of multiple hypo- rior urethral valves) or a neurogenic hematuria. Ultrasound is usually di-
echoic, noncommunicating cysts of bladder. Vesicostomy has proved to agnostic, but a CT scan may be help-
variable sizes are usually diagnostic. be helpful as initial treatment. Trans- ful in older neonates. Complete sur-
Both kidneys should be evaluated be- urethral fulguration is the definitive gical excision is curative for mesob-
cause in one third of cases the con- treatment for posterior urethral lastic nephromas, but differentiation
tralateral kidney is abnormal. Multi- valves. from Wilms tumor can only be done
cystic, dysplastic kidneys are non- after surgical removal and histologic
functional, and therefore, it is examination. Nephroblastomatosis
generally recommended that they be The kidney accounts for and nodular renal blastema are rare
removed to avoid development of hy- more than 50% of neonatal bilateral lesions in which both kidneys
pertension, infection, or carcinoma. abdominal masses. have multiple confluent nodules corn-
Infants with congenital hydrone- posed of metanephric epitheliurn. Ul-
phrosis are usually asymptomatic; trasound and CT are the optimal
the flank mass results from mechan- Intermittent catheterization has methods of diagnosis.
ical obstruction, defects in ureteral been used with some success in pa- Neonatal Wilrns tumor (nephro-
propulsion secondary to muscle dys- tients with neurogenic bladder. blastoma) is rare. Evaluation is usu-
function, or vesicoureteral reflux. Ure- Polycystic kidney disease is usually ally by ultrasound and CT. It is difficult
teropelvic junction obstruction is the bilateral, has a genetic basis, and re- to preoperatively differentiate mesob-
most common cause of hydrone- quires no specific treatment. The in- lastic nephroma from Wilrns tumor.
phrosis (22%) and is considered to fantile form is uniformly fatal. The Surgical excision is the treatment of
be due to intrinsic or extrinsic me- adult form rarely occurs in the neo- choice for both lesions.
chanical obstruction or poor peristal- nate and only requires surveillance Adrenal Gland. The adrenal gland
sis of the proximal ureter. Posterior for the development of hypertension may be the source of a flank mass in
urethral valves, ureterovesical junc- and renal failure. the newborn. The most common
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Renal
Cystic
Hydronephrosis Common May be bilateral Voiding cystourethrogram, renal
scan, decompression/recon-
struction
Multicystic kidney (type Common Ureteral atresia may be bi- Voiding cystourethrogram, renal
II) lateral scan, nephrectomy
Infantile polycystic kid- Uncommon Bilateral pulmonary hypo- Supportive respiratory care and di-
ney disease (type I) plasia, hepatic disease alysis, excretory urogram if
needed for diagnosis
Adult polycystic kidney Rare in neonates Bilateral large renal cysts Follow-up for development of hy-
disease (type III) pertension, renal failure
Solid
Renal vein thrombosis Uncommon Birth trauma, maternal tox- Hydration, acid-base, and electro-
emia or diabetes mellitus lyte correction; CT scan or MRI
if ultrasound nondiagnostic
Mesoblastic nephroma Uncommon Occasional hypertension Chest roentgenogram, CT scan,
or MRI; nephrectomy
Wilms tumor Rare in neonates May be bilateral hyperten- Chest and abdominal CT, radical
sion, hematuna nephrectomy, may require adju-
vant therapy
Nephroblastomatosis Rare Bilateral renal enlargement Possible biopsy, continued follow-
with solid parenchymal up
densities
Adrenal
Cystic
Early hemorrhage Uncommon Birth trauma, anemia, shock Rule out ongoing hemorrhage,
evaluate for caval compression,
serial ultrasound to document
shrinkage
Solid
Late hemorrhage Uncommon Adrenal insufficiency, calci- Serial ultrasound to rule out en-
fled rim around hema- largement as in neuroblastoma
toma
Neuroblastoma Isolated mass or with Involvement of skin, liver, Urinary vanillylmandelic and homo-
metastic disease bone marrow; occasional vanillic acids, tumor resection or
(stage IV-S) hypertension biopsy
Ab,iats: CT, computed tomography; MRI, magnetic resonance imaging.
masses include adrenal hemorrhage and metabolites (eg, vanillylmandelic Other Retroperitoneal Masses.
and neuroblastoma. Birth trauma, acid and homovanillic acid) should be Other tumors, both cystic and solid,
anemia, or evidence of adrenal insuf- measured. Ultrasound shows a ho- may arise in the retroperitoneum.
ficiency may be present in the neo- mogeneous echogenic solid mass Cystic lesions usually are lymphangi-
nate with adrenal hemorrhage. How- frequently associated with stippled omas. Solid masses may be terato-
ever, the usual finding is a palpable calcification. Congenital neuro- mas or soft tissue tumors such as
mass. In 70% of patients, the hem- blastoma often is associated with rhabdomyosarcoma. Ultrasound and
orrhage occurs in the right adrenal skin, liver, and bone marrow metas- CT are useful methods of diagnosis.
gland. Diagnosis is usually by ultra- tases (stage IV-S). Therefore, these MRI may prove to be more useful
sound and no specific treatment is potential metastatic sites should be than CT when evaluating these le-
required. However, if bilateral hem- investigated. CT can be useful in eval- sions. Treatment is by surgical exci-
orrhage (5% to 1 0%) is present, ste- uating the site of origin as well as sion.
roid replacement may be necessary. areas of intraabdominal extension or
Neuroblastoma in the neonate fre- metastases. Treatment depends on
Intraperitoneal Masses
quently arises in the adrenal gland or the stage. Surgical excision of the
sympathetic chain. Stippled calcifica- primary tumor in stages I, II, and IV- Right Upper Abdominal (Hepatobil-
tion in the mass visualized by abdom- S is the usual treatment. Radiation iary) Masses. Most right upper quad-
inal radiograph can help make the and chemotherapy are important ad- rant masses are hepatobiliary in on-
diagnosis. Urinary catecholamines juvant modes of treatment. gin. Cystic masses in this location
include hydrops of the gallbladder, a mass associated with the gastroin- distended bladder or urachal rem-
choledochal cyst, and solitary liver testinal tract may take several days nant. In girls, hydrometrocolpos and
cysts. These masses are palpable or weeks to be identified. When pal- ovarian cysts are additional diagnos-
and either cause no symptoms or pated, most gastrointestinal tract-re- tic possibilities. Masses that originate
cause jaundice (choledochal cyst). lated masses are smooth, round, and from the presacral space include sac-
Solid masses include hepatic heman- mobile. rococcygeal teratoma and anterior
gioendothelioma, cavernous heman- Evaluation of an intrapenitoneal meningomyelocele. The physical ex-
gioma, hamartoma, and hepatoblas- mass suspected of being related to amination by abdominal palpation,
toma. The findings depend on the the gastrointestinal tract should begin rectal examination, and inspection of
specific lesion. Generalized hepato- with an abdominal roentgenograph. the introitus in girls can be helpful in
megaly or a mass contiguous with This may show partial intestinal ob- arriving at a tentative diagnosis.
the liver by physical examination are struction and/or linear calcification in Examination of the introitus in a girl
best evaluated by ultrasound and CT the wall of a cyst. Further evaluation might reveal a bulging membrane in
with intravenous contrast. Cystic should be accomplished by ultra- cases of imperforate hymen or a pin-
masses in the right upper quadrant sound. Gastrointestinal contrast point opening in the perineum asso-
are best evaluated by ultrasound and studies (upper gastrointestinal or bar- ciated with vaginal atresia. An ultra-
radionuclide scanning. If a choledo- ium enema) may be indicated, de- sound will confirm either a dilated
chal cyst is identified, then resection pending on the findings of the other vagina (hydrocolpos) or dilated va-
with Roux-en-Y hepaticojejunostomy studies. gina and uterus (hydrometrocolpos).
should be performed. Duplications are the most common Treatment is surgical and depends on
The initial signs of a hepatic cay- gastrointestinal masses in the neo- the specific problem.
ernous hemangioma or hemangioen- nate. They can occur from the stom- Although the ovary is a pelvic
dothelioma may be indicated by a ach to the rectum, but the ileocecal structure, ovarian lesions in newborn
mass, by generalized hepatomegaly, area is the most common site. Dupli- girls extend out of the pelvis as they
and/or by congestive heart failure. cations are usually cystic and do not grow and most often are detected as
Diagnosis is by CT or liver scan which communicate with the normal gas- an abdominal mass. In fact, a cystic
shows a highly vascular lesion. MRI trointestinal lumen. However, some mass that appears to be mobile
may prove to be a better imaging duplications are tubular, communi- within a female newborn’s lower ab-
modality. Both vascular lesions may cate with the adjacent bowel lumen, domen is most likely to be ovarian in
spontaneously involute. Steroid then- and contain ectopic gastric mucosa. origin. Ultrasound will confirm the
apy may occasionally hasten this Both forms can be detected as a cystic nature of the mass but may
process. However, congestive heart mass or intestinal obstruction (usually not be able to define the organ or
failure may be profound and require partial). The latter variety can cause origin. If the cyst produces no symp-
intervention by hepatic artery embo- bleeding similar to a Meckel’s diver- toms, it may be possible to follow-up
lization, ligation, or resection. ticulum, but this usually does not oc- the infant by serial ultrasounds be-
Hepatic hamartoma is a firm mass cur in the neonatal period. Cysts onig- cause some ovarian cysts will spon-
that is homogeneous by ultrasound mating in the omentum or mesentery taneously resolve. If surgery is war-
or CT. Treatment is by surgical exci- also occur. In utero intestinal perfo- ranted, the goal should be resection
sion. Hepatoblastoma is a malignant ration can produce a meconium cyst
tumor that may be identified in an which often has areas of calcification
infant with massive hepatornegaly or (“meconium peritonitis”) that are visi- TABLE 2. Recommended
a specific mass. It is associated with ble on abdominal radiographs. A Obstetric Management for
an elevated serum a-fetoprotein splenic cyst may rarely be present in lJtrasound-ldentifled Fetal
level. CT and perhaps MRI are the the newborn. Urobogic Abnormalities
optimal imaging techniques. Arteni- Dilated intestinal loops from intra- Term gestation: Vaginal delivery
ography may be indicated to deter- lurninal air or intestinal contents can Intestinal atresia
mine resectability. Surgical excision be mistaken for an intrapenitoneal Mesenteric, omental or splenic
is the treatment of choice. However, mass. The workup should begin with cysts
some lesions that are initially unre- plain abdominal radiographs to rule Intestinal duplications
sectable may be amenable to exci- out obstruction. If obstruction is pres- Omphalocele
sion after a course of chemotherapy. ent, intestinal atresia(s), meconium Small to moderate sacrococcyg-
ileus (cystic fibrosis), volvulus or eal teratoma
Gastrointestinal Masses. Masses Ovarian cyst
related to the gastrointestinal tract Hirschsprung disease are possibili- Term gestation: Possible cesarean
can be detected in an infant by the ties. A CT scan or MRI are unneces- delivery
physical findings of a palpable mass, sary studies because they do not pro- Giant omphabocele
generalized abdominal distention, or vide useful information related to gas- Large sacrococcygeal teratoma
intestinal obstruction. A palpable ab- trointestinal masses or obstruction. Possible preterm gestation: Vagi-
dominal mass associated with intes- nal or cesarean delivery
tinal obstruction almost always origi- Pelvic Masses Gastroschisis
nates in the gastrointestinal tract. In The finding of a mass arising from * Experimental and controversial.
the absence of intestinal obstruction, the pelvis in boys is likely to be a
of the cyst alone, sparing as much PRENATAL DIAGNOSIS gested obstetric management for
ovarian tissue as possible. Solid le- nongenitouninary fetal anomalies di-
sions of the ovary are usually terato- The increasing use of fetal ultra- agnosed by prenatal ultrasound are
mas. Treatment is by excision of the sonography has provided a unique shown in Table 2. Elective cesarean
mass or ovary if necessary to rule out opportunity to prenatally diagnose section can be considered when
malignancy. abdominal masses. Frequently, the those abdominal abnormalities such
Sacrococcygeal teratomas are the neonatologist, pediatrician, and pedi- as giant omphalocele might produce
most common congenital neoplasm. atnic surgeon are consulted by the dystocia during vaginal delivery. Pre-
The diagnosis is usually obvious, be- obstetrician to render advice regard- term delivery has been suggested in
cause they typically are exophytic ing the optimal peninatal care. cases of gastroschisis because of an
masses posterior to the sacrum. Oc- increased risk to the extracoelomic
casionally, they may grow primarily intestinal tract late in the third trimes-
presacrally and extend out of the pel- ten. The etiology of this is not clear
Planning for prompt and
vis and be detected as a mass during but may be due to contraction of the
appropriate postnatal
a rectal or abdominal examination. abdominal wall opening and second-
treatment is the advantage of
Ultrasound usually reveals solid and prenatal diagnosis of an ary vascular compromise to the intes-
cystic components. A CT scan with tine. This concept is not proven, and
abdominal mass.
contrast will help define the local ex- therefore, preterm delivery is these
tent of the tumor and its relationship cases remains controversial.
to surrounding structures. Although Genitouninary anomalies present a
the great majority of sacrococcygeal Prenatal management of fetal in- greater number of management op-
teratomas are benign at birth, prompt traabdominal anomalies are largely tions (Table 3). Patients with unilat-
en bloc removal of the tumor and the obstetric in nature at the present eral renal disease are best managed
tip of the coccyx is indicated because time. They include the need for ma- after term delivery. Adequate renal
prolonged delay increases the risk of ternal transport to a facility in which function in the unaffected kidney
adverse affects from malignancy. An- optimal neonatal and pediatric sur- does not warrant the risks associated
terior myelomeningocele may, occa- gical care are available, determination with in utero manipulation or prema-
sionally, be detected as a pelvic of the type of delivery (cesarean or tune delivery. However, severe bilat-
mass, but the presence of lower ex- vaginal), and whether premature de- eral obstructive uropathy is fre-
tremity paraplegia and muscle atro- livery is appropriate. If resources for quently associated with significant
phy generally makes the diagnosis in utero intervention are available, this postnatal morbidity due to pulmonary
obvious. may be a consideration. The sug- hypoplasia. Successful treatment of
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Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and
trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143.
Copyright © 1989 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.