Abdominal Masses




An abdominal mass or abdominal fullness in a child usually becomes apparent when it enlarges enough to be visualized during bathing or palpable on physical examination. Masses may arise from intraperitoneal, retroperitoneal, or abdominal wall locations and emanate from both solid and hollow viscera ( Figs. 17.1 and 17.2 ). An abdominal mass may prove life threatening (e.g., malignant neoplasm, splenic sequestration crisis in sickle cell disease), arise from congenital malformation or disorganized development (e.g., mesenteric cyst, enteric duplication), or be benign or correctable nonoperatively (fecaloma). Hepatomegaly and splenomegaly often represent systemic illnesses such as infection, hemolysis, storage disease, or malignancy. A child with an abdominal mass requires a prompt and thorough work-up with testing guided by history, physical examination findings, age, and gender. Early surgical referral may assist in this work-up following a directed screening approach.




FIGURE 17.1


Location of select intraabdominal tumors and masses.



FIGURE 17.2


Location of select retroperitoneal tumors and masses.


(See Nelson Textbook of Pediatrics, p. 1766.)


Diagnostic Strategies


Clinical History


A child’s age, gender, and thorough clinical history help to identify the most likely disease category ( Tables 17.1 and 17.2 ). The duration and character of associated symptoms are important for narrowing the differential diagnosis (e.g., fatigue, fever, appetite changes, vomiting, stooling history, weight loss, night sweating, character and frequency of pain, hematuria, flushing, palpitations, lower extremity swelling, lymph nodal prominence, and jaundice). A history of abdominal trauma should be elicited, as solid organ injuries may result in hematoma, seroma, persistent pseudocyst, or arteriovenous malformation. Infectious disease may have sequelae of cyst, lymphadenopathy, or intraabdominal abscess. Some systemic diseases (e.g., glycogen storage, hereditary spherocytosis), genetic syndromes (e.g., Beckwith-Wiedemann, Peutz-Jeghers, familial adenomatous polyposis), and anomalies (aniridia with Wilms tumor, isolated hemihypertrophy with neuroblastoma and Wilms tumor) are associated with intraabdominal tumors. A family and sexual history are also pertinent, particularly in adolescent females. Modern prenatal imaging frequently identifies congenital malformations and neoplasms, requiring postnatal imaging and surgical assessment.



TABLE 17.1

Stepwise Evaluation of an Abdominal Mass























Clinical History



  • Age and gender



  • General symptoms



  • Pain



  • Gastrointestinal symptoms



  • Urogenital symptoms



  • Pulmonary symptoms



  • Family history



  • Sexual history



  • Weight loss



  • Travel

Physical Examination



  • General condition



  • Lymph nodes



  • Associated physical findings



  • Cachexia

Abdominal Palpation



  • Quadrant of the abdomen



  • Organ most likely to be affected



  • Characteristics (soft or hard, mobile or nonmobile, crosses midline, moves with respiration, tender)

Ultrasonography



  • Location



  • Solid or cystic

Depending on the Clinical Suspicion, Evaluation Can Be Continued with One or More of the Following:



  • Laboratory studies: CBC, urinalysis, tumor markers



  • Imaging studies: plain radiography of chest and abdomen, contrast radiography of the gastrointestinal tract, computed tomography, magnetic resonance imaging, angiography, scintigraphy



TABLE 17.2

Age-Related Etiology of Abdominal Masses
























Age Benign Malignant
Neonate (0-1 mo)


  • Congenital hydronephrosis



  • Cystic kidney disease



  • Intestinal duplication



  • Mesenteric/omental cyst



  • Neurogenic bladder



  • Ovarian cyst



  • Renal vein thrombosis



  • Choledochal cyst



  • Mesoblastic nephroma



  • Meconium ileus



  • Hematoma (adrenal, hepatic, splenic)

Neuroblastoma
Infant (0-1 yr)


  • Intestinal duplication



  • Mesenteric/omental cyst



  • Ovarian cyst



  • Mesoblastic nephroma



  • Liver hamartomas



  • Hepatic cavernous hemangioma



  • Liver hemangioendothelioma



  • Teratoma



  • Intussusception



  • Hepatosplenomegaly



  • Choledochal cyst



  • Megacolon




  • Neuroblastoma



  • Hepatoblastoma



  • Wilms tumor (rare)



  • Teratoma

Child


  • Mesenteric/omental cyst



  • Choledochal cyst



  • Appendiceal abscess




  • Neuroblastoma (2-10 yr)



  • Hepatoblastoma



  • Wilms tumor



  • Leukemia



  • Lymphoma

Adolescent


  • Bezoar



  • Hematocolpos



  • Hydrometrocolpos



  • Pregnancy



  • Inflammatory bowel disease



  • Retroperitoneal hematoma (hemophilia)




  • Neuroblastoma (11-16 yr)



  • Hepatocellular carcinoma



  • Ovarian neoplasm



  • Lymphoma



Physical Examination


A complete physical exam should be performed in children with abdominal masses. Attention should be paid to the general condition of the child and to signs of metastatic disease. Enlarged lymph nodes and their locations should be noted, the skin inspected, and the lungs and heart auscultated. Extremities should be evaluated for evidence of swelling, venous phlegmasia, or evidence of embolic disease. Genitourinary exam should make note of any inappropriate virilization, testicular changes, and hymenal patency in the case of a female with a low pelvic mass. In addition, a neurologic examination may reveal signs of nervous system involvement. The eyes should be carefully inspected for periorbital ecchymosis, proptosis, squint, opsoclonus-myoclonus syndrome, heterochromia of the iris, Horner syndrome, and scleral icterus. The patient’s blood pressure must be determined and may be elevated in patients with Wilms tumor, neuroblastoma, or pheochromocytoma.


To successfully perform abdominal palpation in a child, the physician must approach the patient calmly and gently, as the most reliable exams are completed in cooperative and relaxed children. Enlarged organs may be missed in a struggling child who does not lie quietly. When cooperation proves difficult in an infant, the examining hand should be placed and remain static on the abdomen and the exam completed between cries or as the child calms in the parent’s arms. Creative play is sometimes necessary, with the use of pacifiers or bottles to distract the child from the exam. Re-examination after voiding or defecating elucidates contributions of constipation and urinary retention in the child’s presentation.


The abdominal quadrants should be examined systematically ( Table 17.3 ; Figs. 17.1 and 17.2 ). With the patient in the supine position, the symmetry of the abdomen should be inspected, and any visible masses or the presence of ascites should be noted. A very enlarged spleen is frequently visible, with fullness of the left side of the abdomen. The presence of tense fluid-filled hernias or prominent periumbilical veins as sequelae of portal hypertension should be noted. The mass should be localized, and its size, shape, texture, mobility, tenderness, and relation to midline noted. The umbilical position is a useful marker of abdominal asymmetry.



TABLE 17.3

Location and Nature of Abdominal Masses































































































































Organ Congenital Benign Malignant Acquired
Liver and biliary tract Hemangioma Hemangioendothelioma Hepatoblastoma Abscess
Choledochal cyst Hamartoma Lymphoma Hematoma
Leukemia Parasitic disease
Hepatocellular carcinoma Hydrops of the gallbladder
Spleen Cyst Sarcoma Splenomegaly (e.g., mononucleosis)
Kidney Hydronephrosis
Cystic disease
Duplication
Wilms tumor Hematoma
Adrenal gland Neuroblastoma Neuroblastoma Hematoma
Stomach Duplication Leiomyoma Leiomyosarcoma Bezoar
Teratoma Inflammatory pseudotumor Adenocarcinoma
Intestines Duplication Lymphangioma Carcinoma Appendiceal abscess
Megacolon Hemangioma Lymphoma Intussusception
Obstipation
Mesentery Mesenteric/omental cyst Inflammatory bowel disease
Parasitic disease
Tuberculosis
Pancreas Cyst Carcinoma Pseudocyst
Uterus Hydrometrocolpos Myoma Rhabdomyosarcoma Pregnancy
Ovaries Cyst Cyst Yolk sac tumor Tuboovarian abscess
Teratoma Cystic teratoma Embryonal carcinoma
Cystic adenoma Dysgerminoma
Granulosa cell tumor Choriocarcinoma
Bladder Urachal cyst
Posterior urethral valve
Inflammatory pseudotumor Rhabdomyosarcoma Urinary retention
Retroperitoneum Presacral teratoma Ganglioneuroma Neuroblastoma Psoas abscess
Anterior myelomeningocele Aortic aneurysm
Abdominal wall Hernia Hemangioma Rhabdomyosarcoma Hematoma
Omphalocele Rectus sheath hematoma
Gastroschisis Abscess


Signs of peritoneal inflammation must be sought (see Chapter 10 ). Dull visceral pain conducted by slow C nerve fibers may be reported for inflammatory processes in the vascular distributions of the celiac, superior mesenteric, and inferior mesenteric arteries and referred to the epigastrium, umbilical region, or hypogastrium, respectively. When the inflamed process contacts the peritoneum, peritoneal fast A nerve fibers allow discrete localization of sharp pain to the abdominal wall. Ultrasound is often a very useful adjunct in the evaluation of an abdominal mass and is often available at the bedside.


Approximately half of abdominal masses in older children are caused by enlargement of the liver or spleen, or both. The liver is normally palpated in the right upper quadrant and epigastrium extending 1-2 cm below the costal margin. The inferior hepatic margin may be palpated in a thin child, is usually nontender, and moves with respiration. Detection of liver edge by auscultation using skin scratches has been proven unreliable and has been supplanted by the use of readily available ultrasound. The spleen is located in the left upper quadrant and is nonpalpable in most healthy children. To locate an enlarged spleen, the examiner must begin palpation in the patient’s right iliac fossa (to avoid missing a grossly enlarged spleen or liver with extension of the left hepatic lobe into the splenic area in the left upper quadrant); the examiner’s right hand should move toward the patient’s left upper quadrant to find the spleen’s lower pole or medial border. The examiner’s left hand is placed in the patient’s left flank, and gentle displacement of the thoracic cage toward the examiner’s right hand often displaces the spleen forward enough to make it appreciable. The spleen has a rounded tip and should move downward with inspiration and is more superficial than a renal mass. It is equally important for the examiner to palpate the spleen as it is for the spleen to “touch” the examiner during its descent with inspiration. Overaggressive palpation may push the spleen away, whereas gentle or light palpation permits the examiner to feel the spleen’s edge passively. The characteristic notch in the medial or inferior border may not be palpable when the spleen is enlarged only a few centimeters, but the notch’s presence usually clearly distinguishes an enlarged spleen from other abdominal masses on the examination alone. Because the extent to which the spleen extends below the costal margin depends heavily on the patient’s position, the extent of the spleen below the costal margin should be measured with the patient in the supine position. Measurement from the left costal margin to the lower pole of the spleen defines the splenic axis. Ordinarily, the long axis of the spleen is along the length of the 10th rib. As it enlarges, it extends medially and downward. Masses in the left upper quadrant, especially left renal masses, may be difficult to distinguish from an enlarged spleen. In general, the presence of the splenic notch helps identify the mass as a spleen, but nodular masses, such as Wilms tumors of the kidney, neuroblastomas, and retroperitoneal teratomas may masquerade as splenomegaly. Many enlarged spleens are not palpable on physical examination because of their relationship to other organs and the thoracic cage. Hyperinflation of lungs (as occurs in asthma, bronchiolitis, ipsilateral pneumothorax) may make a normal-sized liver or spleen palpable.


Flank masses are the next most frequent, particularly in newborn to toddler-aged children. Renal masses extend caudally, are fixed with respiration, and cause abdominal asymmetry. Lower abdominal masses are most commonly caused by constipation or urinary retention. These may be functional or secondary to neurogenic dysfunction. A perforated appendix with resulting abscess formation may create a tender right lower quadrant mass. Ovarian or uterine tumors often grow undetected in the pelvis until large enough to exit the pelvis as a large palpable abdominal mass.


Laboratory and Imaging Studies


Screening laboratory data, including complete blood count with differential and cell morphology, measurements of serum electrolytes, urinalysis, urine pregnancy test when appropriate, and inflammatory markers, are broadly applicable. Liver function tests, serum amylase, tumor marker levels ( Table 17.4 ), and renal function tests are often important initial objective data points.



TABLE 17.4

Tumor Markers



















Tumor Tumor Markers
Neuroblastoma Urinary catecholamines
LDH
Ferritin
Neuron-specific enolase
Wilms tumor Erythropoietin
Hepatoblastoma, pancreatoblastoma, yolk sac tumors α-Fetoprotein
Germ cell tumors β-hCG

hCG, human chorionic gonadotropin; LDH, lactate dehydrogenase


Plain abdominal radiographs may reveal tumor calcifications, organomegaly, excess fecal load, and mass effect upon intestines. Views in at least 2 different positions should be obtained to appreciate ascites or intestinal obstruction. As a screening modality, ultrasonography is a highly efficient, low-cost, and widely available test. It is noninvasive, nonirradiating, and can give detailed information on the location, vascularity, and nature of the mass and adjacent structures ( Fig. 17.3 ). The most widely used axial imaging technique is computed tomography (CT) ( Fig. 17.4 ), followed by magnetic resonance imaging (MRI). These modalities often provide radiologic diagnosis, are invaluable for surgical planning, and are unhampered by bowel gas, a common limitation of ultrasound. MRI pulse sequences have evolved to discriminate between primary malignant, metastatic, and hamartomatous liver lesions.




FIGURE 17.3


An ultrasonic longitudinal view of the pelvis reveals hydrometrocolpos. The uterus and cervix are readily seen superior to the large collection of fluid in the vagina located at the right of the image.



FIGURE 17.4


Computed tomogram revealing a large Wilms tumor replacing the right kidney. Notice that the renal cortex, enhanced by contrast medium, is splayed out around the mass. This characteristic helps differentiate Wilms tumor from neuroblastoma, which would displace a normal-appearing kidney.




Splenomegaly


(See Nelson Textbook of Pediatrics, p. 2408.)


Unlike many other abdominal masses, splenomegaly is usually secondary to another process; it can be caused by diseases that result in hyperplasia of the lymphoid and reticuloendothelial systems (infections, connective tissue, inflammatory disorders), infiltrative disorders (Gaucher disease, leukemia, lymphoma, histiocytosis, hemophagocytic lymphohistiocytosis), hematologic disorders (thalassemia, hereditary spherocytosis), and conditions that cause distention of the sinusoids whenever there is increased pressure in the portal or splenic veins (portal hypertension) ( Table 17.5 ). In addition, palpable spleens in children and adolescents are not always indicative of disease. A palpable spleen (≤2 cm below the left costal margin) is a normal finding in a child younger than 3 years and may be a normal finding in an older child. Up to 15% of full-term neonates, 10% of children, and 3% of college freshmen have palpable spleens unassociated with an increase in lymphoreticular malignancy and with equivalent health. Painful splenomegaly generally follows stretching of the splenic capsule with rapid enlargement of the spleen. Malfixed spleens may undergo torsion, presenting the upper pole to the abdominal wall as a prominent painful abdominal mass. Splenic enlargement that is noted not in the context of an acute illness (i.e., it is noted incidentally, for instance on a well child examination or check up) is more likely to be caused from a chronic process such as a storage disease than splenomegaly that is noted in the context of an acute illness. Acute onset of splenomegaly is most characteristic of an acute infection or a rapidly progressive malignancy (acute leukemia, lymphoma). Chronic splenomegaly (present for ≥1 month) is much more likely to represent a chronic process, such as storage diseases, congestive processes (portal hypertension, congestive heart failure), hemolysis, chronic infection or inflammation.



TABLE 17.5

Differential Diagnosis of Splenomegaly by Pathophysiology































































Anatomic Lesions



  • Cysts, pseudocysts



  • Hamartomas



  • Polysplenia syndrome



  • Hemangiomas and lymphangiomas



  • Hematoma or rupture (traumatic)



  • Peliosis

Hyperplasia Caused by Hematologic Disorders
Acute and Chronic Hemolysis *



  • Hemoglobinopathies (sickle cell disease in infancy with or without sequestration crisis and sickle variants, thalassemia major, unstable hemoglobins)



  • Erythrocyte membrane disorders (hereditary spherocytosis, elliptocytosis, pyropoikilocytosis)



  • Erythrocyte enzyme deficiencies (severe G6PD deficiency, pyruvate kinase deficiency)



  • Immune hemolysis (autoimmune and isoimmune hemolysis)



  • Paroxysmal nocturnal hemoglobinuria

Chronic Iron Deficiency
Extramedullary Hematopoiesis



  • Myeloproliferative diseases: CML, juvenile CML, myelofibrosis with myeloid metaplasia, polycythemia vera



  • Osteopetrosis



  • Patients receiving granulocyte and granulocyte-macrophage colony-stimulating factors

Infections
Bacterial



  • Acute sepsis: Salmonella typhi, Streptococcus pneumoniae, Haemophilus influenzae type b, Staphylococcus aureus



  • Chronic infections: infective endocarditis, chronic meningococcemia, brucellosis, tularemia, cat-scratch disease




  • Local infections: splenic abscess ( S. aureus, streptococci, less often Salmonella species, polymicrobial infection), pyogenic liver abscess (anaerobic bacteria, gram-negative enteric bacteria), cholangitis

Viral *



  • Acute viral infections, especially in children



  • Congenital CMV, herpes simplex, rubella



  • Hepatitides A, B, and C; CMV



  • EBV



  • Viral hemophagocytic syndromes: CMV, EBV, HHV-6



  • HIV

Spirochetal



  • Syphilis, especially congenital syphilis



  • Leptospirosis

Rickettsial



  • Rocky Mountain spotted fever



  • Q fever



  • Typhus

Fungal/Mycobacterial



  • Miliary tuberculosis



  • Disseminated histoplasmosis



  • South American blastomycosis



  • Systemic candidiasis (in immunosuppressed patients)

Parasitic



  • Malaria



  • Toxoplasmosis, especially congenital



  • Toxocara canis, Toxocara cati (visceral larva migrans)



  • Leishmaniasis (kala-azar)



  • Schistosomiasis (hepatic-portal involvement)



  • Trypanosomiasis



  • Fascioliasis



  • Babesiosis

Immunologic and Inflammatory Processes *



  • Systemic lupus erythematosus



  • Rheumatoid arthritis



  • Mixed connective tissue disease



  • Systemic vasculitis



  • Serum sickness



  • Drug hypersensitivity, especially to phenytoin



  • Graft-versus-host disease



  • Sjögren syndrome



  • Cryoglobulinemia



  • Amyloidosis



  • Sarcoidosis



  • Autoimmune lymphoproliferative syndrome



  • Post-transplant lymphoproliferative disease



  • Large granular lymphocytosis and neutropenia



  • Histiocytosis syndromes



  • Hemophagocytic syndromes (nonviral, familial)

Malignancies



  • Primary: leukemia (acute, chronic), lymphoma, angiosarcoma, Hodgkin disease, mastocytosis



  • Metastatic

Storage Diseases



  • Lipidosis (Gaucher disease, Niemann-Pick disease, infantile GM1 gangliosidosis)



  • Mucopolysaccharidoses (Hurler, Hunter-type)



  • Mucolipidosis (I-cell disease, sialidosis, multiple sulfatase deficiency, fucosidosis)



  • Defects in carbohydrate metabolism: galactosemia, fructose intolerance, glycogen storage disease type IV



  • Sea-blue histiocyte syndrome



  • Tangier disease



  • Wolman disease



  • Hyperchylomicronemia type I, IV

Congestive *



  • Heart failure



  • Intrahepatic cirrhosis or fibrosis



  • Extrahepatic portal (thrombosis), splenic, and hepatic vein obstruction (thrombosis, Budd-Chiari syndrome)

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Apr 4, 2019 | Posted by in PEDIATRICS | Comments Off on Abdominal Masses

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