APPROACH TO ORGANOMEGALIES BY DR. INNOCENT.pptx

A purely class-based revision on general approach to organomegalies in pediatrics.

1. APPROACH TO ORGANOMEGALIES PRESENTER: ARIHO INNOCENT SUPERVISOR: DR.BEATRICE

2. Introduction Organomegaly – Refers to abnormal enlargement of organs Visceromegaly – Refers to enlargement of abdominal organs Organomegaly is a common pediatric problem which can indicate various underlying conditions

3. Forms of Organomegalies Splenomegaly Hepatomegaly Cardiomegaly Hepatosplenomegaly Nephromegaly Lymphadenopathy/Lymphadenomegaly Adrenomegaly Thyromegaly

4. Splenomegaly Spleen is a major site of antigen presentation and platelet reservoir. Splenic macrophages remove damaged or old RBCs. Spleen enlarges as a result of over-activity of any of these processes. The high prevalence of chronic infection (especially malaria) as well as hemolytic anemias in the tropics means that splenomegaly is a common finding. Splenomegaly may lead to abdominal distension , discomfort , and early satiety . Massive splenomegaly may lead to pancytopenia .

5. The term splenomegaly can refer to a finding on physical examination or on imaging (or both). On physical examination , splenomegaly is generally defined as a palpable splenic edge felt >2cm below the left costal margin. NB: A palpable spleen tip may be a normal finding in up to 30% of neonates; however, the frequency drops to approximately 10% in healthy school-age children and <3% in young adults.

6. Usually identified by: Splenic notch Moves with respiration Grows downwards and medially You cannot get above it Dull to percussion

7. On imaging studies , splenomegaly is defined as splenic length or volume that is above the upper limits of normal for age. 1week of age - 4.5 to 5 cm Age 3 months – 6 cm Age 12 months – 7 cm Age 6 years – 9.5 cm Age 12 years – 11.5 cm Age ≥15 years – 12 cm for girls and 13 cm for boys

8. CAUSES OF SPLENOMEGALY I nfections (e.g. EBV, CMV, TB, Malaria, HIV, Babesiosis, Cat scratch disease, Fungal infection, Leishmaniasis, Toxoplasmosis, etc.) Malignancy (e.g. Acute lymphoblastic leukemia (ALL), including precursor B cell ALL and precursor T cell ALL/lymphoma, Acute myeloid leukemia, Hodgkin lymphoma, Non-Hodgkin lymphoma) Other lymphoproliferative disorders Langerhans cell histiocytosis Hemophagocytic lymphohistiocytosis Autoimmune lymphoproliferative syndrome Castleman disease and POEMS syndrome ( p olyneuropathy,  o rganomegaly,  e ndocrinopathy,  m onoclonal protein,  s kin changes)

9. D isorders of immune regulation (e.g., systemic lupus erythematosus, rheumatoid arthritis, common variable immunodeficiency) Liver disease/Portal Hypertension particularly end-stage liver failure (cirrhosis) resulting from congestion due to portal hypertension. S torage diseases (e.g. Gaucher disease, Niemann-Pick disease, and the mucopolysaccharidoses.) S pace-occupying lesions (e.g., hemangioma, cyst, hamartoma) Hemolytic anemia - Congenital and acquired hemolytic anemias represent a major cause of splenomegaly. In these conditions, splenomegaly results from sequestration of destroyed red blood cells (RBCs). E.g. SCD Other  - Other less common causes of splenomegaly in children include sarcoidosis and metastatic tumors (e.g., neuroblastoma).

10. CAUSES OF MASSIVE SPLENOMEGALY A spleen is considered to be massively enlarged if its lower pole is within the pelvis or if it crosses the midline. Only a few diseases cause this degree of splenic enlargement. These include the following disorders: Leukemia (lymphoid or myeloid) Lymphoma, usually the more indolent variants Thalassemia major Acute splenic sequestration in sickle cell disease (SCD) Langerhans cell histiocytosis 

11. Hemophagocytic lymphohistiocytosis Autoimmune lymphoproliferative syndrome Castleman disease Gaucher disease HIV infection with  Mycobacterium avium  complex Kala-azar Hyperreactive malarial splenomegaly syndrome, also called tropical splenomegaly syndrome

12. EVALUATION a) History  — The history may provide valuable clues as to the possible cause of splenomegaly. A history of recent febrile illness , pharyngitis , and fatigue suggests a viral etiology (e.g., Epstein-Barr virus [EBV], cytomegalovirus [CMV], viral hepatitis). If more pronounced constitutional symptoms are noted (e.g., persistent fevers , night sweats , weight loss , decreased activity level ), systemic diseases such as leukemia, lymphoma, systemic lupus erythematosus, malaria, or tuberculosis should be considered. In some cases, the presence of an underlying condition is already known and the spleen may revert to normal size when the underlying disease is brought under control with appropriate therapy.

13. b) Physical examination This includes, inspection , percussion and palpation On Inspection The abdomen may appear visibly distended, especially on the left side A noticeable bulge or mass in the left upper quadrant (Left Hypochondriac region) may be evident, especially in cases of significant splenomegaly. A few skin changes such as jaundice (underlying hemolytic anemia or liver disease), and pallor (anemia).

14. On percussion Percuss along the left anterior axillary line at the lowest intercostal space. If the child is older, ask them to take a deep breath while you are percussing the area; it should stay resonant for the period of inspiration. If the percussion note changes from resonant to dull , splenomegaly should be suspected.

15. On palpation A palpable spleen may represent a normal finding , splenomegaly , or massive splenomegaly . A nontender spleen that is normal or only minimally enlarged will be quite movable with respiration and may be palpable only at the end of inspiration. Using a light touch, with the skin depressed under the left costal margin, one can feel a minimally enlarged spleen as a rounded edge with the consistency of normal liver, which slips under the examiner's fingers at the end of inspiration and back on expiration. A normal spleen is soft and nontender.

16. 1 2 Palpation of the spleen more medially than in 1 Palpation Palpation of the spleen. Start well out to the left .

17. Hackett’s grading system for splenomegaly 

18. Splenomegaly  – A splenic edge felt >2 cm below the left costal margin is an abnormal finding. An abnormal spleen often feels hard, unlike the soft sensation of palpating a minimally enlarged spleen in a healthy child. Tenderness to palpation also suggests an abnormal finding. Massive splenomegaly  – A spleen is considered to be massively enlarged if its lower pole is within the pelvis or if it crosses the midline. In extreme cases, the enlarged spleen may even be palpable in the right upper quadrant. Exquisite splenic tenderness suggests infarction or perisplenitis in such massively enlarged spleens.

19. Other examination findings  — Other physical examination findings that are important to note when evaluating a child with splenomegaly include: Lymphadenopathy  – Lymphadenopathy may be a sign of infection (e.g., EBV or HIV-1) or malignancy Jaundice  – Jaundice may indicate underlying hemolytic anemia or liver disease.  Hepatomegaly and other signs of liver disease  – Hepatomegaly, firm liver edge, ascites, and/or spider angiomata suggest underlying liver disease. Rashes and/or joint swelling  – Rashes and/or joint swelling suggest a systemic autoimmune disorder such as systemic lupus erythematosus or rheumatoid arthritis. Petechiae and ecchymoses  – Petechiae and ecchymoses may be present in patients with thrombocytopenia resulting from splenic enlargement. They may also be a sign of underlying malignant hematologic disease.

20. Investigations Initial diagnostic testing Initial testing for children with unexplained splenomegaly includes imaging (chest radiograph and abdominal ultrasound) and laboratory testing. Imaging modalities  – Ultrasonography is usually the initial imaging test for evaluation of a child with unexplained splenomegaly because it is readily available, noninvasive, does not expose the child to radiation, and does not require sedation. Doppler imaging is useful for evaluating splenic and portal blood flow. A finding of slow or reversed portal blood flow is suggestive of portal hypertension.

21. Laboratory evaluation  - The initial laboratory evaluation of the child with unexplained splenomegaly includes: Complete blood count including platelet count and differential reticulocyte count. Review of the peripheral blood smear Liver function tests Lactate dehydrogenase C-reactive protein and/or erythrocyte sedimentation rate EBV and CMV serologies Biopsy (If malignancy is suspected)

22. HEPATOMEGALY The liver is an abdominal organ with multiple functions including: F iltering blood from the gastrointestinal (GI) tract, S ecreting bile into the GI tract, M etabolizing drugs, and S ynthesizing proteins (e.g., clotting factors). The liver's vascularity, location, and functions make it susceptible to several conditions that result in hepatomegaly , defined as enlargement of the liver beyond its normal size . Hepatomegaly may be suspected based on physical examination findings or imaging .

23. The liver is a wedge-shaped organ located in the right upper quadrant of the abdomen. The liver typically extends from the fifth intercostal space to the right costal margin in the midclavicular line. By ultrasound, a normal liver span is usually < 16 cm in the midclavicular line in adults; however, liver size varies with sex and body size, Liver percussion and palpation are common examination methods, while imaging is used frequently to assess liver size and texture , and to look for abnormalities such as focal lesions, or portal or hepatic vein thrombosis.

24. Causes of hepatomegaly Infectious diseases - Hepatitis A infection, malaria, or infectious mononucleosis Hematological disorders -Thalassemia major Malignancies - Leukemia, lymphoma, hepatoblastoma, neuroblastoma, Wilms’ tumor or other metastatic cancers Cardiovascular diseases - Heart failure Liver disease - Chronic active hepatitis Rare causes - Glycogen storage disorders or amyloidosis

25. EVALUATION The initial evaluation of a patient with hepatomegaly includes: Obtaining a history to identify symptoms suggestive of underlying systemic disease and risk factors for liver disease, P erforming a physical examination to look for clues to the etiology and for signs of liver disease, and O btaining liver biochemical and function tests and Doppler ultrasound.

26. NOTE Hepatomegaly is common in the tropics, therefore; Look for jaundice and signs of Chronic Liver Disease. Palpate the liver, noting texture, and percuss to define size (normal liver is <12cm in mid-clavicular line). Auscultate for hepatic bruit (typically in hepatocellular carcinoma, alcoholic hepatitis) or peritoneal rub over liver (may be present in metastases, liver abscesses).

27. History   I dentify symptoms of systemic disease (e.g., fever, weight loss) and risk factors for liver disease, including exposure to potential hepatotoxins (e.g., alcohol, medications). While some patients with hepatomegaly are asymptomatic , others may report right upper quadrant pain or symptoms related to the underlying disorder.

28. Physical examination : In addition to evaluating liver size and consistency, physical examination includes assessment for: Stigmata of chronic liver disease (e.g., spider nevi, palmar erythema, gynecomastia) Increased jugular venous pressure, a sign of right-sided heart failure Spleen size (a palpable spleen is two to threefold enlarged) Ascites (typically detected by flank dullness to percussion)

29. Physical examination of the liver includes abdominal palpation and percussion . To determine liver span, we percuss and palpate the liver in the midclavicular line with the patient in a supine position Percussion Ask the older child to hold his/her breath on full expiration. Percuss along the mid-clavicular line to identify the upper and lower liver borders. Start percussion at the third intercostal space and move downward. Normally, the liver dullness begins at the fifth intercostal space and continues just below the costal margin.

30. With the patient in a supine position and starting in the third intercostal space , the examiner uses percussion while moving inferiorly until dullness suggests the upper border of the liver (usually located in the fifth intercostal space ). Next, percussion is performed from below the umbilicus in an area of tympany, and the examiner moves superiorly until dullness ( hypotympanic note ) indicates the liver's inferior border. Percussion alone may underestimate liver size because the lower border of the liver may seem r esonant (tympanic) to percussion. However, if the distance from the upper border of the liver to the percussed liver edge is <13 cm, hepatomegaly is unlikely.

31. Palpation The purpose of palpation is to estimate liver span and also check for tenderness or liver lesions. With the patient in a supine position, the examining hand is placed below the level of the dullness noted on percussion in the midclavicular line, parallel to the rectus muscle. The liver is palpated during deep inspiration as it moves inferiorly to meet the examiner's finger tips. The liver edge may be tender in patients with liver inflammation (e.g., acute hepatitis) or congestion (e.g. congestive heart failure). Alternatively, the liver edge may feel firm and nodular in patients with cirrhosis and/or malignancy.

32. 1. Palpation of the liver: preferred method. 2. Palpation of the liver: alternative method.

33. Palpable but nonenlarged liver -  In addition to anatomic variants, a normal, nonenlarged liver may be palpable because it is displaced below the right costal margin. Liver displacement may occur: During deep inspiration In patients with right pleural effusion In patients with chronic obstructive pulmonary disease (hyperinflation of the chest with diaphragmatic descent may result in downward displacement of the liver) In patients with a thin body habitus.

34. Imaging  - Imaging studies that are routinely used to assess the liver include: Ultrasound – Ultrasound is a common method to assess liver size and pathology, and transaxial ultrasound technique provides a quantitative measure of liver span. Computed tomography (CT) M agnetic resonance imaging (MRI)

35. Diagnostic testing  - Initial laboratory testing is performed to look for abnormalities in liver biochemistries or function, while initial imaging evaluates liver size, consistency, and blood flow: Serum aminotransferases: alanine aminotransferase (ALT) and aspartate aminotransferase (AST) Alkaline phosphatase Total bilirubin Serum albumin Prothrombin time/international normalized ratio Doppler ultrasound Biopsy (if malignancy is suspected)

36. Summary of causes of visceromegaly

37. CARDIOMEGALY Causes Congenital heart diseases Cardiomyopathies Hypertension Endocrine disorders (Hyperthyroidism) Investigations Physical examination: Displaced Apex beat Imaging: Chest X-ray Echocardiography

38. MANAGEMENT OF ORGANOMEGALIES Treat the underlying cause : Infection Control, Chemotherapy for malignancies, managing heart failure Supportive Care : Pain management, Nutrition support. Monitoring : Regular follow-up and monitoring of organ size and function Referral : Specialist care for complex cases.

39. Complications or organomegalies Liver Dysfunction : Jaundice, coagulopathy Splenic rapture : Risk in splenomegaly Cardiac Failure : Worsening Heart function Secondary Infections : Due to immunocompromised state.

40. References: Kliegman, R. M., & St. Geme, J. w. (2023). Organomegalies in Pediatrics. In K. L. McMillan (Ed.), UpToDate . Retrieved June 21, 2024 from http://www.uptodate.com Swash, M. (Ed.) (2018). Hutchison’s clinical methods (24 th ed.). Elsevier Saadoon, A. W. (2020). Essential clinical skills in pediatrics . Medical Publishing House. Eddleston, M., Davidson, R.,Brent, A., & Wilkinson, R. (2014). Oxford handbook of tropical medicine (4 th ed.). Oxford University Press. Doe, J., & Smith, A. (2020). The application of Hackett’s classification of splenomegaly in clinical practice. Tropical Medicine Research , 15(2), 123-130. Retrieved from https://www.researchgate.net/publication/123456789_The_Application_of_Hackett’s_Classification_of_Splenomegaly_in_Clinical_Practice