abdominal cyst in children

CYSTIC ABDOMINAL MASSES IN CHILDREN

Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS

Associate Consultant Pediatric Surgery; KAAUH_ PNU

INTRODUCTION

Cyst:Closed cavity or sac with liquid or semisolid contents+ Epithelial lining.

Pseudo cystFluid-filled masses that lack an epithelial lining

Cystic abdominal masses can be difficult to characterize

when they are very large and fill most of the abdomen. Because they distort normal anatomy Their site of origin can be difficult to ascertain

Liver Mes hamartoma Biloma Parasitic cyst

Biliary system Choledochal cyst Hydrops of gallbladder

Spleen Congenital cyst

Pancreas Pseudocyst Congenital cyst Retention cyst Cystadenoma

Kidney/adrenal Hydronephrosis Multicystic dysplastic kidney

Multilocular cystic nephroma Adrenal hemorrhage

Genitourinary/ovary

Functional cyst Teratoma/dermoid Cystadenoma

Hematocolpos Urachal cyst

Gastrointestinal Mes cyst \ Lymphangioma Enteric/duplication cyst

Omental cyst Meconium Pseudocyst

Miscellaneous Abscess Teratoma Necrotic or cystic changes in tumors (Wilms, neuroblastoma, etc.)

CSF Pseudocyst Sacrococcygeal teratoma

DD OF CYSTIC ABDOMINAL MASSES IN CHILDREN

Benign developmental malformation

Consisting of cysts, bile ducts, hepatocytes, mesenchyme, and branches of the portal vein.

80% present within the first 2 y of life

80% arise in the right lobe

20% are pedunculated

Typically: nontender upper Abd mass

They can grow so large that they eventually compress the inferior vena cava or cause respiratory compromise

Liver Mesenchymal hamartoma

Biloma Parasitic cyst



Laboratory: Always are normal, including LFT

AFP can be moderately elevated & will return to normal after resection

Radiological: well-defined tumor avascular centrally

and frequently contains septations and cysts of varying size with displacement of intrahepatic vasculature.

MR angiography has been proven useful both in the diagnosis and planning of the resection of these lesions.



1. Simple surgical Enucleation may be possible.

2. Very Large, Bilobar Tumors that are not amenable to resection can be marsupialized into the peritoneal cavity, but recurrence has been described and development of undifferentiated embryonic sarcoma.

• Complete excision of the lesion with a margin of normal liver is usually curative (including the use of liver transplantation for large, bilobar lesions).

• Spontaneous involution of these lesions has been reported, but this is unusual.

Liver Mesenchymal hamartoma Biloma Parasitic cyst

• Biloma or biliary cyst is a collection of bile inside the abdomen which has become encapsulated with epithelial cells.

• Many situations can contribute to the formation of a Biloma, and there are several treatment options available, depending on the location, size, and severity of the cyst.

• Symptoms can vary, abdominal pain and tenderness or symptoms such as fever and confusion, if the cyst becomes infected

Post traumatic Biloma

Liver Mesenchymal hamartoma Biloma Parasitic cyst

Biliary system

Choledochal cyst Hydrops of gallbladder

Congenital cystic malformations of the biliary tree 1 in 15,000 live births but is 1 per 1000 in Japan.

The most widely accepted etiology is pancreaticobiliary malunion permitting reflux of pancreatic enzymes into the biliary tree.

Most pt. present in the first decade of life. Classic triad of symptoms including

1. Abdominal pain2. Mass 3. Jaundice is seen in only 25% of children.

Biliary system


• Radiological:• Right upper quadrant cystic mass in the porta hepatis, separate from

the gallbladder. • Biliary origin can be confirmed by MR cholangiopancreatography • Surgical resection is necessary to prevent the development of

ascending cholangitis, calculi or malignant change.

Biliary system


If the cyst is extremely inflamed and the adhesions are very dense Do Cyst Mucosectomy

Biliary system


• Massive GB distention in the absence of stones and is probably caused by transient obstruction of the cystic duct or increased mucus secretion with ineffective emptying

• Rare in children + associated with upper respiratory infections, gastroenteritis, scarlet fever and Kawasaki syndrome

• In most cases, it resolves spontaneously

• Cholecystectomy if symptoms intensify

Spleen Congenital Splenic cyst

Primary Splenic cysts containing an epithelial lining (Epidermoid cysts) May present as symptoms related to their size with gastric compression or

pain, an abdominal mass, rupture, or infection with abscess. Simple cysts less than 5 cm may be observed, but cysts that are enlarging,

symptomatic, or larger than 5 cm require treatment.

Spleen Congenital Splenic cyst

1. Percutaneous aspiration and sclerosis utilizing alcohol or other agents have been reported with variable success.

2. Marsupialization is commonly performed but has been associated with a high recurrence rate.

3. Partial splenectomy with cyst resection.

4. Splenectomy

Pancreas

Pseudocyst Congenital cyst

Retention C

Cystadenoma

• Pseudocyst most commonly secondary to pancreatitis resulting from blunt abdominal trauma.

• Congenital pancreatic cysts are rare and probably caused by anomalous of the ductal system. They are usually unilocular and located in the body or tail of the pancreas.

• Retention cysts which represent ectasia of the pancreatic ducts

Pancreas


Retention C

Cystadenoma

• Two-thirds of pancreatic Pseudocyst in children occur in the body or tail of the pancreas, and most are unilocular

• The remainder might track along tissue planes and occur within the peritoneal space, retroperitoneal, or even mediastinum.

• They are thin-walled and usually have no connection with the pancreatic duct.

Pancreas


Retention C Cystadenoma

• Is a very rare benign neoplasm in children that can be serous or mucinous

• The most common presenting symptom is abdominal distention

• Although the mucinous Cyst adenoma is a benign lesion, it is considered to be premalignant and should be completely resected

• The most common cause of Hydronephrosisin children is ureteropelvic junction obstruction caused by an aberrant renal artery, idiopathic stenosis, or aperistaltic ureteral segment.

Kidney/adrenal

Hydronephrosis Multicystic dysplastic kidney


• Other causes include: Posterior urethral valves Vesicoureteral reflux, Ectopic ureterocele Ureterovesical junction obstr

• US demonstrates marked dilatation of the renal pelvis and calyces of the affected kidney. Depending on the cause, the ureter might also be dilated and tortuous.

Kidney/adrenal

Hydronephrosis Multicystic dysplastic kidney


Kidney/adrenal Hydronephrosis Multicystic dysplastic kidney Multilocular cystic nephroma Adrenal hemorrhage

• Common neonatal renal mass• Caused by pyeloinfundibular atresia• US; cysts randomly distributed throughout the kidney without a

dominant medial cyst or evidence of communication between cysts.

Kidney/adrenal Hydronephrosis Multicystic dysplastic kidney Multilocular cystic nephroma Adrenal hemorrhage

• Renal scintigraphy can distinguish MCDK from severe hydronephrosis by confirming absence of function.

• Contralateral abnormalities such as VUR or PUJ obst might be seen in up to 40%

• MCDK usually involutes, but it can occasionally grow.

• Surgery indicated at 24 months of life if the MCDK is not involuted



• Rare cystic renal tumor• Characterized by a well circumscribed

encapsulated mass that contains multiple non communicating fluid filled locules

• Two-thirds of these tumors occur in children younger than 2 years, and there is a male predominance . It is rarely bilateral

• Present with a painless abdominal mass, but hypertension, hematuria and flank pain might be seen.

• Risk of sarcomatous degeneration in adults if it is not removed



• The most common adrenal mass in neonates.

• Trauma and stress at birth, anoxia, and systemic disease have been implicated in its development.

• Neonates can present with palpable flank mass, anemia, prolonged jaundice and hemorrhagic shock

• Rx: follow up for Expected Resolution

Gastrointestinal Mesenteric cyst \ Lymphangioma Enteric/duplication cyst


• Found in the small bowel mesentery.

• One-third are found in children <15 y

• More likely to present acutely with pain, palpable mass, anorexia, vomiting or fever.

• Radiological; Thin-walled uni- or Multilocular cyst that displaces adjacent structures to the periphery of the abdomen

• If hemorrhage occurred, debris might be seen within the fluid.



• When lymphangioma are very large they can be difficult to distinguish from severe ascitis

• Differentiating features suggesting ascites rather than lymphangioma include separation of bowel loops, fluid collecting in the perihepatic spaces and cul-de-sac, and lack of septations

Gastrointestinal Mesenteric cyst \ Lymphangioma Enteric/duplication cystOmental cyst Meconium Pseudocyst

• Spherical or tubular mass adherent to the GI tract that sometimes communicates with it.

• Lined with intestinal epithelium, and there is smooth muscle within its wall.

• The most common location is the terminal ileum, but it can occasionally be seen at the distal esophagus, stomach and duodenum, and elsewhere.

• Most patients present within the first year of life, with symptoms including GI obstruction, palpable mass and abdominal distention.

Gastrointestinal Mesenteric cyst \ Lymphangioma Enteric/duplication cystOmental cyst Meconium Pseudocyst

• US differentiate cystic from solid tumors and demonstrates the intimate association between duplication and the bowel wall.

• Reliable indicators of a duplication cyst include an inner echogenic rim of intestinal mucosa surrounded by a characteristic hypoechoic rim of muscle in the wall, indicating the various layers of the intestine, and internal debris or hemorrhage.

• CT can define its precise anatomic location.• In 15–20% of cases, the cyst contains gastric

mucosa that can bleed; Tc-99m pertechnetate scanning will define this



• A rare cystic lesion of omentum derived from lymphatic tissue.

• Two-thirds of patients presenting before 10 years of age.

• If large can leads to compressive symptoms of the respiratory, GI or urinary tracts.

• Patients sometimes present acutely if there is volvolus, sudden hemorrhage into the cyst or infection.

• An anterior well-defined fluid collection with sediment, a thin wall, and internal septations is the typical appearance of an omental cyst.

http://www.biomedcentral.com/1471-2482/6/18/figure/F4



• Results from inutero bowel perforation

• Meconium deposited in the peritoneal space can form a large meconium- filled cyst lined by a thick inflammatory and fibrotic membrane

• Calcifications and plaques might develop as well

• Ovarian cysts can be classified as physiological, functional or neoplastic.

• Physiological cysts include the normal follicle and corpus luteum and are less than 3 cm in diameter.

• Functional cysts, including failed involution of the follicle or corpus luteum, account for one-fourth of primary ovarian masses, while cystic neoplasms account for about one-half.

Genitourinary/ovary



• US shows a large anechoic mass that can extend from the true pelvis to the liver. The daughter cyst sign is diagnostic of an ovarian cyst in neonates, infants, and young children.

• Typical presenting symptoms include lower abdominal pain and abdominal distention. Torsion can result in acute onset of pain..

Genitourinary/ovary



• Benign cystic teratoma is the most common cystic ovarian neoplasm in children.

• Two thirds of teratomas are complex masses, and the remainder are solid or completely cystic.

• They might contain calcification, fat or hair.

• They appear as a thin-walled cystic mass with multiple septations and intermixed solid areas.

Genitourinary/ovary

Functional cyst Teratoma/dermoid Cystadenoma Hematocolpos Urachal cyst

Benign ovarian teratoma in a 5-year-old girl. a predominantly cystic mass with calcifications (arrow) in the left pelvis. Just anterior to the calcifications, fat is noted within the mass.

• Serous or mucinous Cystadenoma of the ovary, benign or malignant, are rare in children.

• They arise from mullerian germinal epithelium.

• Patients are usually post-pubertal.

• Cysts are large, averaging more than 20 cm in diameter at diagnosis.

• Papillary processes might be seen along the wall.

Genitourinary/ovary


Mucinous Cystadenoma in a 12-year-old girl. a large mass arising from the pelvis. The mass shows central cystic areas and papillary processes along the inner wall

• A fluid-filled, dilated vagina resulting from imperforate hymen, cervical stenosis, atresia or agenesis.

• US: large spherical or ovoid cystic pelvic midline mass with thin wall and internal echoes representing mucoid material and cellular debris.

• The average size is 5–9 cm.

Genitourinary/ovary



• Develop if the urachus closes at both the umbilicus and the bladder but remains patent in between.

• Most become symptomatic when enlarged and infected with signs of lower abdominal pain, fever, and dysuria.

• US shows a thick-walled cystic mass with mixed echogenicity above the bladder.

• CT characteristics include midline location deep to the rectus abdominus muscle and a conical shape extending toward the bladder dome.

Genitourinary/ovary



• Serous or mucinous Cystadenoma of the ovary, benign or malignant, are rare in children.

• They arise from mullerian germinal epithelium.

• Patients are usually post-pubertal.

• Cysts are large, averaging more than 20 cm in diameter at diagnosis.

• Papillary processes might be seen along the wall.

Genitourinary/ovary


Mucinous Cystadenoma in a 12-year-old girl. a large mass arising from the pelvis. The mass shows central cystic areas and papillary processes along the inner wall

Miscellaneous Abscess Teratoma Necrotic or cystic changes in tumors

(Wilms, neuroblastoma, etc.)


• Appendicular abscess In children are more frequently than in adults

• Perforated appendicitis can lead to subcapsular hepatic, pelvic or right lower quadrant abscess.

• US and CT show a loculated, often pericecal fluid collection with mass effect on adjacent bowel loops. Surrounding echogenic fat is less common in children than in adults because of the lack of intraperitoneal fat in children.

• A calcified appendicolith within the mass is diagnostic of periappendiceal abscess.

Thick-walled, rim-enhancing abscess in the pelvis with surrounding inflammation

Miscellaneous Abscess Teratoma Necrotic or cystic changes in tumors

(Wilms, neuroblastoma, etc.)


• Abdominal/retroperitoneal teratoma is an uncommon tumor accounting for 2–4% of all germ cell tumors. These tumors occur mainly in children, and 80% are benign. Mature, immature and malignant types have been described.

• Most children present with a palpable abdominal mass.

• US and CT will demonstrate a solid, multicystic or unilocular mass. Calcification might be seen in three fourths of cases and provides a strong clue to the diagnosis. Fat can be seen by CT in about half of cases.

RPT in a 2-year-old girl.fluid, fat (arrow) and calcification (open arrow) within the mass

Miscellaneous Abscess Teratoma

Necrotic or cystic changes in tumors (Wilms, neuroblastoma, etc.)


• Resulting from VP shunt, with a frequency of approximately 3%.

• Present with abd pain, distention, or mass. • Larger Pseudocyst tend to be sterile,

whereas smaller Pseudocyst are more often infected.

• US and CT show a well-defined cystic mass with the shunt catheter tip identified within the collection.

• A noninfected shunt Pseudocyst appears homogeneous, while an infected collection will demonstrate septations, a fluid-fluid level, or internal debris.

Miscellaneous Abscess Teratoma

Necrotic or cystic changes in tumors (Wilms, neuroblastoma, etc.)


• Develops from pluripotential cells at Hensen’s node. It is more common in girls and is most often external.

• Usually discovered during prenatal US.• Might be completely internal, presenting

with obstructive symptoms from the mass displacing or obstructing the rectum and/or bladder.

• Cystic tumors carry a better prognosis than do solid, hypervascular tumors.

MRI : A cystic Sacrococcygeal teratoma in a newborn boy; displacing the bladder anteriorly (F Foley). An intraspinal component of the cystic mass (arrow) can be seen

CYSTIC ABDOMINAL MASSES IN CHILDREN

Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS

Associate Consultant Pediatric Surgery; KAAUH_ PNU

Thank you

abdominal cyst in children

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