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abdominal wall defects pp radiology pediatrics
- 1.
- 2. Topics • Abdominal walldefects • Omphalocele • Gastroschisis • Extrophy epispadius sprectrum • Body stalk anomalies • Disorder of biliary system liver and pancreas • Billary atresia • Choledocal cyst • Cholelithiasis and acute cholecystitis • Hepatic masses • Pancreatic masses • Burkitt lymphoma
- 3. Abdominal wall defects •omphalocele • Definition: A birth defect where a baby's intestines or other organs protrude through the abdomen at the belly button. • Development: Normally, the fetal gut herniates into the umbilical cord during development and then returns to the abdomen. In omphalocele, this retraction is interrupted. • Features: • Umbilical cord insertion at the apex of the hernia. • Three-layered membrane (peritoneum, Wharton's jelly, amnion) covering the defect.
- 4. Associated Conditions &Management • Associated Conditions: High risk of other birth defects, especially heart and gastrointestinal problems. • Prenatal Diagnosis: Detected by ultrasound. Serial ultrasounds monitor size, contents, and sac rupture.
- 5. Abdominal wall defects:Gastroschisis • Definition: A birth defect where the baby's intestines protrude through a small hole in the abdominal wall, typically to the right of the umbillicus • Key Features: • Location: Right of the umbilical cord insertion. • Membrane: No covering membrane. • Size: Usually smaller than 2 cm. • Associated Anomalies: Rare compared to omphalocele. Management: • Prenatal: Ultrasound diagnosis, fetal surveillance.
- 6. Abdominal wall defects:Cloacal Exstrophy • Definition: A rare and severe birth defect affecting the lower part of the body. • Pathogenesis: Results from an early breakdown of the cloacal membrane during embryonic development. • Manifestations: Involves the hindgut, bladder, genitalia, and often other organ systems. • Characteristic Feature: "Elephant trunk" appearance due to herniation of the hindgut and bowel between the everted bladder halves.
- 7. Abdominal wall defects:Cloacal Exstrophy Associated Conditions: • Renal anomalies (agenesis, dysplasia, ectopia) • Lower extremity defects • Ascites • Narrow thorax • Single umbilical artery • OEIS complex (Omphalocele, Exstrophy, Imperforate anus, Spinal anomalies)
- 8. Abdominal wall defects:Bladder Exstrophy • Definition: A rare birth defect where the bladder wall is exposed outside the body. • Pathogenesis: Results from an incomplete closure of the lower abdominal wall during embryonic development. • Manifestations: Involves the urinary bladder and external genitalia. • Key Features: • Eversion of the posterior bladder wall. • Absence of the anterior abdominal wall over the bladder.
- 9. Abdominal wall defects:Bladder Exstrophy Differential Diagnosis: • Omphalocele: Differentiate by the absence of the bladder and the superior position of the umbilical cord insertion. • Management: • Requires a multidisciplinary team approach. • Complex surgical reconstruction in stages. • Long-term follow-up for urinary incontinence and other complications. • Management of associated conditions (e.g., epispadias, inguinal hernias).
- 10. Epispadias, Prune BellySyndrome, and Other Abdominal Wall Defects • Epispadias: Abnormal opening of the urethra along the dorsal surface of the penis. • Prune Belly Syndrome: Deficiency of abdominal wall musculature with associated urinary tract abnormalities. • Pentalogy of Cantrell: Midline supraumbilical abdominal defect, sternal defect, diaphragmatic defects, cardiac abnormalities. • Body Stalk Anomalies: Malformation of thorax, abdomen, or both, often with craniofacial and limb defects.
- 11. Congenital Diaphragmatic Hernia •Definition: Herniation of abdominal contents into the chest cavity through a defect in the diaphragm. • Types: Bochdalek (posterior, more common, left-sided) and Morgagni (anterior, right-sided). • Complications: Lung hypoplasia, pulmonary hypertension. • Management: Prenatal diagnosis (ultrasound), fetal therapy (tracheal occlusion), postnatal surgical repair.
- 12. Biliary Atresia: Pathophysiologyand Clinical Presentation • Definition: A progressive cholestatic liver disease in infants characterized by the absence or severe destruction of extrahepatic bile ducts. • Pathophysiology: Likely an inflammatory process leading to bile duct fibrosis and obliteration. • Clinical Features: • Progressive jaundice • Dark urine • Acholic stools • Hepatomegaly • Splenomegaly • Pruritus (in later stages)
- 13. Imaging and Managementof Biliary Atresia • Diagnostic Imaging: • Ultrasound: Absence or small gallbladder, triangular cord sign. • Hepatobiliary scintigraphy: Confirms the diagnosis by demonstrating no radiotracer excretion into the bowel. • Management: • Kasai portoenterostomy: Surgical procedure to create a connection between the liver and the intestine. • Liver Transplantation: Indicated in cases with advanced liver disease unresponsive to the Kasai procedure. • Prognosis: Variable and depends on factors like age at diagnosis, response to treatment, and the presence of associated anomalies.
- 14. • Biliary Atresia:Pearls to Remember • ◾ An absent gallbladder or a gallbladder smaller than 15 mm in • length on USG despite adequate fasting • ◾ Triangular cord sign at the porta hepatis • ◾ Hepatobiliary scintigraphy excludes biliary atresia by • demonstration of radionuclide excretion into the small bowel
- 15. Choledochal Cyst • Definition:Congenital cystic dilation of the biliary tract. • Pathogenesis: • Likely multifactorial, including abnormal embryological development and pancreaticobiliary maljunction. • Associated with an increased risk of cholangitis, pancreatitis, and malignancy. • Clinical Presentation: • Abdominal pain (most common) • Jaundice • Fever (in cases of cholangitis) • Less commonly: Renal failure, portal hypertension (in Caroli disease)
- 16. Choledochal Cyst: ImagingEvaluation • Imaging Modality of Choice: Magnetic Resonance Cholangiopancreatography (MRCP) • Provides excellent visualization of the biliary and pancreatic ducts. • Accurately delineates cyst size, extent, and communication with the biliary tree. • Enables assessment of pancreaticobiliary maljunction. • Can detect associated biliary tract strictures and stones.
- 17. Choledochal Cyst: Classificationand Management • Left: Diagram illustrating the five types of choledochal cysts according to the Todani classification. • Right: Flowchart outlining the management algorithm for choledochal cysts. • Text: • Todani Classification: A widely used system for classifying choledochal cysts based on their anatomic location and extent. • Management: • Surgical excision: The mainstay of treatment to reduce the risk of complications. • Endoscopic techniques: May be considered in selected cases. • Long-term Surveillance: Regular follow-up to monitor for complications and recurrence.
- 18. • Choledochal Cyst:Pearls to Remember • ◾ The Todani classification divides choledochal cyst into five main • subtypes. • ◾ Type 5 cyst or Caroli disease complicated by hepatic fibrosis is • known as called Caroli syndrome. • ◾ The central dot sign refers to an ectatic intrahepatic biliary duct • surrounding an enhancing portal vein in Caroli disease.
- 19. Cholelithiasis in thePediatric Population: Risk Factors and Imaging • Definition: Cholelithiasis refers to the presence of gallstones within the gallbladder. • Risk Factors: • Hemolytic anemias (e.g., sickle cell disease, thalassemia) • Cystic fibrosis • Obesity • Prolonged parenteral nutrition • Certain genetic disorders • Imaging Modalities: • Ultrasound: The initial imaging modality of choice, demonstrating the presence, number, and size of gallstones. • Magnetic Resonance Cholangiopancreatography (MRCP): May be utilized in cases of equivocal ultrasound findings or to further evaluate biliary tract anatomy.
- 20. Acute Cholecystitis inPediatrics: Clinical Presentation and Management • Definition: Acute cholecystitis is an inflammatory condition of the gallbladder, typically caused by gallstone obstruction of the cystic duct. • Clinical Presentation: • Right upper quadrant abdominal pain (often colicky) • Fever • Nausea and vomiting • Leukocytosis • Management: • Medical Management: Antibiotic therapy, pain control, hydration. • Surgical Management: • Laparoscopic cholecystectomy is the gold standard treatment for symptomatic cholelithiasis and acute cholecystitis. • Indications for early surgical intervention include severe symptoms, biliary sepsis, and high-risk comorbidities.
- 21. Hepatic Hemangiomas inChildren: An Overview • Introduction: Hepatic hemangiomas are the most common benign liver tumors in children. • Classification: • Congenital: Present at birth, may exhibit rapid involution or remain static. • Infantile: Develop after birth, typically undergo a period of rapid growth followed by involution. • Clinical Significance: Range from asymptomatic to life-threatening, with potential for complications such as high-output cardiac failure and thrombocytopenia.
- 22. Imaging Evaluation ofHepatic Hemangiomas Ultrasound: • Variable echogenicity (hypoechoic to hyperechoic). • Prominent vascularity on Doppler ultrasound. • May demonstrate internal cystic areas or flow voids. • MRI: • T1 hypointense, T2 hyperintense on conventional sequences. • Characteristic progressive centripetal enhancement on dynamic contrast-enhanced MRI. • May demonstrate flow voids within the lesion.
- 23. Management of HepaticHemangiomas in Children • Image: • Left: Flowchart outlining the management algorithm for hepatic hemangiomas. • Right: Diagram illustrating potential complications of large or symptomatic hemangiomas (e.g., high-output cardiac failure). • Text: • Management: • Observation: For asymptomatic, small lesions. • Medical Therapy: Propranolol (first-line), corticosteroids for symptomatic lesions. • Interventional Procedures: Embolization, radiofrequency ablation in selected cases. • Surgical Resection: Reserved for complex cases or those with life-threatening complications. • Prognosis: Most infantile hemangiomas undergo spontaneous involution. Prognosis for congenital hemangiomas varies depending on size and associated complications.
- 24. Mesenchymal Hamartoma ofthe Liver • Definition: The second most common benign liver tumor in children. • Clinical Presentation: Typically presents in young children with painless abdominal distension. • Imaging: • Ultrasound: Multilocular cystic mass with low-level internal echoes. • MRI: Characteristic T1 hypointense and T2 hyperintense signal. • Management: Surgical resection is the treatment of choice due to the potential for growth and rare malignant transformation.
- 25. : Hepatoblastoma • Definition:The most common primary liver malignancy in children. • Risk Factors: Trisomy 18, Beckwith-Wiedemann syndrome, familial adenomatous polyposis. • Clinical Presentation: Abdominal distension, anorexia, weight loss, elevated serum alpha-fetoprotein (AFP) levels.
- 26. Hepatoblastoma: Imaging Featuresand Staging • Imaging: • CT: Heterogeneous enhancement, calcifications, possible vascular invasion. • MRI: Detailed assessment of tumor extent and vascular involvement. • PRETEXT Staging: A crucial prognostic factor for treatment planning. • Based on the number of contiguous liver segments free of tumor involvement. • Guides treatment decisions (surgery, chemotherapy, liver transplantation).
- 27. Hepatoblastoma: Management andPrognosis • Treatment: • Surgery: Primary treatment modality. • Neoadjuvant Chemotherapy: Used to reduce tumor burden prior to surgery. • Liver Transplantation: Considered for unresectable or recurrent disease. • Prognosis: Overall prognosis is favorable with advancements in treatment. Prognostic factors include tumor stage, age at diagnosis, and response to chemotherapy
- 28. • Hepatoblastoma: Pearlsto Remember • ◾ Most common pediatric hepatic malignancy occurring before the • age of 5 years • ◾ Well-defined tumors with lobulated contours and heterogeneous • enhancement • ◾ Calcifications are present in more than half of these tumors. • ◾ The PRETEXT classification is used for risk stratification.
- 29. Hepatocellular Carcinoma (HCC)in Children • Definition: Primary liver cancer arising from hepatocytes. • Risk Factors: Biliary atresia, glycogen storage disease, α1-antitrypsin deficiency, Wilson disease. • Clinical Presentation: Abdominal distension, weight loss, anorexia, elevated serum alpha-fetoprotein (AFP) levels. • Imaging: • CT: Hypervascular mass with variable enhancement patterns. • MRI: Characterization of tumor extent and vascular invasion.
- 30. Fibrolamellar Hepatocellular Carcinoma •Definition: A rare variant of HCC with a distinct clinical and pathological profile. • Key Features: • Occurs in adolescents and young adults. • Typically lacks a background liver disease. • Characterized by a central fibrous scar (hypointense on T2-weighted MRI, often calcified). • Normal or only mildly elevated AFP levels.
- 31. Undifferentiated Embryonal Sarcomaof the Liver • Definition: A rare, aggressive hepatic malignancy with a predominantly mesenchymal origin. • Clinical Presentation: Abdominal distension, weight loss, anorexia. • Imaging: • Typically appears as a large, cystic mass on imaging. • May demonstrate internal septations, hemorrhage, and areas of solid enhancement. • Prognosis: Aggressive tumor with a high risk of recurrence and metastasis.
- 32. Management of HepaticMalignancies in Children • Treatment Strategies: • Surgery: Primary treatment option for resectable tumors. • Chemotherapy: Neoadjuvant and adjuvant chemotherapy for hepatoblastoma. • Liver Transplantation: Considered for unresectable or recurrent disease. • Prognosis: Varies depending on the specific tumor type, stage, and response to treatment. • Importance of Multidisciplinary Care: Collaboration between oncologists, surgeons, radiologists, and pathologists is crucial for optimal patient outcomes.
- 33. Pancreatic Pseudocysts andCystic Neoplasms • Pseudocysts: Most common type of pancreatic cyst in children, typically resulting from pancreatitis or trauma. • Cystic Neoplasms: • Solid and Papillary Epithelial Neoplasm (SPEN): Most common pancreatic neoplasm in children, predominantly affecting females. • Congenital Cystic Neoplasms: Rare, may be associated with genetic syndromes.
- 34. Pancreatoblastoma and OtherPancreatic Tumors • Pancreatoblastoma: Rare but aggressive malignant tumor; may be associated with Beckwith-Wiedemann syndrome. • Other Tumors: • Endocrine tumors (insulinomas, gastrinomas). • Neuroendocrine tumors. • Lymphoma (e.g., Burkitt lymphoma). • Metastatic disease.
- 35. Burkitt Lymphoma: AHigh-Grade Lymphoma with Pancreatic Involvement • Definition: An aggressive B-cell non-Hodgkin lymphoma. • Clinical Presentation: Abdominal pain, constipation, fever, weight loss. • Imaging: • CT: Demonstrates extranodal masses, bowel wall thickening, and potential for solid organ involvement (liver). • PET/CT: Valuable for assessing disease extent and response to treatment. • Management: Chemotherapy is the mainstay of treatment.
Editor's Notes
- #3 Normally during development, the fetal bowel loops herniate through the developing anterior abdominal wall into the umbilical cord during the 6th to 10th weeks of gestation (see Fig. 84.1). Interrupted bowel retraction into the abdominal cavity results in omphalocele [47]. Understanding this process explains the characteristic umbilical cord insertion at the apex of the hernia sac and the three-layered membrane composed of peritoneum, Wharton jelly, and amnion covering the defect (Fig. 84.48) [48]. Associated congenital anomalies are frequently noted in cases of omphalocele, the most common of which are cardiac and GI in nature. The condition is associated with a high risk of underlying genetic abnormalities. In utero detected omphalocele require serial ultrasound monitoring to evaluate the size of the omphalocele and its contents, exclude in utero sac rupture, and determine the route of delivery [
- #5 Gastroschisis is a paraumbilical ventral body wall defect with secondary midgut herniation into the amniotic cavity. This defect is most commonly to the right of cord insertion, not covered by amniotic membrane, and characteristically smaller than 2 cm (Fig. 84.49). Compared with omphalocele, gastroschisis is rarely associated with congenital abnormalities [50]. Also, underlying genetic abnormalities are uncommon in cases of isolated gastroschisis. Postnatal outcome is mainly dependent on the bowel condition at birth with 95% survival rate in cases of uncomplicated gastroschisis.
- #6 Cloacal Exstrophy Cloacal exstrophy occurs secondary to early breakdown of the cloacal membrane, resulting in a severe abnormality involving the lower half of the body, namely the hindgut, bladder, and genitalia. The associated characteristic elephant trunk appearance represents herniation of the hindgut and bowel between the everted two bladder halves [51]. Cloacal exstrophy is frequently associated with other renal anomalies, including renal agenesis, cystic dysplasia, and ectopia. Associated extrarenal abnormalities include lower extremity defects, ascites, a narrow thorax, and a single umbilical artery. Cloacal exstrophy can also be a part of the Omphalocele, Exstrophy Bladder, Imperforate anus, and Spinal anomalies (OEIS) complex, in which cloacal exstrophy is associated with omphalocele, imperforate anus, and spinal defects. The neonatal prognosis depends on the severity of the defect and associated malformations [
- #8 Bladder exstrophy occurs from incorrect retraction of the cloacal membrane, leading to eversion of the bladder plate. The resultant abnormality involves only the urinary bladder and external genitalia (Fig. 84.50). The normal hindgut differentiates this anomaly from cloacal exstrophy. To differentiate bladder exstrophy from omphalocele, it is important to document the absence of the bladder and the position of the umbilical cord insertion superior to the ventral abdominal wall defect [51].
- #12 Biliary Atresia Biliary atresia is thought to arise secondary to an in-utero progressive postinflammatory process with subsequent absent or severely deficient extrahepatic biliary tree. It usually presents in the neonatal period with progressive obstructive jaundice. Death is expected by 2 years of age in untreated cases because of the development of secondary biliary cirrhosis [39]. In 20% of cases, biliary atresia is associated with situs anomalies and is called biliary atresia-splenic malformation syndrome. The gold standard to diagnose biliary atresia is biopsy and intraoperative cholangiogram. Imaging helps establish the diagnosis, especially differentiating it from neonatal hepatitis, and allows establishing to an appropriate plan of management [60]. Imaging is the initial imaging modality of choice in cases of neonatal jaundice. ◾ An absent gallbladder or a gallbladder smaller than 15 mm in length on ultrasound despite adequate fasting is 84.8% sensitive and 76.9% specific for biliary atresia. ◾ On the other hand, a triangular focus of echogenic tissue at the porta hepatis, also known as the triangular cord sign, is reported to be 96% accurate, 23.3% to 100% sensitive, and 89% to 100% specific for diagnosing biliary atresia [61]. ◾ Hepatobiliary scintigraphy can exclude biliary atresia by demonstration of radionuclide excretion into the small bowel (Fig. 84.52) [62,63]. Cases of neonatal hepatitis demonstrate poor hepatic radiotracer uptake and delayed radiotracer excretion into the bowel. FIGURE 84.52 Transverse grayscale ultrasound from a 5-week-old boy with conjugated hyperbilirubinemia shows absent gallbladder despite adequate fasting in biliary atresia (A). Planar anterior and posterior hepatobiliary iminodiacetic acid scan acquired 24 hours after injection of the radiotracer shows adequate radiotracer uptake in the liver without contrast excretion into the duodenum (B). Patients with biliary atresia are commonly treated surgically through creating a hepatic portoenterostomy, also known as the Kasai procedure. Cases that develop biliary cirrhosis and end-stage liver disease usually require liver transplantation [64].
- #15 Left: Diagram illustrating the normal biliary tree and a choledochal cyst. Right: Histological image demonstrating dilated bile ducts (if available).
- #16 Left: Ultrasound image showing a type I choledochal cyst. Right: MRCP image demonstrating a type III choledochal cyst (choledochocele).
- #17 Left: Diagram illustrating the five types of choledochal cysts according to the Todani classification. Right: Flowchart outlining the management algorithm for choledochal cysts.
- #19 Left: Ultrasound image demonstrating multiple gallstones within the gallbladder (similar to Fig. 84.58). Right: Diagram illustrating risk factors for gallstone formation in children. Cholelithiasis and Acute Cholecystitis Major risk factors for gallstones in the pediatric population include hemolytic anemias, cystic fibrosis, obesity, and parenteral nutrition [71]. Similar to the adult population, USG is the mainstay for biliary stones evaluation (Figs. 84.58 and 84.59). MRCP can be used in equivocal cases and shows calculi as areas of signal void within the biliary tree. In contrast to the adult population, 30% of pediatric cases are acalculous in nature. Cholecystectomy is the treatment of choice for symptomatic gallstone disease and in patients with high-risk comorbidities such as hemolytic anemias [72]. FIGURE 84.58 Longitudinal grayscale ultrasound from a 16-year-old young woman with sickle-cell anemia shows multiple calculi within a distended gallbladder (GB). FIGURE 84.59 Transverse grayscale ultrasound image from a 12- year-old girl with shock of unclear origin demonstrates a grossly thickened gallbladder wall (GB) and pericholecystic fluid in keeping with acalculous cholecystitis
- #20 Image: Ultrasound image demonstrating gallbladder wall thickening and pericholecystic fluid (similar to Fig. 84.59).
- #21 Left: Histological image of a hepatic hemangioma (if available). Right: Diagram illustrating the spectrum of hepatic masses in children.
- #22 Left: Ultrasound image demonstrating a large, heterogeneous hepatic hemangioma with prominent vascularity. Right: MRI images showing a hepatic hemangioma with characteristic signal intensity and progressive centripetal enhancement.
- #23 Image: Left: Flowchart outlining the management algorithm for hepatic hemangiomas. Right: Diagram illustrating potential complications of large or symptomatic hemangiomas (e.g., high-output cardiac failure).
- #24 Image: Left: Ultrasound image demonstrating a multilocular, cystic mesenchymal hamartoma. Right: Diagram illustrating the spectrum of hepatic masses in children.
- #25 Left: Photomicrograph of a hepatoblastoma (if available). Right: Diagram illustrating the spectrum of hepatic malignancies in children.
- #26 Left: CT scan demonstrating a large, heterogeneous hepatoblastoma with areas of calcification. Right: Diagram illustrating the PRETEXT staging system for hepatoblastoma.
- #27 Left: Diagram illustrating the treatment algorithm for hepatoblastoma. Right: Illustration of potential metastatic sites (e.g., lungs).
- #29 Left: Diagram illustrating risk factors for HCC in children (e.g., biliary atresia, glycogen storage disease). Right: CT scan demonstrating a solitary, hypervascular hepatic mass suggestive of HCC.
- #30 Left: MRI images demonstrating a fibrolamellar HCC with a central hypointense scar. Right: CT scan showing a fibrolamellar HCC with calcifications within the central scar.
- #31 Left: Ultrasound image demonstrating a cystic-appearing embryonal sarcoma. Right: MRI images showing a heterogeneous, lobulated embryonal sarcoma with areas of hemorrhage.
- #32 Left: Flowchart illustrating the treatment algorithm for hepatoblastoma. Right: Diagram illustrating potential sites of metastasis for hepatic malignancies (e.g., lungs, bone).
- #33 Left: Ultrasound image demonstrating a large pancreatic pseudocyst. Right: CT scan showing a well-defined, cystic pancreatic mass (likely a cystic neoplasm).
- #34 Left: CT scan demonstrating a pancreatoblastoma with heterogeneous enhancement and possible calcifications. Right: Diagram illustrating the spectrum of pancreatic tumors in children.
- #35 Left: CT scan demonstrating diffuse gastric wall thickening due to Burkitt lymphoma. Right: PET/CT scan showing intense FDG uptake in multiple abdominal lymph nodes, suggestive of Burkitt lymphoma.