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Pediatric abdominal mass

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1 Pediatric abdominal mass
Zaid yaseen Adnan alsayyed Saud alzubaidi Saud albishtawi

2 Introduction An abdominal mass in an infant or a child is a challenging diagnostic problem. It includes a spectrum of lesions of diverse origin and significance. They may occur at any age - from the newborn period through adolescence and have a wide range of clinical presentations

3 Approach to the patient

4 Approach to the patient
1) supportive care and resuscitation in an ill-appearing child, including fluid therapy in the presence of dehydration. 2) supplemental oxygen and assisted respiration in patients with respiratory compromise. 3) control of blood pressure in patients with symptomatic hypertension. Once the child is stabilized, additional studies may be undertaken

5 Assessment The patient’s age is one of the most important factors that help narrow the potential etiologies of an abdominal mass in a child as likely etiologies differ between neonates and infants/children. the work up of a pediatric patient with a suspected abdominal mass includes : a careful and detailed history abdominal examination Laboratory Studies: relevant urine and hematological analysis Imaging techniques

6 History Important history components include the length of time since the mass was found, rapidity of growth, and signs of gastrointestinal or genitourinary obstruction. The presence of constitutional symptoms, such as pallor, anorexia, fever, or weight loss, may point toward a malignant lesion, but these findings are not specific. In neonates and young infants, information from prenatal ultrasonography examinations and other prenatal interventions during pregnancy can be helpful. The presence of oligohydramnios or polyhydramnios on prenatal ultrasound might suggest nonacquired disease processes affecting the developing fetal renal system.

7 Physical examination A thorough physical examination can be difficult in the very young or uncooperative child. A parent’s lap is a good substitute for the examination table, especially with anxious and apprehensive young patients. Inspection: The infant or toddler should lay supine with the abdomen exposed for inspection for protrusion, bulging, or asymmetry. Palpation: The examiner’s hands should be warm when touching the patient to minimize discomfort and opposition to the examination. Distraction by the physician or parent can be helpful for a more relaxed abdomen.

8 Physical examination Initial light palpation of all 4 quadrants and the flank areas is essential. A second circuit of palpation can allow deeper examination. The examination should also assess for guarding or tenderness indicative of an inflammatory or infectious process. Percussion helps detect organ or mass size and assists in differentiating the underlying components. Solid masses and fluid-filled cysts are typically dull to percussion, while air-filled structures are tympanic. Auscultation for bowel sounds is necessary to assess for intestinal obstruction.

9 Laboratory Studies The choice of laboratory studies is guided by the type of symptoms and signs that are involved. Studies to be considered include: Urinalysis Complete blood cell count Blood urea nitrogen and creatinine Electrolytes Hepatic transaminases and bilirubin

10 Imaging Techniques Ultrasound
The diagnostic study of choice in most cases of abdominal mass is ultrasound. Exceptions include clinically diagnosed constipation, intrauterine pregnancy, and appendicitis, where further studies are generally unnecessary, or intussusception, where a contrast enema is both diagnostic and therapeutic. Ultrasound examination provides information regarding the location size and character (cystic versus solid, homogeneous versus heterogeneous, calcified) of the abdominal mass and should be the initial study in most cases.

11 Imaging Techniques Abdominal radiographs
Although these are frequently obtained, provide little information in a relatively asymptomatic child. Some exceptions are the use of radiographs to detect calcifications in a mass, organomegaly, or displacement of the intestines, all of which may help narrow the differential diagnosis. Abdominal Masses Commonly Associated with Calcification Neuroblastoma Teratoma Ovarian Sacrococcygeal Adrenal hematoma Hepatic hemangioma Meconium peritonitis

12 Imaging Techniques Abdominal CT
It provides more detailed anatomic information than ultrasound, should be reserved for further delineation of certain masses but may be a substitute when ultrasound is not helpful. Intravenous urography Once the initial study of choice, has now been supplanted by the preceding methods.

13 Abdominal masses and age
Pediatric abdominal masses are broadly classified as: a) Neonatal ( birth-28 days of life) B)infants and older children

14 Neonatal Abdominal Masses
Abdominal masses in the newborn period from birth to one month of age are predominantly benign lesions usually representing defects in embryonic development. 65% of neonatal abdominal masses are retroperitoneal of which 55% are renal in origin. Malignant neoplasms are unusual at this age and the prognosis in general is good

15 Abdominal Masses in Infants and Children
Beyond the newborn period, there is a significant increase in malignant tumors and important differences in the frequency of specific abdominal masses. The incidence of retroperitoneal masses increases significantly , reflecting dramatic increase in the frequency of non-renal masses like primary Neuroblastoma. Renal masses occur with about the same frequency and although many are still benign, the increased incidence of Wilm's tumor: nephroblastoma is a cancer of the kidneys that typically occurs in children and relative decrease in cystic abnormalities alter their prognosis.

16 Abdominal masses are most common in children under the age of 5 years
Most abdominal masses in neonates are retroperitoneal, of kidney origin and are not malignant The older the child the more likely the mass represents a malignant process

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18 URINARY BLADDER DISTENTION
NORMAL MASSES CONSTIPATION URINARY BLADDER DISTENTION

19 CONSTIPATION Differential Diagnosis : Infectious causes. Drugs.
Toxic causes. Neoplasms. Anal lesions. Metabolic disorders. Functional (most common). Inflammatory and immune disorders. Neurogenic causes.

20 FUNCTIONAL CONSTIPATION
Usually chronic (idiopathic, nonorganic). 25% of all cases of constipation. Pathophysiology of the disease : Positive feedback type mechanism : Predisposing factor  retained stool in distal colon loses water  fecal motility slows  more water loss  hard stool  painful defecation  more stool retention , which causes : Acquired mega colon (distended distal colon ). Tonically contracted internal sphincter. Loss of ability to sense rectal fullness. Classic sign  large infrequent stool blogging the rectum. Encorporesis (is voluntary or involuntary fecal soiling in children who have usually already been toilet trained). Rectal prolapse in severe constipation.

21 FUNCTIONAL CONSTIPATION
Evaluation : History. Physical examination : Palpable mass is present. PR  inspect for fissures and hemorrhoids.  large amount of stool is found. Should check for occult blood. Treatment : Dietary changes  mild constipation : give stool softeners.  severe constipation : disimpaction followed by maintenance lasting for months to years. Behavioral modification involves unhurried toilet sitting for 10 minutes, two to three times a day after meals .

22 URINARY BLADDER DISTENTION
Might be caused by : Obstruction (mass, stricture). Neurologic (spinal cord injury). Voluntary (due to dysuria). Evaluation : History  ask about urinary symptoms and trauma to the spinal cord. Physical examination  smooth suprapubic mass on palpation. Treatment : Perform urethral catheter . Treat the underlying cause .

23 CYSTS MESENTRIC CYST CHOLEDOCAL CYST

24 MESENTRIC CYST Definition : Clinical picture : Evaluation :
Abdominal cysts that may occur in any part of the mesentery from the duodenal mesentery to the rectal mesentery Most frequently cysts are localized in small bowel mesentery (ileum in 60%) and meso colon (ascending colon in 40%). Clinical picture : Mesenteric cysts rarely cause abdominal symptom, In symptomatic cases diverse unspecific symptoms may occur most frequently present symptom is chronic undefined abdominal pain. Evaluation : Physical exam  palpable, partly movable and painless abdominal mass. Treatment : Surgical enucleation of the cyst is therapeutic method of choice.

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26 CHOLEDOCAL CYST Definition : Clinical features :
Cyst of the choledochal duct ( common bile duct ). Approximately 1 in live births More common in females (3-4:1). Possible genetic influence, common with other anomalies (congenital hepatic fibrosis, intrahepatic biliary cysts, and polycystic kidney disease). Clinical features : Mass. Jaundice. RUQ pain.

27 CHOLEDOCAL CYST Classification : “Todani classification”
Type I - Cystic or fusiform dilatation of the CBD, most frequent type (90-95% of the cases). Type II - Diverticulum of the CBD, with normal size CBD. Type III - Choledochocele, a cystic dilatation of the distal intramural portion of the CBD, typically protruding into the second portion of the duodenum. Type IV - Cystic or fusiform dilatation of the CBD associated with cystic, fusiform, or saccular dilatation of intrahepatic bile ducts, also termed form FRUSTE. Type V - Cystic, fusiform, or saccular dilatation of the intrahepatic bile ducts associated with a normal CBD; may be associated with hepatic fibrosis (referred to as Caroli disease).

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29 CHOLEDOCAL CYST Treatment : Type I-IV: Type V: Cystojejunostomy
Total Excision and Cystojejunostomy Biliary reconstruction can be performed with a Roux-en-Y hepaticojejunostomy as high as possible, near the hilum of the liver. Type V: Hepatic Lobectomy

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31 HERNIAS UMBILICAL HERNIA INGUINAL HERNIA

32 UMBILICAL HERNIA Normal umbilicus : Umbilical hernia :
Umbilical cord consists of two arteries and one vein surrounded by gelatinous substance. Umbilical cord is cut at birth and the remnant dries and falls off after day 7 – 21 spontaneously . Umbilical hernia : Its is an out pocketing of layers of skin, fascia, and/or peritoneum. This presents as a bulge at the umbilicus. It is caused by failure of the facial opening to close completely. Most commonly found in : African American infants . Premature infants . Infants with other medical conditions such as congenital hypothyroidism .

33 UMBILICAL HERNIA Treatment :
Most defects close spontaneously by the age of 5 years . Incarceration or strangulation rarely occurs . The likelihood of closure is inversely related to the size of the hernia, with most small- to moderate-size hernias closing spontaneously . Treatment : If the hernia is < 2 cms  closes spontaneously . If the hernia is > 2 cms, if it persists for 5 years  hernial repair .

34 SHOULD BE DIFFIRENTIATED FROM HYDROCELE.
INGUINAL HERNIA SHOULD BE DIFFIRENTIATED FROM HYDROCELE. They both result from failure of closure of processus vaginalis during fetal life but hernial sac contains loops of intestines and hydrocele contains fluids.

35 Inguinal Hernia Hydrocele Treatment : Direct : 1-5% of children .
More common in males . Indirect : Bulge at the inguinal canal . Present either at rest or when straining . Treatment : Surgical repair to decrease risk of incarceration or strangulation . Scrotal mass that transilluminates when not with hernia. May be communicating with the peritoneal cavity or noncummunicating . They resolve spontaneously but if not after 1-6 months the patient should be referred to a surgeon.

36 also known as infantile hypertrophic pyloric stenosis (IHPS)

37 Environmental Factors
Pyloric stenosis is a narrowing of the pylorus, Diffuse hypertrophy and hyperplasia of the smooth muscle of the antrum of the stomach and pylorus proper narrow the channel, which then can become easily obstructed. This causes a functional gastric outlet obstruction. outflow obstruction stomach musculature Gastritis with becomes prolonged vigorous peristalsis hypertrophied and dilated stasis Dehydration as a result of vomiting (Hypochloremic alkalosis). Causes: No definitive cause for HPS has been found. (multifactorial) Environmental Factors infantile hypergastrinemia, abnormalities in the myenteric plexus innervation, cow's milk protein allergy, and exposure to macrolide antibiotics. Hereditary Factors hypertrophic pyloric stenosis occurs in as many as 7% of infants of affected parents.

38 Age: Most infants who develop symptoms of pyloric stenosis are usually between 3 to 5 weeks. It is one of the most common causes of intestinal obstruction during infancy that requires surgery. Race : Hypertrophic pyloric stenosis is less prevalent among blacks, Asians, and Hispanics. Sex : HPS has a well-known predilection for occurring more often in males than in females, with reported ratios ranging from 2:1 to 5:1. First-born male children are believed to have the highest risk of developing the disease. Signs and symptoms Symptoms of pyloric stenosis generally begin around 3 weeks of age. They include: Vomiting: It is the first symptom of pyloric stenosis. At first it may seem that the baby is simply spitting up frequently, but then it tends to progress to projectile vomiting, in which the breast milk or formula is ejected forcefully from the mouth, in an arc. Projectile vomiting usually takes place soon after the end of a feeding, although in some cases it may be delayed for hours.

39 In some cases, the vomited milk may smell curdled because it has mixed with stomach acid. The vomit will not contain bile. A baby with HPS is usually hungry again soon after vomiting and will want to eat. 2. Changes in stools: Babies with pyloric stenosis usually have fewer, smaller stools because little or no food is reaching the intestines. Constipation or stools that have mucus in them may also be symptoms. 3. Failure to gain weight and lethargy: Most babies with pyloric stenosis will fail to gain weight or will lose weight. As the condition worsens, they are at risk for developing fluid and salt abnormalities and becoming dehydrated. 4. Dehydration: infants are less active than usual, and they may develop a sunken "soft spot" on their heads, sunken eyes, and their skin may appear wrinkled. Because less urine is made it may be more than 4 to 6 hours between wet diapers. 5. After feeds, increased stomach contractions may make noticeable ripples, or waves of peristalsis, which move from left to right over the baby's belly as the stomach tries to empty itself against the thickened pylorus.

40 On Physical Examination: An enlarged pylorus, classically described as an "olive" can be palpated in the right upper quadrant or epigastrium of the abdomen in 60-80% of infants. In order to assess the pylorus, the patient must be calm and cooperative. A pacifier or small amount of dextrose water may help. If the stomach is distended, aspiration using a nasogastric tube is necessary. The baby is dehydrated and has sunken eyes and depressed fontanelles. Investigations: Blood tests + serum electrolytes: The loss of electrolytes — such as sodium, potassium, magnesium and calcium — may be a sign of continual vomiting and dehydration. Ultrasound: It is the imaging modality of choice. Contrast X-ray: For this test, the baby swallows a small amount of a liquid that coats the stomach (barium) . This contrast material helps any abnormalities show up more clearly on an X-ray. (it reveals a swollen stomach and narrowed pylorus).

41 IHPS in a 7-week-old boy with a 4-week history of vomiting
IHPS in a 7-week-old boy with a 4-week history of vomiting. The pyloric canal is narrowed and elongated (arrows), and the base of the duodenal bulb is stretched by the pyloric mass. U/S: This infant was given glucose water before the scans. Enough fluid passed through the pylorus to outline the duodenal bulb (large arrow). The thickened pyloric wall (small arrows) is also visible.

42 Treatment IHPS is a medical emergency: Immediate treatment requires correction of fluid loss, electrolytes, and acid-base imbalance. The definitive treatment is corrective surgery: The Ramstedt Pyloromyotomy is the procedure of choice because it is easily performed and is associated with minimal complications. During which underlying antro-pyloric mass is split leaving the mucosal layer intact. The surgery can also be performed through laparascopy. This is a technique that uses a tiny scope placed in an incision in the belly button allowing the doctor to see the area of the pylorus. With the help of other small instruments placed in nearby incisions, the surgery is completed. Postoperative management: Continue IV maintenance fluid until the infant is able to tolerate enteral feedings. Feedings can begin within 8 hours following surgery. Addition of a histamine 2 (H2) receptor blocker sometimes can be beneficial. Treat persistent vomiting expectantly because it usually resolves within 1-2 days.

43 Intussusception Intussusception is a process in which a segment of intestine invaginates into the adjoining intestinal lumen, causing bowel obstruction. When this occurs, it can create a blockage in the bowel, with the walls of the intestines pressing against one another. This, in turn leads to swelling, inflammation, and decreased blood flow to the part of the intestines involved. The swelling can lead to perforation (tearing) and generalized abdominal infection. Shock and dehydration can occur very rapidly. Intussusception is the most common cause of intestinal obstruction in children between 3 months and five years of age. It is extremely rare in children under 3 months of age or in older children and adults. Intussusception occurs primarily in infants (boys more often than girls) but can also occur in adults and older children.

44 Intussusception presents in 2 variants:
Idiopathic intussusception: which usually starts at the ileocolic junction and affects infants and toddlers. Viral and bacterial infections of the intestine may possibly contribute to intussusception in infancy. Enteroenteral intussusception: (jejunojejunal, jejunoileal, ileoileal), which occurs in older children and it is associated with special medical situations (eg, Henoch-Schönlein purpura [HSP], cystic fibrosis, hematologic dyscrasias) or may be secondary to a lead point and occasionally occur in the postoperative period. lead points : Meckel diverticulum, Enlarged mesenteric lymph node, Benign or malignant tumors of the mesentery or of the intestine, including lymphoma, polyps, ganglioneuroma and hamartomas associated with Peutz-Jeghers syndrome. Mesenteric or duplication cysts Sutures and staples along an anastomosis. Intestinal hematomas secondary to abdominal trauma, Foreign body and Hemangioma.

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46 Risk factors: Age, Sex ,abnormal intestinal formation at birth (Malrotation, a condition present at birth in which the intestine doesn't develop correctly), A prior history of intussusception. Signs and Symptoms Severe abdominal pain that comes and goes (intermittent pain). Stool mixed with blood and mucus (sometimes referred to as "currant jelly" stool because of its appearance). A lump in the abdomen. Swollen (distended) abdomen. Vomiting, possibly vomiting bile (yellow-brown or greenish fluid) . Diarrhea, Fever, Dehydration , Lethargy. The classic triad: vomiting, abdominal pain, and passage of blood per rectum occurs in only one third of patients.

47 Physical Examination Right hypochondrium sausage-shaped mass and emptiness in the right lower quadrant (Dance sign). Abdominal distention, bowel sound diminished, absent or high pitch. If intestinal gangrene and infarction have occurred, peritonitis can be suggested on the basis of rigidity and involuntary guarding. Occult blood in the stools is the first sign of impaired mucosal blood supply. Later on, frank hematochezia and the classic currant jelly stools appear. Fever and leukocytosis are late signs and can indicate transmural gangrene and infarction. Unusual looking anus; may look like rectal prolapse. Shock like state with rapid pulse, pallor, and marked sweating. The infant can be pale, diaphoretic, and hypotensive if shock has occurred.

48 Diagnosis Complications
Ultrasonographic imaging has been found to have a high sensitivity and specificity in the detection of ileocolic intussusception. (97%) Abdominal radiographs can also reveal diagnostic characteristics of intussusception. The traditional and most reliable way to make the diagnosis of intussusception in children is to obtain a contrast enema (either barium or air). Contrast enema is quick and reliable and has the potential to be therapeutic. Enema is contraindicated in patients in whom bowel gangrene or perforation is suspected. Complications Ischemia in the affected part Tearing Peritonitis Shock Tissue Death Perforation

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50 Treatment From a clinical perspective, using a cutoff age of 3 years is helpful for dividing patients with intussusception into 2 groups: Patients aged 5 months to 3 years rarely have a lead point (ie, idiopathic intussusception) and are usually responsive to nonoperative reduction. Older children and adults more often have a surgical lead point to the intussusception and require operative reduction. Start IV resuscitation and NG decompression ASAP. Barium reduction : After the diagnosis is confirmed, intussusception is generally reduced (resolved) by gentle pressure exerted within the intestine, using barium or air enemas. Surgical : (manual or laproscopic) Children who are too ill to have this diagnostic procedure. Who may have significant infection in the abdomen. If intussusception does not resolve with the enema. If the child has several episodes of intussusception.

51 Hirschsprung Disease Hirschsprung disease results from the absence of enteric neurons within the myenteric and submucosal plexus of the rectum and/or colon. Enteric neurons are derived from the neural crest and migrate caudally with the vagal nerve fibers along the intestine. These ganglion cells arrive in the proximal colon by 8 weeks' gestation and in the rectum by 12 weeks' gestation. Arrest in migration leads to an aganglionic segment. This results in clinical Hirschsprung disease.

52 Clinical Presentation :
Sex 4 times more common in males than females. Age Nearly all children with Hirschsprung disease are diagnosed during the first 2 years of life. Approximately one half of children affected with this disease are diagnosed before they are aged 1 year. A small number of children with Hirschsprung disease are not recognized until much later in childhood or adulthood. A family history of a similar condition is present in about 30% of cases. Clinical Presentation : Symptoms include failure to pass MECONIUM (a newborn’s first stool) within 48 hours of birth, repeated vomiting and ABDOMINAL DISTENTION. Hirschsprung’s disease that involves only a short segment of the colon may remain undetected into childhood and even early adulthood, producing primarily symptoms of chronic CONSTIPATION and intermittent abdominal distress.

53 Physical Examination Physical examination in the newborn period is usually not diagnostic, but it may reveal a distended abdomen , abdominal mass ,and/or spasm of the anus. Children with Hirschsprung disease are usually diagnosed by age 2 years. Older infants and children with Hirschsprung disease usually present with chronic constipation. Upon abdominal examination, these children may demonstrate marked abdominal distention with palpable dilated loops of colon. Rectal examination commonly reveals an empty rectal vault and may result in the forceful expulsion of fecal material upon completion of examination. Less commonly, older children with Hirschsprung disease may be chronically malnourished and/or present with Hirschsprung enterocolitis.

54 Diagnosis: Laboratory Studies: Imaging Studies
CBC count: Order this test if enterocolitis is suspected. Elevation of WBC count should raise concern for enterocolitis. Imaging Studies Plain abdominal radiography: Perform this test with any signs or symptoms of abdominal obstruction. (distended bowel loops with a paucity of air in the rectum). Barium Enema: identifying a transition zone between a narrowed aganglionic segment and a dilated and normally innervated segment. The study may also reveal a nondistensible rectum, which is a classic sign of Hirschsprung disease. A transition zone may not be apparent in neonates, because of insufficient time to develop colonic dilation, or in infants who have undergone rectal washouts, examinations, or enemas. The definitive diagnosis of Hirschsprung disease rests on histological review of rectal tissue. Obtain tissue either by suction rectal biopsy or transanal wedge resection.

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56 Treatment Intravenous hydration, withholding of enteral intake, and intestinal and gastric decompression. Administer broad-spectrum antibiotics to patients with enterocolitis + biopsy + vaccinations. Surgical Care The surgical options vary according to the patient's age, mental status, ability to perform activities of daily living, length of the aganglionic segment, degree of colonic dilation, and presence of enterocolitis. Remove the defective segments of bowel, connecting the ends of healthy bowel to maintain the integrity of the lower intestinal tract. Sometimes the surgery takes place in two operations, the first to remove the defective bowel and the second to reconstruct the colon. A temporary COLOSTOMY allows digestive waste to leave the body during the interim HEALING phase. Most infants who undergo surgical repair before toxic megacolon develops heal completely and without residual complications. Hirschsprung’s disease often coexists with other congenital anomalies, notably DOWN SYNDROME.

57 Necrotizing enterocolitis
A gastrointestinal disease that mostly affects premature infants, NEC involves infection and inflammation that causes destruction of the bowel (intestine) or part of the bowel. Although it affects only 1 in 2,000 to 4,000 births, or between 1% and 5% of neonatal intensive care unit (NICU) admissions, NEC is the most common and serious gastrointestinal disorder among hospitalized preterm infants. NEC usually occurs within the first 2 weeks of life, usually after milk feeding has begun. The exact cause of NEC is unknown, but one theory is that the intestinal tissues of premature infants are weakened by too little oxygen or blood flow. So when feedings are started, the added stress of food moving through the intestine allows bacteria normally found in the intestine to invade and damage the wall of the intestinal tissues. The damage may affect only a short segment of the intestine or can progress quickly to involve a much larger portion.

58 poor tolerance to feedings
In severe cases of NEC, a hole (perforation) may develop in the intestine, allowing bacteria to leak into the abdomen and cause life-threatening infection (peritonitis). Because the infant's body systems are immature, even with quick treatment for NEC there may be serious complications. Some experts believe that the makeup of infant formula, the rate of delivery of the formula, or the immaturity of the mucous membranes in the intestines can cause NEC. (Babies who are fed breast milk can also develop NEC, but their risk is lower.) Another theory is that babies born through difficult deliveries with lowered oxygen levels can develop NEC. When there isn't enough oxygen, the body sends the available oxygen and blood to vital organs instead of the gastrointestinal tract, and NEC can result. Signs and Symptoms: poor tolerance to feedings feedings stay in stomach longer than expected decreased bowel sounds abdominal distension (bloating) and tenderness greenish (bile-colored) vomit redness of the abdomen increase in stools, or lack of stools bloody stools

59 Diagnosis and Treatment:
More subtle signs of NEC might include apnea (periodic stoppage of breathing), bradycardia (slowed heart rate), diarrhea, lethargy, and fluctuating body temperature. Advanced cases may show fluid in the peritoneal (abdominal) cavity, peritonitis (infection of the membrane lining the abdomen), or shock. Diagnosis and Treatment: The diagnosis of NEC is usually confirmed by the presence of an abnormal gas pattern as seen on an X-ray. This is indicated by a "bubbly" appearance of gas in the walls of the intestine, large veins of the liver, or the presence of air outside of the intestines in the abdominal cavity. A surgeon may insert a needle into the abdominal cavity to withdraw fluid to determine whether there is a hole in the intestines. Abdominal x-ray. Stool for occult blood test (guaiac). Elevated white blood cell count in a CBC. Thrombocytopenia (low platelet count). Lactic acidosis.

60 Severe cases of NEC may require removal of a segment of intestine
Severe cases of NEC may require removal of a segment of intestine. Sometimes after removal of diseased bowel, the healthy areas can be sewn back together. Other times, especially if the baby is very ill or there is spillage of stool in the abdomen, the surgeon will bring an area of the intestine or bowel to an opening on the abdomen (called an ostomy). Most infants who develop NEC recover fully and do not have further feeding problems. In some cases, scarring and narrowing of the bowel may occur and can cause future intestinal obstruction or blockage. Another residual problem may be malabsorption. This is more common in children who required surgery for NEC and had part of their intestine removed. Most infants with NEC are treated medically, and symptoms end without the need for surgery. Treatment includes: stopping feedings, NG tube, IV fluids, antibiotics and frequent examinations.

61 What is leukemia? Leukemia is cancer of the blood and develops in the bone marrow. When a child has leukemia, the bone marrow, for an unknown reason, begins to make white blood cells that do not mature correctly, but continue to reproduce themselves. Normal, healthy cells only reproduce when there is enough space for them to fit. The body can regulate the production of cells by sending signals when to stop. With leukemia, these cells do not respond to the signals to stop and reproduce, regardless of space available. These abnormal cells reproduce very quickly and do not function as healthy white blood cells to help fight infection. When the immature white blood cells, called blasts, begin to crowd out other healthy cells in the bone marrow, the child experiences the symptoms of leukemia (i.e., infections, anemia, bleeding). Leukemia is the most common form of cancer in childhood. It accounts for about 30 percent of childhood cancers.

62 What are the symptoms of leukemia?
There are different types of leukemia According to the American Cancer Society: Acute lymphocytic leukemia (ALL) is the type of leukemia that most commonly affects children, most often between the ages of 2 and 4 years. Acute myelogenous leukemia (AML) is the second most common form of leukemia in children. AML generally occurs by the age of 2 years, and is not often seen in older children until the teenage years. AML is the most common type of acute leukemia in adults. The chronic forms of leukemia are rarely seen in children. The majority of childhood leukemias are acquired genetic diseases. This means that gene mutations and chromosome abnormalities in cells occur sporadically (by chance) and are not inherited from a parent . What are the symptoms of leukemia? irregular bone marrow function, anemia, bleeding and/or bruising, recurrent infections, bone and joint pain, swollen lymph nodes, abdominal distress: Abdominal pain may also be a symptom of leukemia. Leukemia cells can collect in the kidney, liver, and spleen, causing enlargement of these organs. Pain in the abdomen may cause a child to have loss of appetite and weight loss.

63 How is leukemia diagnosed?
Bone marrow aspiration and/or biopsy . Complete blood count (CBC). Computed tomography scan (CT or CAT scan). Magnetic resonance imaging (MRI). Ultrasound (Also called sonography.) Lymph node biopsy. Spinal tap/lumbar puncture. Treatment for leukemia: Chemotherapy. Radiation therapy. Bone marrow transplantation or peripheral blood stem cell transplantation. Blood transfusions (red blood cells, platelets). Antibiotics (to prevent/treat infections).

64 Wilms Tumor Also called Nephroblastoma
second most common intra-abdominal malignancy in children accounts for virtually all pediatric renal neoplasms. Higher in people of African descent Age: Peak incidence is at 3 years of age Rare in children > 5 years

65 Etiology Wilms tumor is thought to be caused by alterations of genes responsible for normal genitourinary development  common congenital anomalies associated with Wilms tumor: cryptorchidism double collecting system horseshoe kidney hypospadias.

66 Clinically Most patients present with an asymptomatic abdominal mass or abdominal distention. It can go unnoticed owing to the tumor's retroperitoneal location and painless quality. Occasional symptoms: abdominal pain vomiting hematuria hypertension.

67 Evaluation The mass is smooth and firm
in 5% to 10% of patients, masses are bilateral In some cases the mass is so large that diffuse distention, rather than a discrete mass, is felt. An abdominal sonogram or CT scan shows a solid intrarenal mass.

68 CT scan in a patient with a right-sided Wilms tumor with favorable histology.

69 Staging: I Limited to kidney; complete resection II Extent beyond kidney, complete resection III Unresectable, confined to abdomen, Lymph node metastasis IV Hematogenous mets (lung, liver, bone, brain) or lymph nodes outside abdomen V Bilateral renal involvement at diagnosis

70 Treatment combination of surgical resection (nephrectomy) and chemotherapy. Success rates depend on the extent of disease and the clinical stage at the time of diagnosis. Approximately 15% are metastatic at diagnosis (most commonly to the lungs) Histology is most important prognostic factor (favorable histology vs. anaplastic)

71 Neuroblastoma a malignant tumour that is derived from the sympathetic nervous system in the adrenal medulla (38%) or from any site along the sympathetic chain in the paravertebral sites of the abdomen (30%), chest (20%) and, rarely, the neck or pelvis. Nonmalignant form is ganglioneuroma

72 one of the small, blue, round cell tumors of childhood.
patients tend to be somewhat younger than with wilms tumor(Average age is 18 months; 80% < 5 years) more common in whites male-to-female ratio is 1.3:1

73 Clinically Has been called the great mimicker because of its many clinical presentations related to the site of the primary tumor, metastatic disease, and its metabolic tumor by-products. Unlike wilms tumor, patients appear more ill; an indication of the high incidence of metastatic disease at diagnosis (approximately 60%)

74 Abdominal mass (frequently crossing midline)
abdominal pain, urinary obstruction, flushing, sweating, diarrhea (caused by tumor secretion of vasoactive intestinal peptide) Signs of metastatic disease: anorexia, Malaise site-specific symptoms from metastases (bone,skin, liver, or central nervous system) neuroblastoma can become metastatic to the retrobulbar region, leading to rapidly progressive, unilateral, painless proptosis; periorbital edema; and ecchymosis of the upper lid. 

75 Evaluation A firm, irregular, nontender mass is palpable in the abdominal region. Other physical examination findings include: pallor subcutaneous nodules Hepatomegaly Urinary levels of catecholamine metabolites, are elevated in 90% to 95% of patients CT : Identification of a solid adrenal or paraspinal mass, which is calcified in 80% of patients.

76 Treatment Treatment may include bone marrow transplantation, chemotherapy, radiation therapy, and surgical debulking or resection. The prognosis depends on the stage of the disease, the age of the patient, the site of the primary tumor, and the findings on histologic evaluation.

77 Hepatoblastoma Definition : it is a form of liver cancer that usually occurs in infants that arises in an otherwise normal liver. Hepatoblastoma is the most common malignant primary liver tumor in infants and children The median age of diagnosis is 1 year; most patients are diagnosed by 2 years of age.

78 Hepatoblastoma Clinical picture : Most patients present with an enlarging abdominal mass. The right lobe is involved three times more commonly than the left, with bilobar involvement seen in 20%-30%, and multicentric involvement in 15%. Less common symptoms are anorexia, weight loss, and pain. Metastases at diagnoses occur in 10%-20% of patients with the lung being the predominant site of metastases both at presentation and relapse

79 The tumor's ability to synthesize AFP reflects its fetal origin
Hepatoblastoma Diagnosis : Levels of AFP in hepatoblastoma are often as high as 100, ,000 mcg/mL. It was found that AFP levels are elevated in 97% of patients. The tumor's ability to synthesize AFP reflects its fetal origin elevated AFP levels are not specific for hepatoblastoma, but they provide an excellent marker for response to therapy, disease progression, and detection of recurrent disease.

80 Hepatoblastoma

81 Treatment surgical resection, adjuvant chemotherapy prior to resection, and liver transplantation have been used to treat these neoplasms The presence of metastases is the most potent predictor of poor prognosis

82 These tumors may cause an abdominal mass
Lymphoma Lymphomas are malignant neoplasms of lymphoid lineage. Broadly classified as either Hodgkin disease (Hodgkin's disease) or as non-Hodgkin lymphoma,  Abdominal tumors are usually (non hodgkins): small noncleaved cell lymphoma [SNCCL] B-cell large cell lymphoma [LCL] These tumors may cause an abdominal mass

83 Clinically abdominal pain constipation ascites
Constitutional symptoms are uncommon in non-Hodgkin lymphoma, except in patients with anaplastic (LCL). Many of these patients have low-grade fever, malaise, anorexia, and/or weight loss. An acute abdomen occasionally is observed and may be mistaken for appendicitis.

84 Treatment Current treatment regimens are based primarily on the immunophenotype of the particular lymphoma (B cell vs T cell). T-cell therapies are longer and less intensive (particularly with respect to the use of alkylating agents) than B-cell therapies. Treatments for B-cell lymphomas involve relatively high doses of alkylators and antimetabolites.

85 Metastasis Metastasis to the liver : Neuroblastoma (most common)
lymphoma leukemia Wilms tumor. Metastatic lesions are usually multiple, and their imaging appearance is generally nonspecific

86 Thank You


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