Clinical Pathological Conference 2004-12-29 三軍總醫院 小兒科部 劉家宏/華一鳴 Case Presentation Present Illness This one-year-six-month-old boy came to our pediatric emergency department with the chief compliant of bilious vomiting and intermittent irritable crying since 4 hours ago. Case Presentation Personal and Family History Past history: Before this presentation,the child was in good condition without any compliant of abdominal symptoms. Case Presentation Physical Examination Vital sign: PR: ↑ 136 /min (70-110), RR: 20 /min(20-30), BT: 35.7 ºC BP: ↑ 112/70(90-105,50-70), decreased urine output. HEENT: dehydration with dry lip. Abdomen : soft, neither sign of peritonitis nor hepatosplenomegaly . Case Presentation Radiologic & Lab Findings Plain film of abdomen : showed a nonspecific local ileus pattern over right upper quadrant. Laboratory data: white blood count: ↑ 25300/nl (6000-17500/nl) with 75% segment(54-62%) and 18% lymphocyte(25-33%). Case Presentation Radiologic & Lab Findings Serum biochemistry: Sodium :145 (139-146)mEq/L Potassium :4.5 (3.5-5.0)mEq/L Chloride : ↑ 130 (98-106)mEq/L BUN :19 mg/dl, Creatinene :0.4(0.2-0.4)mg/dl C reative protein level :0.1mg/dl. Case Presentation Radiologic & Lab Findings Abdomen sonography :revealed a soft tissue mass over right lower quadrant, but no evidence of sign of target appearance; besides, the relationship of superior mesentery artery and superior mesentery vein was in right position. Case Presentation Hospital Course-I Under the impression of intestinal obstruction, he was admitted to our pediatric department for further evaluation and management. After admission, this patient had persistent bilious vomiting. Case Presentation Hospital Course-II Twelve hours later, heart rate increased to 150-180 per minutes. Meanwhile, decreased urine output and downhilled blood pressure happened to him despite of intravenous fluid supplement. Under the impression of intestinal obstruction complicated with shock, our pediatric surgeon arranged emergency laparotomy. Case Presentation Hospital Course-II Post-operative course was relative smooth,and started feeding on the 4th day of lapatotomy smoothly. His condition was stable during the follow-up period at our out patient clinics. Major Problems Bilious vomiting A soft tissue mass over right lower quadrant Downhilled blood pressure(shock) Minor Problems Intermittent irritable crying Decreased urine output Leukocytosis Hyperchloremia Local ileus pattern over right upper quadrant Tachycardia Hypertension Questions –about past, personal, family history Birth history? History of trauma? Child abuse? Operation history? foreign body aspiration? Drug history? Food history? Family history about tumor? About cystic fibrosis? Questions- about Physical Examination and laboratory data Anemic conjunctiva? Lymphoadenopathy? RLQ soft tissue mass movable or nonmovable? Bowel sound? Hyperactive to be replaced with hypoactive bowel sounds? Stool rontine examination? occult blood? WBC? pattern? Blood smear? blast cell? Questions-about radiologic finding Plain film of abdomen :further finding? Foreign body/Bezoars? Abdomen Sonography: further finding? Appendix? Ascites?Kidney? Any finding about tumor? Bilious vomiting Vomitus or nasogastric aspirate containing bile which in children almost always indicates bowel obstruction distal to the sphincter of Oddi. By contrast, infants with pyloric stenosis have non-bile-stained vomiting. Intestinal obstruction Intraluminal :polyp, mass, parasites, and tumor. Intramural :stricture, tumor, hematoma. Extrinsic:postoperative adhesion, adhesion from peritonitis, hernia, volvulus,and tumor. Clinical Classification of Shock Septic shock :bacterial,Viral,Fugal.. Cardiogenic shock : ischemia, cardiomyopathy ,congestive heart failure Distributive shock:toxins,anaphylaxis… Hypovolemic shock :enteritis,hemorrhage, Obstructive shock :tension pneumothroax Hyperchloremia Pathophysiology Metabolic Acidosis with a normal Anion Gap Causes Artifact (low Anion Gap) Metabolic and Endocrine Hyperparathyroidism, Renal Tubular Acidosis, Hypernatremia. Bromide intoxication Nervine, Sominex Acetazolamide Carbonic anhydrase inhibition Boric acid , Triamterene ,Ammonium Chloride Excess IV Normal Saline Hyperchloremia Gastrointestinal Dehydration Prolonged Diarrhea Loss of pancreatic secretion Ileal loops Ureteral colonic anastomosis (From: Pediatric Decision-making Strategies accompanied by Nelson) Acute Yes Surgery Consult Signs or symptoms suggestive of an acute abdomen History and physical exam Malrotation with volvulus Appendicitis Other causes of intestinal obstrution Congenital structural abnormalities Postsurgical adhesions Foreign body/Bezoars Meckels diverticulum Incarcerated inguinal hernia Meconium ileus Intussusception Hirschsprungs disease Superior mesenteric artery syndrome Duodenal hematoma Testicular or ovarian torsion Vomiting No Chronic Sign or symptoms suggestive of increased ICP? Sign or symptoms suggestive of increased ICP Yes No Malrotation with volvulus Appendicitis Congenital structural abnormalities Postsurgical adhesions Foreign body/Bezoars Meckels diverticulum with bleeding Incarcerated inguinal hernia Meconium ileus Intussusception Hirschsprungs disease Superior mesenteric artery syndrome Duodenal hematoma Testicular or ovarian torsion (From: Pediatric Decision-making Strategies accompanied by Nelson) Yes Abdomen US Yes No History and physical exam Neonate? No Abdomen mass Hepatomegaly or splenomegaly present Lower abdomen mass in female Abnormal result Perform abdomen US Normal result No Yes Perform Abdomen CT Not neonate , Not female with lower abdomen mass Splenomegaly Wilms tumor Adrenal cortical neoplasms Pancreatic masses/cysts Neuroblastoma Hydronephrosis Rhabdomyosarcoma Urinary retention Hepatic lesion Teratoma Bezoar Appendiceal abscess Intestinal tumor Mesenteric cyst Omental cyst Lymphangioma Lymphoma Choledochal cyst Constipation Inflammatory bowel disease Retroperitoneal hematoma Foreign body/Bezoars Meckels diverticulum Hirschsprungs disease Appendicitis Intestinal tumor Neuroblastoma Rhabdomyosarcoma Lymphoma Congenital structural abnormalities Foreign body/Bezoars (bezôr) An accumulation of exogenous matter in the stomach or intestine. peak incidence between the ages of 6 mo and 3 yr 90% of foreign bodies are opaque. vomiting, anorexia, and weight loss. An abdominal plain film may suggest the presence of a bezoar, which can be confirmed on ultrasound or CT examination. Gastric trichobezoar Pediatric Emergency Care. 19(5):343-7, 2003 Oct. On plain abdominal radiographs, the bezoar will appear as a mottled heterogenous mass that may be mistaken for a food-filled stomach. The classic sonographic appearance is described as a band of increased echogenicity in the region of the stomach with complete loss of posterior echoes. Small bowel obstruction and covered perforation in childhood caused by bizarre bezoars and foreign bodies. Small bowel obstruction with perforation is an unusual and rare complication of bezoars. Israel Medical Association Journal: Imaj. 2(2):129-31, 2000 Feb. Hirschsprungs disease Abnormal innervation of the bowel . Most common cause of lower intestinal obstruction in neonates . Usually begin at birth with the delayed passage of meconium. Some infants pass meconium normally but subsequently present with a history of chronic constipation. Hirschsprungs disease Failure to thrive, with hypoproteinemia from a protein-losing enteropathy, is a less common presentation. Rectal examination demonstrates normal anal tone and is usually followed by an explosive discharge of foul-smelling feces and gas. Hirschsprungs disease Rectal manometry and rectal suction biopsy are the easiest and most reliable indicators of Hirschsprung disease. Barium enema examination is useful in determining the extent of aganglionosis. Sonography may also help in determining the dynamic or adynamic state of fluid-filled or solid-filled bowel loops. Meckels diverticulum Remnant of the embryonic yolk sac Arise within the 1st 2 yr of life . Intermittent painless rectal bleeding by ulceration of the adjacent normal ileal mucosa. Meckels diverticulum Brick colored or currant jelly colored. Obstruction occurs when the diverticulum acts as the lead point of an intussusception. A Meckel diverticulum may occasionally become inflamed (diverticulitis) and present similarly to acute appendicitis. The most sensitive study is a Meckel radionuclide scan Meckel's diverticulum. Internal hernia and adhesions without gastrointestinal bleeding--ultrasound and scintigraphic findings. US study was particularly helpful in this case because it shows a nonperistaltic region, which is consistent with a diverticulum or an internal hernia. Clinical Nuclear Medicine. 21(12):938-40, 1996 Dec. Meckel's diverticulum mimicking infantile colic: sonographic detection. Abdominal sonography at 6 months of age demonstrated an abdominal mass with an anechoic center and a double-layered wall, surrounded by bowel loops. Histologic examination of the resected mass revealed a Meckel's diverticulum with a perforation sealed off by the neighboring bowel and mesentery to form an inflammatory mass. Journal of Clinical Ultrasound. 28(6):314-6, 2000 Jul-Aug Gastrointestinal bleeding in infants and children: Meckel's diverticulum and intestinal duplication. Seminars in Pediatric Surgery. 8(4):202-9, 1999 Nov. Meckel's diverticula and intestinal duplications may cause gastrointestinal bleeding in almost any age group and require a high index of suspicion for diagnosis. Appendicitis The risk of perforation is greatest in 1- to 4yr-old children (70–75%) and is lowest in the adolescent age group (30–40%) . The classic triad consists of pain, nausea with vomiting, and fever. The progression from onset of symptoms to perforation usually occurs over 36–48hr. Appendicitis History included onset of pain before vomiting or diarrhea, loss of appetite, migration of pain from periumbilical to right lower quadrant. Auscultation may reveal normal or hyperactive bowel sounds in early appendicitis, to be replaced with hypoactive bowel sounds as it progresses to perforation. Appendicitis Findings of appendicitis on abdominal films include calcified appendicolith, small bowel distention or obstruction, and soft tissue mass effect. Graded compression ultrasonography is a noninvasive study with false-negative and falsepositive rates of 8–10% . CT is more sensitive and specific than ultrasonography and more likely to change patient management. Pediatric appendicitis in "real-time": the value of sonography in diagnosis and treatment. Pediatric Emergency Care. 17(5):334-40, 2001 Oct. The natural progression in appendicitis from initial symptoms to perforation is about 36 to 48 hours . However, perforation may occur more rapidly in the younger child, sometimes within 6 to 12 hours . Extensive necrosis of the appendix may render it difficult to visualize . Pediatric appendicitis in "real-time": the value of sonography in diagnosis and treatment. Pediatric Emergency Care. 17(5):334-40, 2001 Oct. We may have to rely on the other ultrasound features of “peri-appendiceal inflammation.” Studies have shown that the presence of loculated pericecal fluid, prominent pericecal fat, atonic bowel loops, thickened bowel walls, and the circumferential loss of the appendiceal submucosal layer on ultrasound were the significant predictive factors for perforation . Distal Intestinal Obstruction Syndrome In the older child or young adult with CF, the distal small bowel may by obstructed by thick stool. This condition was called "meconium ileus equivalent" by Jensen in 1962 . Palpable mass in the right lower abdominal quadrant. Bilious vomiting as a result of the intestinal obstruction Distal Intestinal Obstruction Syndrome Radiographs of the abdomen demonstrate dilated small bowel loops and a bubbly ileocecal softtissue mass . Carcinoid tumor About 85% of carcinoid tumors develop in the gastrointestinal tract, usually the appendix. Carcinoid syndrome:flushing,diarrhea, wheezing. Carcinoid crisis:generalized flush, tachycardia, severe diarrhea with abdominal pain, hypotension converting to hypertension, and central nervous system changes leading to coma and then death. Carcinoid tumor Approximately 40% of the tumors occurred within 2 feet of the ileocecal valve, with very few in the proximal small intestine. These tumors frequently elicit a mesenteric fibrosing reaction, in which the bowel becomes shortened and kinked, frequently causing partial small bowel obstruction. Carcinoid tumor On CT, the mesenteric extension from carcinoid will usually appear as a soft tissue-density mesenteric mass . Calcification can be seen in up to 70% of cases . Lymphoma from manual of pediatric hematology and oncology ,3rd edition Non-Hodgkins lymphoma: peak age 5-15 years ,rick factor including genetic and poettransplantation immunosuppression. Clinical feature:Head and neck(13%), medicatinum(26%),abdomen(35%). Non-Hodgkins lymphoma The ileum is mostly involved due to a higher number of lymphocytes in the distal gut, accounting for about 50% of small bowel lymphomas Present with abdomen pain, vomiting and diarrhea, abdominal distension, palpable mass, intussusception,peritonitis, ascites, GI bleeding, hepatosplenomegaly. Multidetector-row computed tomography and 3-dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. Lymphoma can appear as a single mass lesion, which varies in size.These can lead to intussusception, but rarely will result in obstruction because the masses are typically pliable and soft. Again, because the masses are characteristically soft, it is rare that the mesenteric vasculature is compromised. Journal of Computer Assisted Tomography. 28(1):106-16, 2004 Jan-Feb. Neuroblastoma from manual of pediatric hematology and oncology,3rd edition Give rise to adrenal medulla and the sympathetic ganglia. Most common tumor in infancy ,peak incidence is 2 years of age Clinical finding related to anatomic site of abdomen :anorexia ,vomiting,abdomen pain,massive involvement of the liver with metastasis (especially in the newborn) Neuroblastoma Paraneoplastic manifestations : excessive catecholamine secretion (sweating,flushing, paller,palpitation, hypertension) ,VIP secretion (watery diarrhea,abdomen distension, hypokalemia) ,and acute myoclonic encephalopathy. Neuroblastoma Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed., A neuroblastoma is usually solid with a heterogeneous echotexture. Calcification is evident by the presence of echogenic foci with posterior acoustic shadowing. Anterior displacement and encasement of the aorta and inferior vena cava (IVC) by this retroperitoneal tumour is characteristic. Rhabdomyosarcoma from manual of pediatric hematology and oncology,3rdedition Two age peaks:2-6 years and 15-19 years. Rare primary sites for rhabdomyosarcoma include the GI-hepatobiliary tract(3%), where in presents with obstructive jaundice and a large abdomen mass. These tumors arise in the common bile duct and may extend into both lobes of the liver. Final diagnosis 1.Meckels diverticulum with diverticulitis or congenital structural abnormalities 2.Ruptured Appendicitis THANK YOU!! Mesenteric cyst / Omental cyst They ranged in age from 1 month to 14 years; 75% were younger than 5 years. The main presenting symptom is abdominal pain, followed by nausea and vomiting. Some mesenteric cysts may present as an acute abdomen due to a possible complication, such as hemorrhage, rupture, or torsion of the cyst. Mesenteric cyst / Omental cyst Mesenteric cysts in children Surgery 1994;115:571-7 Acute symptoms are related to compression of intra-abdominal organs or stretching of the mesentery by rapid expansion. Among these categories, the cystic lymphangioma is differentiated from the others because it is far more common in children. Lymphangioma Five pathologic patterns account for most mesenteric cysts, namely, lymphangioma, enteric duplication cysts, enteric cysts, mesothelial, and nonpancreatic pseudocysts. 溴化物中毒之神經精神異常表現 陳建良、劉義聰、蔡瑞安、林自強 溴化物離子在各種膜性傳導系統上，可取代氯離子，特別是在 神經系統，高濃度的溴化物，膜性抑制作用會逐漸損害神經元 的傳導。 長期服用含有溴化物藥劑，可能造成慢性溴化物中毒，而表現 出各式各樣神經精神異狀如意識混亂、躁動、頭暈、步態不穩、 運動失調、視力模糊、視野缺損、嗅覺障礙、短期記憶力障礙、 幻想、幻覺、麻木等週邊神經病變。 一般認為血中溴化物濃度超過50 mg/dl(6.3 mEq/l)將產生一些 神經和精神症狀，實驗室檢查發現除了溴化物濃度過高以外亦 可發現血氯偏高及負陰離子間隙。 我們報告一病例因長期服用感冒糖漿造成溴化物中毒，臨床表 現出胸痛，呼吸急促與焦躁不安等症狀，起初血液檢查顯示出 高血氯，負陰離子間隙，因而聯想到溴化物中毒，經測血中溴 化物濃度高達164 mg／dl (正常值＜50 mg／dl) 。住院後給予 靜脈輸液利尿治療，一週後胸悶、胸痛、焦躁不安等症狀完全 改善。（臨床醫學 2004; 54: 189-93） 2003.12.27 【林杰樑（林口長庚 醫院臨床毒物科主任）】 《透視食品添加物》硼砂 增加食品彈性口感 新聞來源： 硼砂主要是在食品品質改良的應用。它增加食品的韌性、 彈性、保水性以及保存性。防止蝦頭的黑變，使蝦有好 的賣相；魚丸、年糕、油條、鹼粽…等，較常見的則是 在油麵的製程中添加，使食品的口感、脆感、保鮮等用 途，已被禁止使用。 硼砂進入體內後，經胃酸作用，轉變為硼酸（Boric acid），會影響消化酵素作用。 可能引起食慾減退，消化不良，抑制營養素之吸收。 硼酸中毒症狀為嘔吐、腹瀉、紅斑、循環係統障礙、休 克及昏迷等硼酸症徵狀，而且有致死量，大人約20公克、 小孩約為5公克。 Leiomyoma of the small bowel with hypercalcaemia: presence of a substance with parathormone activity Nouvelle Presse Medicale. 8(40):3245-6, 1979 Oct 22. A leiomyoma of the small bowel produced laboratory features of hyperparathyroidism which disappeared promptly after tumour resection. Hypercalcaemia, hypophosphatemia, hyperchloremia, elevated chloride/phosphorus ratio, increased urinary cyclic AMP, and blood levels of immunoreactive parathormone were present. Retroperitoneal hematoma In the majority of cases there is an associated pathological condition of a viscus or vessel due to trauma, aneurysm, atherosclerosis, eroding primary or secondary tumors, or pancreatitis. Furthermore, some have stated that hemophilia and anticoagulant therapy may be the cause in some cases. Retroperitoneal hematoma These patients usually present with mild to severe abdominal pain, nausea and vomiting. Physical examination reveals signs of shock, ileus and flank mass. CT scans are used usually to establish the diagnosis of retroperitoneal hematoma. Dehydration Hypertension Leukocytosis Hyperchloremia Bilious vomiting intermittent irritable crying one-year-six month-old boy Soft tissue mass over RLQ by Abdomen sonography Decreased urine output and downhill BP in 16 hours Wilms tumor It may be associated with hemihypertrophy, aniridia, and other congenital anomalies, usually of the genitourinary tract. Sign of Wilms tumor:palpable mass in abdomen (60%),hypertension (25%), hematuria(15%),abdomen mass is the most common presenting symptom and sign ,occasionally there is abdomen pain,especially when hemorrhage occurs in the tumor following trauma. Wilms tumor Some patients may present with abdominal pain and vomiting and, infrequently, hematuria. Occasionally, rapid abdominal enlargement and anemia may occur owing to bleeding into the renal parenchyma or pelvis. Wilms tumor Grainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed., On ultrasound examination the tumour appears as a well-defined, solid mass of mixed echogenicity.Areas of haemorrhage and necrosis can produce focal hypoechoic lesions within the mass. Ultrasound may be used for periodic surveillance of the opposite kidney. Superior mesenteric artery syndrome The classic example is an adolescent who starts vomiting after application of a body cast for orthopedic surgery. Other associated factors include anorexia, prolonged bed rest, weight loss, abdominal surgery, and exaggerated lumbar lordosis. The diagnosis is established radiologically with the demonstration of a cutoff of the duodenum just to the right of the midline. Superior mesenteric artery syndrome An extrinsic compression of the duodenum in children after rapid weight loss and in a supine position. The compression is thought to occur as the mesentery loses its fat and allows the superior mesenteric artery to collapse on the duodenum, compressing it between the superior mesenteric artery anteriorly and the aorta posteriorly. Inflammatory bowel disease The most common time of onset of IBD is during adolescence and young adulthood. A bimodal distribution has been shown with an early onset at 15–25 yr of age and a second smaller peak at 50–80 yr of age. Nonetheless, IBD may begin as early as the 1st yr of life. In developed countries, these disorders are the major causes of chronic intestinal inflammation in children beyond the 1st few yr of life. Ulcerative colitis Bloody stool and diarrhea are the typical presentation of ulcerative colitis. Constipation may be observed in those with proctitis. Fever, severe anemia, hypoalbuminemia, leukocytosis, and greater than five bloody stools per day for 5 days is what defines fulminant colitis. Ulcerative colitis Extraintestinal manifestations that tend to occur more commonly with ulcerative colitis than with Crohn disease include pyoderma gangrenosum, sclerosing cholangitis, chronic active hepatitis, and ankylosing spondylitis. Crohns disease Crohn colitis may be associated with bloody diarrhea, tenesmus, and urgency. Children with Crohn disease often appear chronically ill, weight loss linear growth retardation. Crohns disease The initial presentation most commonly involves ileum and colon (ileocolitis) but may involve the small bowel alone in about 30% or colon alone in 10%–15%. Children with ileocolitis typically have cramping, abdominal pain, and diarrhea, sometimes with blood. Ileitis may present as right lower quadrant abdominal pain alone. Crohns disease There may be abdominal tenderness that is either diffuse or localized to the right lower quadrant. The diagnosis of Crohn disease depends on finding typical clinical features of the disorder (history, physical examination, laboratory studies, and endoscopic or radiologic findings). Crohns disease Plain films of the abdomen may be normal or may demonstrate findings of partial small bowel obstruction or thumbprinting of the colon wall. An upper gastrointestinal contrast study with small bowel follow-through may show aphthous ulceration and thickened, nodular folds as well as narrowing of the lumen anywhere in the gastrointestinal tract. Duplication Duplications can be classified into three categories: localized duplications, duplications associated with spinal cord defects and vertebral malformations, and duplications of the colon. Duplications may cause bowel obstruction by compressing the adjacent intestinal lumen, or they may act as the lead point of an intussusception or a site for a volvulus. If they are lined by acid-secreting mucosa, they may cause ulceration, perforation, and hemorrhage of the adjacent bowel. Patients may present with abdominal pain, vomiting, palpable mass, or acute gastrointestinal hemorrhage. Radiologic studies such as barium studies, ultrasonography, CT, and MRI are helpful but usually nonspecific, demonstrating cystic structures or mass effects. Radioisotope technetium scanning may localize ectopic gastric mucosa. The treatment of duplications is surgical resection and management of associated defects. Atypical presentation of an intestinal duplication in a three month old child Journal de Radiologie. 85(6 Pt 1):773-5, 2004 Jun. Intestinal duplication is an uncommon congenital anomaly that often is diagnosed during childhood. Ultrasound diagnosis is based on the presence of a characteristic double-walled cystic mass. We report a case of duplication in a three Month old child presenting with small bowel obstruction. Intestinal duplication presenting as spontaneous hemoperitoneum. Journal of Pediatric Gastroenterology & Nutrition. 31(2):181-2, 2000 Aug. In approximately 60% of the cases, the condition appears during the first year of life as a palpable abdominal mass or as complications such as intestinal obstruction due to extrinsic compression, volvulus, or intussusception. Intestinal duplication presenting as spontaneous hemoperitoneum. Journal of Pediatric Gastroenterology & Nutrition. 31(2):181-2, 2000 Aug. It was probably caused by erosion of a blood vessel adjacent to the perforation that had been sealed off at laparotomy. Sudden infant death, large intestinal volvulus, and a duplication cyst of the terminal ileum. American Journal of Forensic Medicine & Pathology. 21(1):62-4, 2000 Mar. If an intussusception or volvulus is identified, careful search for predisposing lesions or conditions such as duplication cysts, mesenteric cysts, mesenteric defects, Meckel's diverticula, mesenteric lymph nodes, polyps, neoplasms, mural hematomas, or cystic fibrosis should also be undertaken. Jejunal and Ileal Atresia and Obstruction Jejunoileal atresias have been attributed to intrauterine vascular accidents leading to ischemic necrosis of the sterile bowel and resorption of the affected segments. Most infants become symptomatic during the 1st day of life with abdominal distention and bilestained emesis or gastric aspirate. Plain radiographs demonstrate many airfluid levels or peritoneal calcification associated with meconium peritonitis. In meconium ileus, plain films of the abdomen show a typical hazy or groundglass appearance in the right lower quadrant. Pneumoperitoneum is most readily seen as free air between the liver and the diaphragm on an upright radiograph of the abdomen; if there is a large amount of free air, the entire abdomen may look like a football from distention with air; the ligamentum teres is sometimes clearly visible in the midline. Malrotation Incomplete rotation of the intestine during fetal development The majority of patients present within the 1st yr of life with symptoms of acute or chronic obstruction. Infants often present within the 1st wk of life with bilious emesis and acute bowel obstruction. An acute presentation of small bowel obstruction in a patient without previous bowel surgery is usually a result of volvulus associated with malrotation. The abdominal plain film is usually nonspecific but may demonstrate evidence of duodenal obstruction with a double-bubble sign. Barium enema usually demonstrates malposition of the cecum but may be normal in 10% of patients. Upper gastrointestinal series demonstrates malposition of the ligament of Treitz. Ultrasonography demonstrates inversion of the superior mesenteric artery and vein. A superior mesenteric vein located to the left of the superior mesenteric artery is suggestive of malrotation. Surgical intervention is recommended for any patient with a significant rotational abnormality, regardless of age. Intussception A portion of the alimentary tract is telescoped into an adjacent segment. The most common cause of intestinal obstruction between 3 mo and 6 yr of age. Sixty per cent of patients are younger than 1 yr, and 80% of the cases occur before 24 mo; it is rare in neonates. The male:female ratio is 4:1. Most intussusceptions do not strangulate the bowel within the first 24hr but may later eventuate in intestinal gangrene and shock. Sudden onset, severe paroxysmal colicky pain Vomiting occurs in most cases and is usually more frequent early. 60% of infants pass a stool containing red blood and mucus, the currant jelly stool. Tender sausage-shaped mass, which may increase in size and firmness during a paroxysm of pain and is most often in the right upper abdomen, with its long axis cephalocaudal. Plain abdominal radiographs may show a density in the area of the intussusception. Neonate with abdomen mass Neuroblastoma Congenital Hydronephrosis Multiple cystic kidney Infantile polycystic kindey disease Neurogenic bladder Renal vein thrombosis Collecting system duplication Intestinal duplication Sacrococcygeal teratoma Adrenal hemarrhage Mesoblastic nephroma Pancreatic cyst Hepatoblastoma Meconium ileus Hematoma(hepatic,splenic) Magacolon(obstruction) Anterior myelomenihgocele Appendiceal abscess Intestinal tumor Mesenteric / Omental cyst Choledochal cyst Neuroblastoma Congenital Hydronephrosis Multiple cystic kidney Infantile polycystic kindey disease Neurogenic bladder Renal vein thrombosis Collecting system duplication Intestinal duplication Sacrococcygeal teratoma Adrenal hemarrhage Mesoblastic nephroma Pancreatic cyst Hepatoblastoma Meconium ileus Hematoma(hepatic,splenic) Magacolon(obstruction) Anterior myelomenihgocele Appendiceal abscess Intestinal tumor Renal Vein Thrombosis In newborns and infants, RVT is commonly associated with asphyxia, dehydration, shock, sepsis, and infants born to mothers with diabetes mellitus. Sudden onset of gross hematuria and unilateral or bilateral flank masses, microscopic hematuria, flank pain, hypertension, or oliguria. Renal Vein Thrombosis RVT is usually unilateral. Bilateral RVT results in acute renal failure. Most patients also have a microangiopathic hemolytic anemia and thrombocytopenia. Ultrasonography shows marked enlargement, whereas radionuclide studies reveal little or no renal function in the affected kidney(s). Doppler flow studies of the inferior vena cava and renal vein confirm the diagnosis. The advent of technetium (Tc) 99m pertechnetate radionuclide scanning has greatly facilitated the diagnosis of Meckel's diverticula and may also be useful for intestinal duplications. A positive scan requires the presence of ectopic gastric mucosa, which may be identified in both Meckel's diverticula and intestinal duplications. The significance of ectopic gastric mucosa is that it contains acid-secreting parietal cells, which may cause ulceration and bleeding. Only rarely are intestinal duplications diagnosed preoperatively. After initial fluid resuscitation, bleeding from Meckel's diverticula and intestinal duplications require surgical intervention. Resection is the treatment of choice. A barium enema shows a filling defect or cupping in the head of barium where its advance is obstructed by the intussusceptum (coiled-spring sign) . Ultrasonography is a sensitive diagnostic tool in the diagnosis of intussusception. The diagnostic findings of intussusception include a tubular mass in longitudinal views and a doughnut or target appearance in transverse images . Posttraumatic retroperitoneal rupture of the right colon simulating a retroperitoneal hematoma. Journal of Trauma-Injury Infection & Critical Care. 42(4):741-2, 1997 Apr. This case illustrates the diagnostic problems encountered in a patient with posttraumatic retroperitoneal abscess caused by perforation of the posterior wall of the cecum, simulating a retroperitoneal hematoma. Blunt colonic injuries are rare and difficult to diagnose. Septic signs are unexpected in case of posttraumatic retroperitoneal hematoma and should suggest the diagnosis of retroperitoneal colonic perforation. Incarcerated inguinal hernia An inguinal hernia appears as a bulge in the inguinal region that extends toward and possibly into the scrotum. The hallmark signs of an inguinal hernia on physical examination are a smooth, firm mass that emerges through the external inguinal ring lateral to the pubic tubercle and enlarges with increased intra-abdominal pressure. A quiet infant can be made to strain the abdominal muscles by stretching out supine on the bed with legs extended and arms held straight above the head. Most infants struggle to get free, thus increasing the intra-abdominal pressure and pushing out the hernia. Incarcerated inguinal hernia The infant or child with an incarcerated inguinal hernia is likely to have associated findings suggestive of intestinal obstruction such as abdominal distention, vomiting, and multiple air-fluid levels evident on plain radiographs. Wilms tumor The incidence is approximately 8 cases/million children younger than 15 yr of age. It usually occurs in children between 2–5 yr of age, although it has also been encountered in neonates, adolescents, and adults. Neonatal intestinal perforation caused by congenital defect of the small intestinal musculature: report of one case. Acta Paediatrica Taiwanica. 40(4):271-3, 1999 JulAug. Congenital defect of the small intestinal musculature is a rare cause of neonatal spontaneous intestinal obstruction or perforation. Histology examination demonstrates multifocal deficiency of the inner circular muscle layer three cm around the perforation site. The clinical and histological characteristics are reviewed and discussed. We propose that the muscle defect of small intestine, especially ileum, is secondary to ischemic injury rather than an embryological malformation.