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

4 th

day

of lapatotomy smoothly.

His condition was stable during the follow-up period at our out patient clinics.

Major Problems Minor Problems Bilious vomiting A soft tissue mass over right lower quadrant Downhilled blood pressure(shock) 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 non movable?

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

Signs or symptoms suggestive of an acute abdomen

Yes History and physical exam

Vomiting

Surgery Consult

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

No Sign or symptoms suggestive of increased ICP?

Yes No Chronic Sign or symptoms suggestive of increased ICP

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 Abnormal result No Neonate?

History and physical exam

Abdomen mass

Hepatomegaly or splenomegaly present

Yes No Yes Lower abdomen mass in female No Perform abdomen US Normal result 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 tricho

bezoar

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

bezoar

s and foreign bodies.

Small bowel obstruction with perforation is an unusual and rare complication of

bezoar

s.

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 of meconium

. with the

delayed passage

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 duplication

s may cause gastro

intestinal

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 4 yr -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 to perforation

.

as it progresses

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 false positive rates of 8–10% .

CT is more sensitive and specific ultrasonography than

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 younger child hours . However, perforation may occur more rapidly in the , 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, factors for and the circumferential loss of the appendiceal submucosal layer on ultrasound were the significant predictive 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 quadrant. Bilious vomiting the intestinal obstruction abdominal as a result of

Distal Intestinal Obstruction Syndrome Radiographs of the abdomen demonstrate dilated small bowel loops and a bubbly ileocecal soft tissue 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 coma and then death.

hypertension , and central nervous system changes leading to

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 ,3 rd 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,3 rd 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 evident by the presence of echogenic foci is 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,3 rd edition 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 cyst

s 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 的傳導。 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 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 改善。 164 mg （臨床醫學 ／ 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

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 途，已被禁止使用。 … 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 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

laboratory features of bowel produced 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.

Bilious vomiting intermittent irritable crying one-year-six month-old boy Dehydration Hypertension Leukocytosis Hyperchloremia 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

hematuria(15%) ,abdomen mass (25%), 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

yr of life.

in children beyond the 1st few

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 )

may involve the small bowel alone in but 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 of the colon.

, duplications associated with spinal cord defects and vertebral malformations, and Duplications may cause

bowel obstruction intestinal lumen

lead point of an intussusception or a site for a by

volvulus

.

compressing the adjacent

, or they may act as the

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

ectopic gastric mucosa

. scanning may localize 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

old child in a three Month 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 bile stained emesis

or gastric aspirate .

Plain radiographs demonstrate many

air fluid levels or peritoneal calcification

associated with meconium peritonitis. In

meconium ileus

, plain films of the abdomen show a typical

hazy or ground glass

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 usually a result of

small bowel obstruction

in a patient without previous bowel surgery is

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

patients.

but may be normal in 10% of 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

an adjacent segment. into 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,

increase in size and firmness during a paroxysm of pain and is most often in the which may

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 Mesenteric / Omental cyst

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 duplication

s. A positive scan requires the presence of ectopic gastric mucosa, which may be identified in both Meckel's diverticula and

intestinal duplication

s.

The significance of ectopic gastric mucosa is that it contains acid-secreting parietal cells, which may cause ulceration and bleeding. Only rarely are

intestinal duplication

s diagnosed preoperatively. After initial fluid resuscitation, bleeding from Meckel's diverticula and

intestinal duplication

s 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

images .

appearance in transverse

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

into the scrotum. that extends toward and possibly 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

the bed

pushing out the hernia.

strain the abdominal muscles by stretching out supine on

with legs extended and arms held straight above the head. Most infants struggle to get free, thus increasing the intra-abdominal pressure and

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

, encountered in although it has also been

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 Jul Aug.

Congenital defect of the small

intestinal

musculature is a rare cause of neonatal spontaneous

intestinal obstruction

perforation. or 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.