Dokter mengambil bagian sangat kecil dari rektum untuk dilihat di bawah mikroskop. Hirschsprungs disease is a condition characterized by the absence of ganglion cells. For a condition that is sometimes treated surgically, acquired megacolon amc is poorly understood and diagnostic criteria remain obscure 16. The authors studied 26 patients with congenital megacolon.
Hirschsprungs disease and other congenital functional. If there is a very large amount of extra bowel, there may be huge redundant loops of bowel that. Bowel movements build up in your colon instead of passing through to your rectum. Megacolon itself is not a specific disease entity, but it will usually result in obstipation inability to defecate, since feces is retained in the. Cats may have constipation that later progresses to megacolon, either from true disease progression or poor owner compliance in administrating medications. Megacolon congenitum definition of megacolon congenitum. Risperidone and megacolon article pdf available in singapore medical journal 4310. Generally, there are 3 types of megacolon, congenital megacolon hirschsprung disease, idiopathic megacolon imc, and acquired megacolon amc. Hirschsprungs disease hscr or congenital megacolon is one of the differential diagnoses of chronic constipation mostly in infancy and may indeed represent a challenge for pediatricians. See detailed information below for a list of 24 causes of megacolon, symptom checker, including diseases and drug side effect causes. Megacolon itself is not a specific disease entity, but it will usually result in obstipation inability to defecate, since feces is retained in the colon in a larger diameter than is able to. Other symptoms may include vomiting, abdominal pain, diarrhea and slow growth.
Complications may include enterocolitis, megacolon, bowel. Water intoxication in congenital megacolon documents. A modification of the swenson technique for congenital megacolon. The most accepted theory of the cause of hd is that there is a defect in the craniocaudal migration of neuroblasts originating from the neural crest, a process that begins at four weeks of gestation and ends at week 7 with the arrival of neural crestderived cells at the distal end of the colon 5.
Using the measurements from this paper, there is an overlap between patients with constipation and megacolon. One of the root cause is the parents upbringing pattern. This article is devoted to the acute development of megacolon. An unusual cause of idiopathic megacolon in a juvenile. Congenital megacolon hirschsprungs disease radiology. The term toxic means that this problem is very dangerous.
Toxic megacolon is a potentially lethal complication of inflammatory bowel disease ibd or infectious colitis that is characterized by total or segmental nonobstructive colonic dilatation plus systemic toxicity. Megacolon, massive enlargement and dilation of the large intestine colon. It denotes dilatation of the colon that is not caused by a mechanical obstruction. The dilation is often accompanied by a paralysis of the peristaltic movements of the bowel. Congenital megacolon hirschsprung disease is a topic covered in the 5minute clinical consult. You may be born without the nerves in your colon that give you the urge to have a bowel movement.
Megacolon, as well as megarectum, is a descriptive term. The megacolon congenital agangliosi and a hereditary disorder of the gi tract, more common in rabbits with the gene enen, that what gives the characteristics of the mantle hotot, inglese spot, etc ie, white with black or brown spots around the eyes and or back. This type of megacolon may be due to birth defects congenital and can be seen in infants, or acquired where it may develop in childhood, adolescence or adulthood. Congenital megacolon hirschprungs disease megacolon larger colon.
Hirschsprungs disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. Congenital megacolon is a disease of infancy and childhood due to a reduction or total absence of parasympathetic ganglia in a segment of colon. Megacolon knowledge for medical students and physicians. Although toxic megacolon is most commonly considered a complication of ibd, especially ulcerative colitis and to a less. The following medical conditions are some of the possible causes of. Acquired megacolon can be seen in mechanical obstructions, spinal cord injuries, tumors, inflammatory diseases, and in responses like abscesses. Megacolon can arise as a serious congenital problem in the newborn, caused by abnormal innervation of the colon and frequently requiring surgery. Virtually all infants and children affected with this disorder died. Children with constipation should, from a practical. There is another form of megacolon which causes the same gross anatomic changes as the primary or congenital type and which occurs as the result of slowly progressive and chronic obstruction of the distal segments of the large intestine. Lately, the epidemiology has shifted toward infectious causes, specifically due to an increase of. Jci hirschsprungs disease, down syndrome, and missing. This usually occurs, secondary to chronic constipation and retention of feces, but may be a congenital dysfunction. Hirschsprungs diseasehd is tenfold more common in down syndrome.
Megacolon is an abnormal dilation of the colon also called the large intestine. Twentyone pullthrough procedures for congenital aganglionic megacolon. I should now like to relate our interim clinical experience with these patients and particularly to stress diagnostic criteria of this disease. The pathogenesis of the dilationhypertrophy of the colon without mechanical obstruction was puzzling, and the subject of great debate. Source diseases database an abnormal enlargement of the colon. The clinical syndrome known as hirschsprung disease hd, or congenital megacolon, had been recognized for more than a century. Pdf hirschsprungs disease hscr is characterized by a lack of enteric nervous system ganglion cells. Children with constipation should, from a practical standpoint, be. Surgery is used to eliminate the infected portion of the colon which is devoid of the nerve cells.
Because the diameter of the large intestine varies, the. Megacolon definition of megacolon by the free dictionary. Get a printable copy pdf file of the complete article 652k, or click on a page. Genetic aganglionic megacolon in rats is a congenital condition similar to hirschsprungs disease in humans, lethal white foal syndrome in horses, piebald related megacolon in mice, and some. Complications may include enterocolitis, megacolon, bowel obstruction and intestinal perforation. Treatment for congenital megacolon or congenital aganglionic. Megacolon is the term used for an abnormally enlarged colon. Acute megacolon ogilvies syndrome is the acute dilation of the colon, characteristically seen in severely medicallysurgically ill patients. This consideration deals primarily with the congenital form of this disease.
We are fasttracking and publishing the latest research and articles related to covid19 for free. Megacolon, the irreversible dilation of a colonic segment, is a structural sign associated with various gastrointestinal disorders. The lack of ganglion cells is in the myenteric plexus auerbachs plexus, which is responsible for moving food in the intestine. In 1946ehrenpreis disproved the congenital megacolon theory by showing.
Jan 31, 2018 acquired megacolon amc is a condition involving persistent dilatation and lengthening of the colon in the absence of organic disease. Toxic megacolon occurs when swelling and inflammation spread into the deeper layers of your colon. Over the past few decades many genetic and nongenetic causes of distal bowel. Although toxic megacolon is most commonly considered a complication of ibd, especially ulcerative colitis and to a less extent crohns. An adult with megacolon the differential diagnosis of hirschsprungs disease christopher j. Toxic megacolon may occur in people with an inflamed colon due to. Megacolon itself is not a specific disease entity, but it will usually result in obstipation inability to defecate, since feces is retained in the colon in a larger diameter than is able to pass. Symptoms and diagnostic criteria of acquired megacolon a. Megacolon is the stretching and widening of your colon. Hirschsprungs disease congenital megacolon is caused by the failed. Hirschsprungs disease hscr is a deadly birth defect in which the enteric nervous system ens is missing from the end of the bowel 1, 2. Hirschsprung disease is characterized by congenital absence of ganglion. Hd is characterized by the absence of myenteric and submucosal ganglion cells in the distal alimentary tract. Congenital condition in which the colon does not have the normal network of nerves.
Modern concept of congenital megacolon hirschsprungs disease documents. Hirschsprungs disease congenital megacolon is caused by the failed migration of colonic ganglion cells during gesta tion. Congenital megacolon or congenital aganglionic megacolon occurs due to the genetic defects in the babies. Congenital aganglionic megacolon in adults a case report edward d. Colostomy above the rectosigmoid in four patients with congenital megacolon relieved abdominal distention and colonic stasis. Feb 03, 2020 megacolon is the stretching and widening of your colon. Two years ago i had the privilege of presenting to this academy our concepts of the pathology, the related dysfunction of the colon, and the surgical treatment of congenital megacolon. Megacolon definition of megacolon by medical dictionary. It is characterized by acute colonic dilatation in the absence of an intrinsic mechanical obstruction or an extrinsic inflammatory process toxic megacolon. Megacolon american college of veterinary surgeons acvs. During the past 25 years occasionally a report of an adult case has appeared.
Varying lengths of the distal colon are unable to relax, causing. Other forms of megacolon include pseudoobstruction, acute colonic ileus, or congenital colonic dilation. A modification of the swenson technique for congenital. Penyakit hirschsprung pada bayi yang berusia 6 bulan dengan riwayat konstipasi. Abstract hirschsprungs disease hd, also known as congenital aganglionic megacolon, is an uncommon disease caused by a failure of migration of neural crest cells to the distal colon. Doc referat megacolon congenital new tito wiga tri. Congenital megacolon american academy of pediatrics. Sep 25, 2019 megacolon, as well as megarectum, is a descriptive term.
Chronic megacolon may arise with various diseases including chronic neurological disorders, metabolic conditions and systemic diseases. Symptoms usually become apparent in the first two months of life. The two main types of the syndrome are congenital megacolon, or hirschsprung disease, and acquired megacolon. Hirschsprung disease pediatrics msd manual professional edition. During the past 25 years oc casionally a report of.
Secondly, there must be sufficient length of free dilated colon to pull through the. Although the babies would get relief, the symptoms might last for some time. For the past sixty years the term hirschsprungs disease has been used to describe enlargement of the colon exclusive of megacolon due to demonstrable organic disease. Toxic megacolon autenrieth 2012 inflammatory bowel.
New concepts of the etiology, diagnosis, and treatment of. Jan 24, 2020 acute colonic pseudoobstruction acpo is a rare condition first reported in 1948 by sir william ogilvie. Recent advances in the treatment of megacolon pdf free download. Megacolon can also be caused by longterm constipation. The colon is often described as distended or dilated 10. In 1887, hirschsprung published a paper entitled sluggishness of stool in the newborn resulting from dilatation and hypertrophy of the colon. Jan 28, 2019 surgery is the main mode of treatment for congenital megacolon or congenital aganglionic megacolon. Hirschsprungs disease most commonly involves the rectosigmoid region of the. In congenital megacolon, the lowermost portion of the large intestine is congenitally lacking in normal nerve fibres. In more extreme cases, the feces consolidate into hard masses inside the colon, called fecalomas literally, fecal tumor, which can require surgery to be removed. Acute colonic pseudoobstruction acpo is a rare condition first reported in 1948 by sir. More rarely the entire colon and part of the small intestine may be involved. Surgery is performed to eliminate the diseased portion of the intestine so that the passage of the stool is clear.
In congenital megacolon, the lowermost portion of the large intestine is congenitally lacking in normal nerve. The information on free feeding study on repeat stasisgassy bunnies. Megacolon congenitum definition of megacolon congenitum by. It is often associated with extra redundant loops of bowel. Linings of infected portion are removed and the healthy part is directly joined to the rectum. Dilatation of the colon, often to alarming dimensions. Acute colonic pseudoobstruction acute megacolon, ogilvie. Infections of the colon such as clostridium difficile. Scribd is the worlds largest social reading and publishing site. There are various types of megacolon including congenital megacolon in hirschsprung disease, idiopathic megacolon in constipation, and toxic megacolon. Occasionally, in the case of persistent longterm constipation, surgical treatment resection of the elongated portion of the intestine.
Megacolon is a term used to describe a very dilated, flaccid, incompetent colon. Dictionary entry details congenital megacolon noun sense 1. Ulcerative colitis, or crohn disease that is not well controlled. Hirschsprungs disease hd or hscr is a birth defect in which nerves are missing from parts of the intestine. Megacolon congenital megacolon hirschprungs disease megacolon larger colon dolicocolon longer colon. Diagnosis depends on subjective radiological, endoscopic or surgical findings in the context of a suggestive clinical presentation. Congenital megacolon noun the noun congenital megacolon has 1 sense 1. If there is a very large amount of extra bowel, there may be huge redundant. The term refers to a colon of increased diameter and increased length, in the absence of organic disease 79. Megacolon is the dilation of the colon in the absence of a mechanical obstruction e. Congenital megacolon definition of congenital megacolon. Congenital megacolon congenital aganglionosis, hirschsprungs disease a disease affecting 1.1369 1479 771 471 1568 77 1505 593 1380 1663 784 1569 1651 551 34 761 259 424 1231 208 272 535 153 490 1532 1534 504 598 324 1348 1202 1113 1455 709 727 515 271