Hirschsprungs disease symptoms, diagnosis and treatment. A term male infant with a birth weight of 3,300 gm was born by normal spontaneous vaginal delivery after an uncomplicated pregnancy to a 28yearold white woman. These patients present within the first 24 hours of life with abdominal. Meconium plug syndrome, cystic fibrosis, and exocrine. Other familiar examples in the syndrome include arguments to merge church school classes, reduce the number of worship services, or merge.
Meconium aspiration syndrome genetic and rare diseases. The authors experience with 14 cases of the meconium plug syndrome and cases of hirschsprungs disease is presented and the roentgenographic findings. Treatments for meconium plug syndrome including drugs, prescription medications, alternative treatments, surgery, and lifestyle changes. Meconium plug syndrome usually occurs in infants who are otherwise. Within this subset exists another entity known as small left colon syndrome, which presents in a similar fashion but is noted radiologically by the presence of a transition zone from a narrow caliber left.
Advances in the management of meconium aspiration syndrome. Normally, 98% of neonates pass meconium in the first 24 hours of life. Meconium plug syndrome is the intraluminal obstruction of the colon by abnormal meconium. It is generally regarded as a functional immaturity of the colon, resulting in failure to pass the first stool. Meconium aspiration syndrome mas is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. Usually, it is benign and not associated with other intestinal dysfunction. Meconium plug syndrome functional colonic obstruction in the fullterm neonate is. Across the united states, church leaders use this common planning strategy to fix perceived problems and challenges. The disease is diagnosed and treated by radiographic contrast enema, but hirschsprung disease and cystic fibrosis should be excluded. Meconium plug syndrome and meconium ileus springerlink. Anal stenosis accounts for 20% of anorectal anomalies and treatment is with. These conditions are considered separately from functional or anatomic causes of neonatal intestinal obstruction, such as hirschsprung disease, intestinal atresia, and anorectal malformations. In theotherreport, meconium plug syndrome is in fact defined as meconium located in the colon but does not speak of whether the population is meconium exclusively in the colon 4.
Meconium disease in aschrafts pediatric surgery 5th edition. Meconium plug syndrome usually occurs in infants who are otherwise healthy. Meconium plug syndrome merck manuals consumer version. Meconium plug syndrome is most often a benign cause of obstruction that clears after rectal stimulation or administration of contrast enema.
Meconium forms a cast of the colon, and the colon remains normal in caliber. Infants present with obstipation, abdominal distention, and, finally, vomiting as in other forms of distal bowel obstruction. Meconium plug syndrome usually occurs in infants who are otherwise healthy, but it is more common among premature infants or infants born to diabetic mothers or mothers with severe preeclampsia or eclampsia who were given magnesium sulfate. Meconium plug syndrome msd manual professional edition. The clinical picture is frequently indistinguishable, without contrast study of the colon, from other forms of mechanical intestinal. In biology, meconium describes the metabolic waste product from the pupal stage of an insect that is expelled through the. About 50 to 90% of neonates with hirschsprung disease fail to pass meconium in the first 48 hours of life. Meconium plug syndrome is the intraluminal obstruction of the colon by. Meconium plug syndrome extremely hard portion of meconium that has completely blocked intestinal lumen causing bowel obstruction. The meconium plug syndrome mps is a common cause of low intestinal obstruction in newborn infants. May be associated with downs syndrome and multiple endocrine neoplasia type iia. The success of nonoperative treatment is probably a result of the lower site and. In this situation, it is also termed the small left colon syndrome.
Meconium stained amniotic fluid and meconium aspiration syndrome incidence consequences diagnosis treatment key points references incidence meconium can be found in the gastrointestinal tracts of fetuses as early as 1416 weeks gestational age. Features are consistent with meconium plug syndrome. It is usually transient and affects the left colon with meconium plugging the bowel distal to this segment. In meconium plug syndrome, there is meconium obstruction of the colon, often seen in infants of diabetic mothers or mothers who received magnesium sulfate for eclampsia. Meconium plug syndrome neonatology diseases and disorders. The meconium plug syndrome roentgen evaluation and differentiation from hirschsprungs disease and other pathologic states trn he meconium plug syndrome isa relatively frequent cause of colonic mechanical obstruction in the newborn272527 and isrelated tothefailure to pass meconium inthe first days oflife. Meconium plug syndrome pediatrics msd manual professional. The meconium blockage syndrome, also known as the meconium plug syndrome 1 or temporary meconium ileus 2, was described over a decade ago. Hirschsprung malrotasi volvulus atresia intestinal kelainan mekonium meconium plug syndrome mps caty, michael. The meconiumplug syndrome is unrelated to cystic fibrosis and meconium ileus.
Hirschsprung disease msd manual professional edition. Meconium diseases in infancy cannot be neatly separated into discrete categories of meconium plug syndrome, meconium ileus, and meconium peritonitis. Ob viously the presence of this sign in mi was not necessarily a contraindication for treatment with contrast enema, as proposed before 7. However, it may sometimes be difficult to transport the neonates to the fluoroscopy room 9. Meconium plug syndrome symptoms, diagnosis, treatments and.
Meconium aspiration syndrome mas is defined as respiratory distress in newborn infants born through meconium stained amniotic fluid msaf whose symptoms cannot be otherwise explained. That we had no patients with cystic fibrosis supports a theory that a meconium plug obstruction in the colon is a different disease. Pulling the plugmanagement of meconium plug syndrome in. No patients were diagnosed with cystic fibrosis, meconium ileus, malrotation, or intestinal atresia.
Meconium plug syndrome is a diagnosis of exclusion. A mass of inspissated meconium in the distal colon or rectum is a relatively common cause of neonatal intestinal obstruction. This contrasts to the intraluminal obstruction of the terminal ileum in meconium ileus. The meconium plug syndrome is unrelated to cystic fibrosis and meconium ileus. Infants with meconium plug present with delayed passage of meconium, abdominal distention, and perhaps vomiting. Meconium stained amniotic fluid and meconium aspiration. Abdominal xray and contrast enema demonstrating multiple loops of dilated bowel proximal to a colon packed with retained meconium. Longterm outcome after neonatal meconium obstruction.
The word meconarion means from poppy, or like opium, suggestingthat the ancients believed it to bethe substancemaintaining the foetus asleep in theuterus. The meconium plug syndrome should always be considered in the differential diagnosis of low small bowel or colonic obstruction in the newborn infant, as prompt diagnosis and adequate medical treatment will in most cases completely resolve the problem and avoid an unnecessary surgical procedure. The term meconium disease refers to meconium ileus and meconium plug syndrome. The modern definition of the term was made by clatworthy and his coworkers in 1956 when they noted that newborn infants with intestinal obstruction due to the inability of the colon to rid itself of the meconium residue of nine months of fetal life. The primary differential consideration is hirschsprung disease, which is diagnosed eventually in approximately 1030% of patients with apparent meconium plug syndrome. This can cause breathing difficulties due to swelling inflammation in the babys lungs after birth. Meconium plug syndrome is a transient functional abnormality of the newborns digestive system which consists of the delayed passage of the meconium beyond 2448 hours, abdominal distension, vomiting, and dilatation of the left colon. Meconium plug syndrome must be differentiated from other diseases that cause a failure to pass meconium or abdominal distension in infants, including small left colon syndrome. The meconiumplug syndrome and hirschsprungs disease. Meconium plug syndrome radiology reference article.
Meconium ileus in cystic fibrosis journal of cystic fibrosis. Meconium plug syndrome refers to a functional colonic obstruction in a newborn due to an obstructing meconium plug. Diagnosis is based on radiographic contrast enema and sometimes testing for hirschsprung disease. Meconium plug syndrome associated with cystic fibrosis and. Neonatal intestinal obstruction pediatrics clerkship the. Meconium plug syndrome, also termed functional immaturity of the colon, is a transient disorder of the newborn colon characterized by delayed. The association of mps and cf was noted to have been previously reported in only one patient. Meconium plug syndrome merck manuals professional edition. Meconium plug syndrome is colonic obstruction caused by thick meconium.
A condition that can occur in newborns where a mass of thickened meconium obstructs the large intestines. Treatment may include suctioning the newborns mouth as soon as the head emerges during delivery, deep suctioning of the. Meconium ileus mi is often the first manifestation of cystic fibrosis cf and occurs in approximately 20% of patients diagnosed with cf. Meconium plug syndrome may be a sign of other disorders.
Presents with vomiting, abdominal distension, andor enterocolitis. Meconium plug syndrome, meconium ileus, and meconium ileus equivalent are conditions of gastrointestinal tract obstruction by thickened contents which will not pass. Meconium plug syndrome mps is a relatively benign cause of bowel obstruction in neonates in whom the passage of meconium is delayed during the first 2448 h of life. Meconium blockage syndrome in prematurity is a distinct entity from the diseases described above. Meconium plugs found on contrast enema are associated with a.
Sonographyguided gastrografin enema for meconium plug. Initial treatment is bowel irrigation, followed by. Meconium aspiration syndrome is a serious condition in which a newborn breathes a mixture of meconium and amniotic fluid into the lungs around the time of delivery. Hymansonandkahn, 1919, butit is only recently when serologists have. Request pdf pulling the plugmanagement of meconium plug syndrome in neonates the significance of meconium plug syndrome mps is unclear but has. Generally, infants with meconium plug syndrome have normal bowel function after. Most of the time, surgery is the only cure for a hernia. This diagnosis should not bemade without excluding cystic fibrosis and hirschsprungs disease, asboth ofthese entities may present astile meconium plug syndrome fig. It focuses on the medical and surgical management emphasizing the importance of nutrition and a multidisciplinary approach to improve both shortterm and longterm outcomes for.
Plain film diagnosis in meconium plug syndrome, meconium ileus. We describe the cases of three infants in whom there was intestinal obstruction consistent with the mps and in which cystic fibrosis cf was later documented. In a barium enema, meconium plug syndrome shows a normal or dilated colon as compared to microcolon in meconium ileus. Mas can present with varying degrees of severity from mild respiratory distress to. We present a unique case of meconium plug syndrome associated with both cystic fibrosis and hirschsprungs disease. Hirschsprung disease due to presence of meconium plug syndrome and normal velocity stool with rectal stimulation however, both of these exams are limited 15% of infants with meconium plug syndrome also have hirschsprung disease 32% percent who had inconclusive findings on contrast enema were ultimately diagnosed with hd. Pulling the plug management of meconium plug syndrome in neonates. The purpose of this study was to evaluate the usefulness of sonographyguided gastrografin enema for the diagnosis and treatment of meconium plug syndrome. The condition may occur as a result of other disorders such as cystic fibrosis, colon atresia, narrowed colon, impaired intestinal motility hirschsprung disease or for no apparent reason. Meconium has interested people for a long time davy, 1844. Meconium blockage syndrome encompasses a wide spectrum of diseases including meconium ileus, transient functional ileus of sick newborn, and meconium plug syndrome in fullterm neonates. Contrast enema usually eliminates congenital small bowel obstruction and rare colon abnormalities such as atresia and duplication. But there are cases where you dont need to go under the knife. Cystic fibrosis presenting with the meconium plug syndrome.
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