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Meconium syndrome and short bowel syndrome
- 1. Saleamlak T.(PSR-IV) March, 2019 MODERATOR Dr. Hanna G. ( consultant pediatrics surgeon)
- 2. OUTLINESOUTLINES Meconium syndrome Case scenario Introduction MECONIUM ILEUS Historical perspective Epidemiology Genetics Pathogenesis of CF Pathogenesis of MI Classification Clinical Presentation Diagnosis Management Meconium plug syndrome Meconium disorder of prematurity MI equivalent Short Bowel Syndrome Introduction Epidemiology Etiology Pathophysiology Clinical course Intestinal adaptation Treatment
- 3. Case scenarioCase scenario Hx. A 36hr old neonate has fed poorly & has now developed abdominal distention. He is full term infant with no prenatal problem. He vomited his two past feedings and just vomited a small amount of bile. He has no passed meconium. P/E v/s – normal wt 2.9kg Abdomen- markedly distended with few visible bowel loops but soft and nontender PR – some white mucous with no meconium Ix CBC- normal Abdominal x ray
- 4. IntroductionIntroduction The meconium syndromes Encompass multiple gastrointestinal disorders leading to intestinal obstruction from meconium or foreign material This group of diseases 1)Meconium ileus (MI: simple and complex) 2)Meconium plug syndrome 3)MI equivalent 4)Meconium Disease of Prematurity
- 5. MECONIUM ILEUSMECONIUM ILEUS Meconium ileus (MI) Is one of the most common causes of intestinal obstruction in the newborn Accounting for 9–33% of neonatal intestinal obstructions Characterized By extremely viscid, protein-rich, inspissated meconium Causing an intraluminal obstruction in the distal ileum, usually at the ileocecal valve Often the earliest clinical manifestation of cystic fibrosis (CF) 10-20% of patients with CF Often considered pathognomonic for CF
- 6. MI: Historical perspectiveMI: Historical perspective Landsteiner in 1905Landsteiner in 1905 In 1936, FanconiIn 1936, Fanconi In 1938, AndersonIn 1938, Anderson In 1948, Hiatt and Wilson • Enterotomy and saline irrigation In 1957, Bishop and Koop • Proximal end-to-side ileal anastamosis, and distal ostomy In 1961, Santulli • Side -to-end anastomosis and proximal stoma In 1953, Gross • Mikulicz side-by-side enterotomies
- 7. EpidemiologyEpidemiology Occurring primarily in white populations The meconium syndromes are closely linked to CF CF is one of the most common serious genetic diseases in whites 5% -6%-carriers of the genetic defect The incidence 1 in every 2,500 childbirths in USA Transmission An autosomal recessive trait CF and MI 80-90 % MI have CF MI will be the initial clinical manifestation in 10% to 20% of affected infants 10-30% of MI have a family history of CF Gender Almost equal MI is uncommon in premature infants
- 8. GeneticsGenetics Located on the long arm of chromosome 7 Encodes the cystic fibrosis transmembrane regulator (CFTR) Chloride channel protein that resides in the apical membrane of epithelial cells Defective chloride transport in the apical membrane of epithelial cells Respiratory Gastrointestinal Biliary ,pancreatic, and Reproductive systems
- 9. Pathogenesis of CFPathogenesis of CF CF is a systemic illness Diverse clinical presentations in various exocrine glands throughout the body Abnormally thick and viscous mucous secretions May obstruct the bronchoalveolar tree, pancreatic ducts, and intestinal tract Nasal mucus membranes, sweat and salivary glands, liver, and reproductive organs are also frequently affected Common manifestations pancreatic insufficiency---90% DM---------- 20% Obstructive biliary disease----15-20% Meconium ileus-----------10-20%
- 10. Pathogenesis of MIPathogenesis of MI The secretion of hyperviscous mucus and subsequent intestinal obstruction begins prenatally Nature of meconium in MI Low water content, minerals, and protein-bound carbohydrate Elevated albumin, mucoprotein, and calcium Possible causes of hyperviscousity Abnormal intestinal glands Pancreatic insufficiency Abnormal concentrating ability of proximal intestine
- 11. Pathogenesis of MIPathogenesis of MI Common location of inspissated meconium Distal ileum and proximal colon Leads distal intestinal obstruction Proximal bowel dilatation Meconium becomes hard pellets Increased absorption of fluid Colon Disuse atrophy Small caliber colon or microcolon
- 12. ClassificationClassification MI is usually classified A.Simple (uncomplicated) B.Complex (complicated)
- 13. Simple Meconium IleusSimple Meconium Ileus The distal ileum becomes obstructed By abnormal meconium as a result of a simple obturation The proximal ileum Thickened , dilated, and packed with tarlike meconium Distal to the site of obstruction Collapsed bowel Contains concrete, puttylike or beadlike pellets of gray, inspissated meconium Microcolon Poorly developed May contain small pellets of meconium.
- 14. Complex Meconium IleusComplex Meconium Ileus Ischemia Volvulus Perforation Meconium peritonitis Intestinal necrosis or atresia Pseudocyst Bacterial peritonitis
- 15. Meconium PeritonitisMeconium Peritonitis An aseptic, chemical, or foreign-body reaction Resulting from prenatal spillage of meconium into the peritoneal cavity due to intestinal perforation Three pathologic types Generalized If perforation occurs late prenatal or early neonatal period Fibroadhesive Most common Perforation occurs early before delivery Calcification occurs Cystic If sealing is not effective • pseudocyst Giant cystic meconium peritonitis
- 16. Clinical PresentationClinical Presentation Simple Meconium Ileus Hx Often SGA, rarely premature Relatively normal within the 1st 24hrs • Except they may not pass meconium spontaneously Following birth • Progressive abdominal distention, bilous vomiting Maternal polyhydramnios- in 20% P/E Distended ,doughy loop of bowel • Indent on palpation PR • Small rectum and anus • Classic finding Presence of white mucous rather than meconium
- 17. Clinical PresentationClinical Presentation Complex Meconium Ileus More severely ill Commonly presents immediately after birth Features of peritonitis Respiratory compromise Hypovolemia or shock • Due to third space losses
- 18. Diagnostic workupDiagnostic workup Cystic Fibrosis Sweat test Definitive diagnosis study for CF • Na+ and Cl- level >60mEq/L Boerhinger Mannhiem test Occasionally used • < 4-6 weeks Measurement of albumin level in the meconium • >80mg/ 1g of meconium Antenatal and neonatal genetic screening
- 19. Prenatal (Radiologic)Prenatal (Radiologic) Prenatal ultrasonography Echogenic bowel after 20weeks of Gestation Dilated bowel loops Intraabdominal calcification Ascites Polyhydramnios Intra -abdominal cysts
- 20. PostnatalPostnatal Plain film Features of simple MI Dilated intestinal loops of various size Relative absence of air fluid level Soap bubble • Singleton’s sign or Ground glass appearance • Neuhauser’s sign Features of complicated MI More severe distention Features of atresia or volvulus Air fluid level • Cystic Scattered calcification
- 21. PostnatalPostnatal Contrast enema If simple MI is suspected Features Empty microcolon with inspissated pellets outline within the terminal ileum Bolus of impacted meconium outline as filling defect Failure to pass contrast beyond atretic segment Abd. u/s
- 23. Non-Operative treatmentNon-Operative treatment Volume resuscitation Gastric decompression Respiratory support if needed Empirical antibiotics Hyperosmolar enema washout
- 24. Hyperosmolar EnemaHyperosmolar Enema Aims For hydration and softening of meconium mass Techniques Must be performed under fluoroscopic control Slow infusion to the rectum Post procedure Meconium pellets usually will pass in the next 24-48 hours Warm saline enemas with 1% N-acetylcysteine After successful evacuation 5 ml of 10% Nacetylcysteine through NGT Q6 hours Consider pancreatic enzymes if suspecting CF Success rate 63-83%
- 25. Hyperosmolar EnemaHyperosmolar Enema Complications Intestinal perforation Colonic mucosal and submucosal inflammation Necrotizing enterocolitis Hypovolemic shock Death
- 26. Operative ManagementOperative Management Indications: Simple MI Inadequate meconium evacuation or complications of contrast enema Failure of hyperosmolar enema to promote passage of meconium in 24-48 hours Complicated MI ALL • Except :asymptomatic, extraluminal intraperitoneal, calcified meconium, or contained-free perforation
- 27. PrinciplesPrinciples Manual evacuation Enterotomy Intraoperative saline irrigation, Mechanical separation of pellets from bowel wall Resection of the extremely dilated proximal segment (in order to avoid size discrepancy) anastomosis Complication is leak The other option; Resection , with creation of stoma
- 28. Operative ManagementOperative Management Three procedures can be used: 1) Enterostomy and decompression 2) Resection and stoma formation; or 3) Resection and anastomosis
- 29. Enterostomy and decompressionEnterostomy and decompression Enterotomy and irrigation Appendiceal irrigation Tube enterostomy
- 30. Resection and stoma formationResection and stoma formation Mikulicz procedure Bishop–Koop procedure Santulli–Blanc enterostomy
- 31. Mikulicz procedureMikulicz procedure Mikulicz Double barrel enterostomy Advantages: Less operative time Risk of leak is avoided Solubilizing agents can then be administered in distal and proximal loops Disadvantages: Potential high stoma output and fluid losses Long resections can result in short bowel The need for another surgery to restore continuity in later time
- 32. Bishop–Koop procedureBishop–Koop procedure Resection and Anastomosis Between the end of the proximal segment and the side of the distal segment of bowel The distal end is brought out as the ileostomy Advantage The intestinal contents preferentially pass into then distal ileum and colon Management of the distal obstruction through the ileostomy Disadvantage Loss of bowel length at the time of the initial procedure The need for an intraperitoneal anastomosis, and The need for a second operative procedure
- 33. Santulli–Blanc enterostomySantulli–Blanc enterostomy Distal limb end is anastomosed to the side of proximal limb Advantage Enhanced proximal irrigation and decompression No need for evacuation at the time of surgery Disadvantage Risk of high output stoma Consequent electrolyte disturbance and dehydration
- 34. Primary resection and anastomosisPrimary resection and anastomosis Resection of the obstructed segment Irrigation of remaining pellets in the distal bowel Ileocolic anastomosis Disadvantages : Anastomosis complications Resection of additional bowel Terminal ilium containing pellets with the dilated segment of ileum
- 37. Post-operative managementPost-operative management Resuscitation, maintenance fluids Replacement of fluid losses Through NGT or ileostomy 2 or 4% N-acetylcystein via NGT or ileostomy Workup of Cystic fibrosis Keeping the placed stoma in mind for future closure Respiratory physiotherapy
- 38. Nutrition SupportNutrition Support As bowel function resumes Oral feeding can start with breast milk or infant formula Supplemental pancreatic enzymes and vitamin Those with long post-surgical course might require enteral feeds or TPN watch for TPN associated cholestasis Glutamine-enriched formulas to the distal bowel may be tried to enhance bowel growth
- 39. COMPLICATIONS OF MECONIUMCOMPLICATIONS OF MECONIUM ILEUS AND CYSTIC FIBROSISILEUS AND CYSTIC FIBROSIS Gastroesophageal Reflux Disease Biliary Tract Disease Distal Intestinal Obstruction Syndrome Appendicitis Intussusception Fibrosing Colonopathy
- 40. Meconium Plug SyndromeMeconium Plug Syndrome MPS is a transient form of distal colonic obstruction caused by inspissated and dehydrated meconium MPS was first reported by Clatworthy in 1956 Described a syndrome of colonic obstruction because of inspissated meconium Also called functional immaturity of the colon The most common form of functional bowel obstruction in the newborn Incidence of 1/500 newborns
- 41. EtiologyEtiology The exact cause is not known The abnormality Due to functional immaturity of the colon in the newborn
- 42. Associated ConditionsAssociated Conditions Hirschsprung’s disease (10–30 %) and cystic fibrosis (30–40 %) Other risk factors
- 43. Clinical FeaturesClinical Features Delayed passage (> 24–48 h) of meconium and intestinal dilatation Premature newborns that are otherwise normal The impacted meconium leads to: Failure to pass meconium Abdominal distension Vomiting
- 44. Differential DiagnosisDifferential Diagnosis Hirschprung’s disease Neuronal intestinal dysplasia Visceral neuropathies Meconium ileus Small left colon syndrome, and Megacystis -microcolon-hypoperistalsis syndrome
- 45. DiagnosisDiagnosis Clinical Delayed passage (> 24–48 h) of meconium Premature newborns that are otherwise normal Features distal intestinal obstruction Plain abdominal x-ray: Moderately distended loops Absence of rectal gas suggests a low gastrointestinal tract obstruction Contrast enema and Treatment: Are not only diagnostic but therapeutic as well Only rarely is a second enema needed surgery is not indicated
- 46. Meconium Disease of PrematurityMeconium Disease of Prematurity Premature infants pass meconium later than term infants The meconium becomes obstructive These infants become symptomatic. The diagnosis is usually made when the infant is between 5 and 30 days of age They have a distended but otherwise benign abdomen and are not systemically ill.
- 47. Abdominal x ray Resemble those of meconium ileus With dilated loops and a paucity or absence of air-fluid levels Contrast enemas Should be obtained for diagnostic as well as therapeutic reasons. Very dilute Gastrografin (1:5) or Iso -osomolar contrast is recommended In these tiny and vulnerable patients, a trip to radiology has risks. Have no increased risk of cystic fibrosis or Hirschsprung disease
- 48. Distal Intestinal Obstructive SyndromeDistal Intestinal Obstructive Syndrome The term meconium ileus equivalent has now been replaced by the term distal intestinal obstructive syndrome (DIOS The current, and most clinically relevant definition Fecal obstruction unresponsive to usual laxatives Patients with DIOS are almost exclusively older than 5 years of age
- 49. Case scenarioCase scenario Hx. A 36hr old neonate has fed poorly & has now developed abdominal distention. He is full term infant with no prenatal problem. He vomited his two past feedings and just vomited a small amount of bile. He has no passed meconium. P/E v/s – normal wt 2.9kg Abdomen- markedly distended with few visible bowel loops but soft and nontender PR – some white mucous with no meconium Ix CBC- normal Abdominal x ray
- 50. Short Bowel SyndromeShort Bowel Syndrome
- 51. IntroductionIntroduction Short bowel syndrome (SBS) A term that is loosely used to define the pathophysiologic disorders that result from the removal of a large portion of the small intestine Reasons An anatomic loss or deficiency of intestinal surface area Normal intestinal mucosal surface area but perturbed intestinal absorption, motility, or both The clinical syndrome leads to Malnutrition Weight loss Steatorrhea Diarrhea As a consequence of the inability of the gastrointestinal tract to absorb nutrients
- 52. IntroductionIntroduction Defn. Defined as intestinal failure Due to a loss of intestine resulting in inadequate length of bowel for maintaining the nutrition and hydration of the individual The minimum length of small bowel required 25 cm • In the presence of an intact ileocaecal valve (ICV) and colon 40 cm • Without an ICV and large bowel Defined as a need for prolonged PN following bowel resection Usually for more than 3 months
- 53. EpidemiologyEpidemiology Mercifully rare Incidence In Europe 2 per million USA Around 3 per 100,000 births per year. In Africa Unknown because survival is close to zero
- 54. EtiologyEtiology Congenital Atresia Gastroschisis HSD(long segment) Congenital Atresia Gastroschisis HSD(long segment) Acquired Midgut volvulus Mesenteric infarction e.g., sickle cell crisis NEC Adhesive band obstruction/strangula tion Trauma Acquired Midgut volvulus Mesenteric infarction e.g., sickle cell crisis NEC Adhesive band obstruction/strangula tion Trauma
- 56. PathophysiologyPathophysiology Water absorption The jejunum-44% Ileum -70% Colon -> 90% Salt absorption Jejunum-13% Ileum- 72% Colon- >90% Water absorption The jejunum-44% Ileum -70% Colon -> 90% Salt absorption Jejunum-13% Ileum- 72% Colon- >90%
- 57. Clinical courseClinical course Initial period Time extreme fluid and electrolyte loss Lasts 1-2weeks Intestinal adaptation period Starts as soon as 48hr and may continue for more than 1year All layers of remaining bowel are involved Predominantly mucosal hyperplasia • Increased villus height and crypt depth • Increased diameter and bowel length Plateau or maintenance period Reached after 1-2 year Time when adaptive response has maximized
- 58. INTESTINAL ADAPTATIONINTESTINAL ADAPTATION 1. Morphologic changes Taller villi, deeper crypts, and greater caliber and length of the intestine Serve to expand the mucosal digestive and absorptive surface area 1. Functional adaptation Adaptive increases in sodium and water absorption Active Na+/substrate transporters, Na+/H+ exchangers (NHE), and Passive Na+ channels Mechanisms and mediators Luminal nutrients Gastrointestinal secretions, and Humoral factors
- 59. TreatmentTreatment Medical and Nutritional management Surgical Management
- 60. Medical and Nutritional managementMedical and Nutritional management In the immediate period Fluid and electrolyte balance Management of Gastric hypersecretion Nutritional therapy TPN Nearly all patients with SBS will require parenteral nutrition to survive the period while the bowel adapts Enteral feeding How to monitor EN tolerance • Stool output : >40-50ml/kg Reduce the rate and provide 8hrs bowel rest • Gastric aspirate If more than 4x previous hours infusion Withhold feeds for 12hrs
- 61. Medical and Nutritional managementMedical and Nutritional management Pharmacologic agents used to slow intestinal transit Diphenoxylate , and loperamide Antibiotics Dugs that promote adaptation Glutamine-0.5g/kg/day orally
- 62. How can we Give nutritional management In short bowel syndrome in low-income countries? How can we Give nutritional management In short bowel syndrome in low-income countries?
- 63. Triple tube therapyTriple tube therapy
- 67. SURGICAL OPTIONSSURGICAL OPTIONS Indications Complications of parenteral nutrition Failure to advance enteral nutrition Worsening tolerance of enteral feeding Time of surgery Not too early or not too late A period of at least 1 year should be the minimum interval Goal of the intervention To increase intestinal absorptive capacity by solving the following abnormalities Rapid intestinal transit – intestinal valves, colonic interposition Decreased mucosal surface area- intestinal lengthening, SBtx Ineffective peristalsis- tapering Enteroplasty Reduced intestinal length- intestinal lengthening, SBtx
- 68. Algorithm for sequentialAlgorithm for sequential advance for SBSadvance for SBS
- 69. INTESTINAL VALVESINTESTINAL VALVES Intestinal valve A.The outer seromuscular layer is circumferentially stripped Leaving the underlying mucosa intact A.The now redundant mucosa is intussuscepted to create a valve B.Finally, the serosa is reapproximated
- 71. INTESTINAL LENGTHENING PROCEDURESINTESTINAL LENGTHENING PROCEDURES Bianchi’s LILT procedure STEP
- 72. Bowel TransplantationBowel Transplantation Indications Unable to have TPN Usually due to TPN-related liver disease or Difficulty with venous access for TPN administration Types Isolated bowel For those with good liver function and normal motility Bowel plus liver For those with liver disease Multivisceral which includes liver, bowel, stomach, and pancreas For those with multiple abdominal organ failure and dysmotile bowel
- 74. ReferencesReferences Coran Pediatric Surgery, 7th ed Ashcraft’s pediatric surgery, 5th edition. Principles and practice of pediatrics surgery, 4th ed Pediatric Surgery (SpringerSurgery Atlas Series) Operative pediatric surgery, Lewis Spitz, Arnold G Coran. 7th edition Journal
Editor's Notes
- share various overlapping aspects in pathophysiology, clinical presentation, diagnostic techniques, and management. The most common of these gastrointestinal meconium disorders, and their critical pathogenetic and molecular relationship to cystic fibrosis (CF), are discussed in this chapter.
- Often the earliest clinical manifestation of cystic fibrosis (CF) occurring in approximately 16% of patients with CF Although MI can occur with other uncommon conditions such as pancreatic aplasia and total colonic aganglionosis It is often considered pathognomonic for CF MI may be an early indication of a more severe phenotype of cystic fibrosis, as suggested by significantly diminished pulmonary function found in children with a history of MI compared to age- and gender-matched children with CF who did not have MI
- First described by Landsteiner in 1905, MI is the newborn bowel obstruction caused by inspissated meconium, and associated with cystic degeneration and fibrosis of the pancreas. Pancreatic exocrine deficiency leading to the abnormal production of thick, viscous intestinal mucus was ultimately shown to be the primary cause of atypical meconium development In 1936, Fanconi first reported CF, noting the association of pancreatic insufficiency and chronic pulmonary disease (4). Guido Fanconi (English: /fɑːnˈkoʊni/) (1 January 1892 – 10 October 1979) was a Swiss pediatrician. He was born in Poschiavo, a small village in the Canton of Grisons. Fanconi is regarded as one of the founders of modern pediatrics. Dorothy Hansine Andersen (May 15, 1901 – March 3, 1963) was an American pathologist and pediatrician who was the first person to identify cystic fibrosis and the first American physician to describe the disease Shortly thereafter in 1938, Anderson established the critical link between CF and MI, characterizing the histologic similarities between the bowel and pancreatic tissues
- Occurring primarily in white populations, the meconium syndromes are closely linked to CF. CF is one of the most common serious genetic diseases in whites. Approximately 5% to 6% of white individuals are carriers of the genetic defect (3,8). The incidence is about 1 in every 2,500 childbirths in the United States (9,10,11). CF is transmitted as an autosomal recessive trait, thus both parents must be heterozygotes for the gene in order to have an affected child. Each offspring has a one in four chance of developing the disease. MI will be the initial clinical manifestation of this disorder in 10% to 20% of affected infants. Ten to 30% of patients diagnosed with MI have a family history of CF. In families in which the first CF child has had MI, 29% of subsequent siblings with CF have MI, compared with 6% in families in which the first child with CF did not have MI (12,13). The number of males and females affected is almost equal. MI is uncommon in premature infants
- Cystic fibrosis is a systemic illness with diverse clinical presentations in various exocrine glands throughout the body. Abnormally thick and viscous mucous secretions may obstruct the bronchoalveolar tree, pancreatic ducts, and intestinal tract. Nasal mucus membranes, sweat and salivary glands, liver, and reproductive organs are also frequently affected. Common manifestations of this disease include pancreatic insufficiency in 90%, diabetes mellitus in 20%, obstructive biliary disease in 15–20%, and meconium ileus in 10–20% of all patients. Azoospermia occurs in nearly all affected males (22). Exocrine glands throughout the body are affected by this chloride transport defect. Abnormally thick and viscous mucus secretions obstructing the bowel, the pancreatic ducts, and air passages in the lung cause the most severe clinical manifestations
- The secretion of hyperviscous mucus and subsequent intestinal obstruction begins prenatally. The meconium P.1229 involved in MI is typically low in water content, minerals, and protein-bound carbohydrate and has several biochemical abnormalities, including elevated albumin, mucoprotein, and calcium, as well as low trypsin levels (23,24). The decreased carbohydrate and increased protein concentrations likely account for the hyperviscosity of the meconium (25,26). Neonates with MI and CF have a meconium protein content of 80% to 90%, compared with 7% in normal infants (27). Most of this extra protein has been shown to be albumin (28). The high viscosity and tenacity of the meconium result from a combination of a hyperviscous mucus secreted by abnormal intestinal glands, abnormal concentrating mechanisms of the proximal small intestine, and pancreatic exocrine deficiency in utero. Infants with MI and CF have severely affected intestinal glands, but mild pancreatic disease. It is believed that the intestinal glandular abnormality may be the primary precipitating factor for the development of meconium ileus, with the pancreatic disease playing a secondary role (29).
- The distal intestinal lumen becomes obstructed with meconium, leading to proximal bowel dilatation. Inspissated meconium located in the distal ileum and proximal colon develops into hard pellets due to the increased absorption of fluid from the stool. Disuse atrophy in utero results in a small-caliber colon or microcolon
- In simple cases, the distal ileum becomes obstructed by abnormal meconium as a result of a simple obturation. The proximal ileum is thickened, dilated, and packed with tarlike meconium. Distal to the site of obstruction, the bowel is collapsed and contains concrete, puttylike pellets of gray, inspissated meconium. The meconium in the distal bowel sometimes has a beadlike appearance. Due to the proximal obstructive disease, there is a microcolon, which is poorly developed and may contain small pellets of meconium. There is no classic histologic appearance for MI; however, tissue sections of the bowel may show intracellular inclusion bodies and intestinal crypts filled with inspissated meconium
- In utero, simple obturation obstruction of the bowel may progress to complicated MI. Intestinal necrosis or atresia, meconium peritonitis, pseudocyst, or all these conditions may occur in cases of complex MI. Most often, this is related to volvulus or ischemia of the dilated segment of the proximal ileum. Depending on the time at which the volvulus occurs and the in utero evolution of the process, the complicated form of this disease may result in intestinal atresia, perforation, or meconium peritonitis. Bacterial peritonitis results from postnatal volvulus and perforation, but occurs infrequently
- Meconium peritonitis was first reported by Morgagni in 1761 in De Sedibus et Causis Morborum as an aseptic, chemical, or foreign-body reaction resulting from prenatal spillage of meconium into the peritoneal cavity due to intestinal perforation (34,35). Ischemic necrosis and perforation result from the large meconium bolus located P.1231 proximal to the obstruction. A segmental volvulus often occurs when the meconium-filled segment of redundant ileum twists. The narrow base of the volvulus may lead to ischemic necrosis of the bowel and result in isolated or multiple ileal atresias, formation of a stricture, or perforation. The perforation is typically secondary to segmental volvulus followed by gangrene with focal perforation (Fig. 77-4). Infants with complicated MI may present with intestinal perforation in the form of either free or encysted peritonitis. Three pathologic types of meconium peritonitis have been described, including generalized, fibroadhesive, and cystic (36). Generalized meconium peritonitis with ascites develops if the perforation and meconium extravasation occur in the late perinatal or early neonatal period. Diffuse distribution of meconium throughout the peritoneal cavity initiates the inflammatory process. However, due to the brief duration, no calcification occurs. The adhesions between bowel loops are more fibrinous, rather than fibrous, in generalized peritonitis. Most commonly, however, fibroadhesive meconium peritonitis occurs. When the perforation has occurred early enough prior to delivery, dense adhesions and calcification occur. Intraperitoneal meconium may form calcifications within 48 hours of perforation (37). The meconium contains digestive enzymes that induce an intense chemical peritonitis, initiated by the sterile meconium. Subsequently, dense, fibrous adhesions and agglutination of the bowel may result. The calcified and fibrous adhesions are often effective in sealing the site of perforation and frequently obscure identification of the site. When the fibroblastic reaction has not been effective in sealing the site of perforation, meconium may continue to leak into the peritoneal cavity, resulting in cystic meconium peritonitis. Segmental volvulus with ischemic necrosis of the bowel along with extravasation and liquefaction of meconium may lead to development of a pseudocyst. The pseudocyst consists of densely adherent, partially necrotic loops of intestine surrounding liquefied meconium. Frequently, a calcified fibrous peel or wall envelops this mass. This cyst may fill with air after birth and may then be called giant cystic meconium peritonitis (38). Colonic perforation distal to the obstruction may result from excessive intraluminal pressure from a contrast enema; however, this is not generally a difficult event to recognize and differentiate from the previous lesions.
- Infants with MI are often small for gestational age, but are rarely premature. The in utero bowel obstruction may result in lower birth weights in neonates with MI due to the decreased absorption of amniotic fluid-derived nutrients. Often the first 24 hours of life are relatively normal for most neonates with simple MI, except that they may not pass meconium spontaneously. Following birth, progressive abdominal distension occurs as the proximal small intestine fills with air, feedings, and intestinal secretions. In the next 24 to 48 hours, bilious vomiting occurs and there is little or no passage of meconium. Distended, doughy loops of intestine that indent on palpation are frequently observed on abdominal physical examination. These are the proximal, meconium-filled loops of small bowel. The rectum and anus are often small in these infants, and this may be misinterpreted as anal atresia or stenosis. A classic finding on rectal examination is the presence of white mucous rather than meconium, reflecting the fact that an obstruction is interposed between the ampulla of Vater and the anus. Although more common with complex MI, a maternal history of polyhydramnios occurs in nearly 20% of infants with simple MI. Associated congenital anomalies are rarely present
- Complicated MI or meconium peritonitis commonly presents immediately (within 24 hours) after birth. In the case of antenatal pseudocyst formation, with or without calcification, prenatal ultrasound diagnosis is often established in contemporary practice. If distal obstruction is present without perforation, such as with distal ileal atresia or volvulus, the infant may tolerate feeds for the first day of life prior to developing symptoms of obstruction. Neonates with complicated MI are more severely ill compared with those with simple MI. Progressive abdominal distension occurs that may be associated with erythema and edema of the abdominal wall. Pneumoperitoneum and peritonitis may also present with these findings. The erythema and edema of the abdominal wall usually indicates an underlying pseudocyst or peritonitis. The abdomen may be very tense, and there may be a palpable mass usually secondary to a volvulus. Neonates with meconium peritonitis or giant cystic meconium peritonitis frequently have abdominal distension at birth and have bile-stained gastric aspirates. Respiratory compromise secondary to the abdominal distension may be present. Third-space losses may lead to hypovolemia that left untreated may progress to severe cardiovascular instability. Some patients with complex MI may also become septic late in their course
- Prenatal ultrasonography may be helpful in diagnosing MI. The presence of echogenic bowel after 20 weeks&apos; gestation has been associated with MI (simple) and meconium peritonitis (40). This association is even stronger when screening fetuses with a family history of CF. Dilated bowel, intraabdominal calcification, or ascites are suggestive of complicated meconium ileus. Intrauterine intestinal perforation leading to intraperitoneal meconium causes calcifications in the abdomen or scrotum, whether the cause is MI or another event. Meconium peritonitis may be noted in utero, and the ultrasound usually demonstrates polyhydramnios, ascites, dilated fetal bowel, and intraabdominal echogenic masses (41,42). It is crucial to recognize that intraluminal calcified meconium is not associated with intestinal perforation and meconium peritonitis. This distinction may have a significant impact on the decision for and timing of surgical intervention Figure 1: Transverse view of the abdomen. A large, thick-walled pseudocyst partly filled with meconium is seen in the dependent (left) side of the fetal abdomen. A smaller, thick-walled pseudocyst, filled with meconium, is seen just superior to the larger one and just anterior to the fetal spine
- An enema using water-soluble contrast material should be performed when simple MI is suspected. This condition is characterized by an empty microcolon with inspissated pellets outlined within the terminal ileum. The bolus of impacted meconium is outlined as a filling defect. Neonates with atresia also have a small-caliber terminal ileum with dilated intestine proximal to the atresia. With MI, the contrast material may then be used to wash out the obstructing plugs and inspissated meconium. When complicated MI is suspected, a contrast enema is used primarily to confirm the diagnosis, if necessary. In patients with complicated MI (calcification, cyst), a contrast enema study is not required routinely prior to laparotomy because the plain films are usually sufficient to indicate the diagnosis. Contrast material should not be given by mouth in suspected cases of MI due to the risk of aspiration. The diagnosis of an intestinal atresia is confirmed when the contrast material does not pass the atretic segment. Sometimes the symptoms of the complications of MI predominate and an associated atresia may be discovered incidentally at operation. The pathologist may also make the diagnosis of MI during examination of a resected surgical specimen or by postmortem evaluation. Figure . Classic radiographic findings of meconium ileus are seen on this retrograde contrast study First, a “micro colon of disuse” is seen. The colon is extremely small and unused Second, inspissated pellets (filling defects) of meconium are seen in the more proximal small bowel. Third, note there is a small bowel obstruction as the contrast material has not reached the markedly dilated loops of small bowel.
- • Careful resuscitation, hydration, appropriate electrolytes repletion and maintenance of normothermia.
- Complications of hyperosmolar enemas include intestinal perforation, colonic mucosal and submucosal inflammation, necrotizing enterocolitis, hypovolemic shock, and death. A straight catheter, as opposed to a balloon catheter, is recommended for the enemas to prevent rectal perforation. Perforation of the ileum or cecum has been reported 12 to 48 hours after the enema is administered. Extreme bowel distention by the osmotic process or an injury to the intestinal wall by the contrast material are possible factors leading to this late perforation.
- Intestinal obstruction is the most frequent indication for operation. Patients with clinical or radiologic evidence of complicated MI, including volvulus, intestinal gangrene, perforation, peritonitis, or small bowel atresia require immediate surgery. The exceptions are asymptomatic prenatally diagnosed infants in which the process resolves before delivery. This occasionally occurs with asymptomatic, extraluminal intraperitoneal, calcified meconium, or contained-free perforation. Surgical intervention is also required for simple MI patients who fail contrast enema treatment
- After the diagnosis had been confirmed at laparotomy, a 14F T tube catheter with multiple holes cut in both limbs was prepared, each limb being 3 to 4 cm long. The tube was inserted through an enterotomy into the dilated and hypertrophied ileum about 3 cm proximal to the distal narrowed segment, and secured with a double purse string suture. It was immediately irrigated with saline and 5% acetyl cystine or Gastrografin to loosen the compacted distal inspissated pellets and proximal viscid meconium. The T tube was brought out through a stab incision in the right iliac fossa and the enterostomy was secured to the anterior abdominal wall. Starting the day after operation, the T tube was gently irrigated twice daily with a solution of 5% acetyl cystine or diluted Gastrogafin until complete decompression had been achieved. Parenteral nutrition was given until normal bowel function had returned, after which replacement of pancreatic enzymes was introduced orally and milk feeds were started. The T tube was removed 18-21 days after the operation.
- Abstract Meconium ileus accounts for 9–33% of all neonatal intestinal obstructions, with an incidence of 1:2500 newborns, representing the third most common cause of neonatal small bowel obstruction after atresia and malrotation. This study aimed to compare various surgical procedures used in the treatment of meconium ileus and to assess their efficacy regarding survival and complications. A retrospective study was done to all cases of meconium ileus admitted to the neonatal intensive care unit of Basrah hospital of maternity and children and Basrah children specialty hospital during the period of 10 years (2005 to 2015). The medical records of 57 cases of meconium ileus were studied. The comparison included: Mikulicz procedure, Bishop-Koop procedure and resection with primary anastomosis in both simple and complex meconium ileus. The parameters used for comparison were anastomotic leaks, high output diarrhea with dehydration and failure to thrive, sepsis, need for reoperation, wound complications, early adhesions, hospital stay and mortality. The mean age of presentation of neonates with meconium ileus was 3.9 days. Male to female ratio was 0.9: 1. About 10.5% were preterm. About 33.3% of cases were diagnosed as simple meconium ileus. Intestinal Volvulus is the predominant complications encountered (47.4%). Non-operative treatment was effective in 45.5%. The most common procedures done in our center were Mikulicz procedure (61.5%), followed by Bishop-Koop procedure (30.8%), and resection with primary anastomosis (7.7%). There was a significant association between mortality and high output fistula, anastomotic leaks, sepsis, and reoperation. Predominant complications in Mikulicz procedures were high output fistula (50%) and skin excoriation (53.1%), while in Bishop-Koop procedure were sepsis (75%), reoperation (50%), and adhesions (25%). In primary anastomosis, significant complications were anastomotic leak (75%), sepsis (50%), and reoperation (50%). Mortality was highest in primary anastomosis (75%), followed by Bishop-Koop procedure (62.5%), and lowest with Mikulicz procedure (40.6%). The overall mortality of meconium ileus was high 45.6% (42.9% for simple meconium ileus and 52.6% for complex meconium ileus). All neonates treated non-operatively survived, while the survival rate for those treated surgically was 50%. In conclusion, resection with stoma creation is superior to primary anastomosis. Mikulicz procedure is the safest procedure to be done with best survival and less complications. Bishop- Koop procedure is of value in a situation where the surgeon is afraid from high output diarrhea so proximal stoma is mandatory
- Meconium plug syndrome was first reported by Clatworthy in 1956. • He described a syndrome of colonic obstruction because of inspissated meconium. • Meconium plug syndrome is also called functional immaturity of the colon. • Meconium plug syndrome is the most common form of functional bowel obstruction in the newborn, with an incidence of 1/500 newborns. • Meconium plug syndrome is a transient disorder characterized by a delayed passage (&gt; 24–48 h) of meconium and intestinal dilatation. • In general, meconium plug syndrome is observed in premature newborns that are otherwise normal. • Cystic fibrosis and Hirschsprung’s disease may be associated with meconium plug syndrome and should be excluded
- The exact cause of meconium plug syndrome is not known. • The abnormality is due to functional immaturity of the colon in the newborn
- Associated Conditions • Prematurity: Meconium obstruction in prematures is a distinct entity. It occurs in premature infants who develop obstructive symptoms several days after having passed meconium initially. • Maternal diabetes has been associated with functional colonic obstruction. • Generally, infants with meconium plug syndrome have normal bowel function after passing meconium but there is an association with Hirschsprung’s disease (10–30 %) and cystic fibrosis (30–40 %).
- Is a transient disorder characterized by a delayed passage (&gt; 24–48 h) of meconium and intestinal dilatation. In general, observed in premature newborns that are otherwise normal. Cystic fibrosis and Hirschsprung’s disease may be associated should be excluded
- This includes: • Hirschprung’s disease, neuronal intestinal dysplasia, visceral neuropathies, meconium ileus, small left colon syndrome, and megacystis-microcolon-hypoperistalsis syndrome
- Is a transient disorder characterized by a delayed passage (&gt; 24–48 h) of meconium and intestinal dilatation. In general, observed in premature newborns that are otherwise normal. Cystic fibrosis and Hirschsprung’s disease may be associated should be excluded Features distal intestinal obstruction Failure to pass meconium Abdominal distension Vomiting This includes: • Hirschprung’s disease, neuronal intestinal dysplasia, visceral neuropathies, meconium ileus, small left colon syndrome, and megacystis-microcolon-hypoperistalsis syndrome Plain abdominal x-ray: The initial radiograph demonstrates numerous moderately distended loops of bowel with the absence of rectal gas which suggests a low gastrointestinal tract obstruction (Fig. 28.1a). Fig. 28.1 a and b Plain abdominal x-ray showing dilated bowel loops with absent gas in the rectum. Gastrograffin contrast enema showing meconium plug syndrome Recommended Reading 197 • Contrast enema: Gastrografin is a hypertonic solution containing both wetting and detergent agents. However, it is associated with complications secondary to hyperosmolarity which can lead to dehydration (Fig. 28.1b).
- Defined as intestinal failure Due to a loss of intestine resulting in inadequate length of bowel for maintaining the nutrition and hydration of the individual without either intravenous or oral supplementation With some reported exceptions, the minimum length of small bowel required for infant survival on enteral feeds is generally 25 cm in the presence of an intact ileocaecal valve (ICV) and colon, and 40 cm without an ICV and large bowel. Note that norms of intestinal length vary considerably, with a range of 250 cm to 300 cm of small bowel at term. The estimated length in a preterm infant of 26 and 32 weeks gestation is 70 cm and 120 cm, respectively. Thus, gestational age is an important factor. However, an infant is considered to have SBS when he or she behaves as if SBS is present, and the infant should be treated as such
- Short bowel syndrome is mercifully rare. In Europe, the incidence is estimated to be approximately 2 per million. According to Gupta et al.,1 the incidence of SBS in neonates is around 3 per 100,000 births per year. In Africa, the incidence is unknown because survival is close to zero
- Some of the common causes of short bowel syndrome SBS can be congenital, but is more generally acquired from surgical resection of bowel. Of the congenital bowel atresias type 3b (“apple peel” type) and type 4 (multiple atresias, “string of sausages” type) are most likely to result in SBS. Functional SBS can also occur where there is severe malabsorption despite adequate bowel length or intact bowel
- slowest transit time, between 24 and 150 hours. The efficiency of salt and water absorption also varies in the different parts of the intestine. The jejunum is very inefficient, with an efficiency of water absorption of 44% compared to 70% in the ileum and greater than 90% in the colon. The corresponding estimates for efficiency of salt absorption are 13% in the jejunum, 72% in the ileum, and greater than 90% in the colon By removing specific portions of the small bowel, certain complications become more prevalent Jejunectomy =produces no permanent defect in the absorption of macronutrients and electrolytes because the ileum is capable of taking over these absorptive functions =Several of the intestinal hormones responsible for inhibiting gastric secretion are distributed mainly in the jejunum, and therefore, jejunectomy is more likely to result in gastric hypersecretion In contrast, the ileum has a pronounced effect in slowing intestinal transit Thus, ileal resection generally results in more rapid intestinal transit The ileum is also an essential site for the absorption and recycling bile salts As such, bile salt waste is associated with extensive ileal resection. Under these circumstances, the bile salt pool becomes depleted, leading to a high incidence of cholelithiasis and malabsorption of fat (11). It is for this reason that prophylactic cholecystectomy may be beneficial in the setting of SBS. The malabsorption of fat leads to deficiency of fat-soluble vitamins A, D, E, and K. In addition, vitamin B12 malabsorption after ileal resection may necessitate parenteral vitamin B12 on a monthly basis
- The final stage following massive small bowel resection is the plateau phase, which occurs following maximal adaptation. This stage is generally reached within 1 to 2 years from the time of resection. It is during this final stage that decisions regarding additional surgical intervention are generally undertaken. Remedial surgical procedures may be indicated earlier in the face of the appearance of liver dysfunction or failure to advance enteral calories. .
- Because diarrhea is a common complication of massive intestinal resections, adaptive increases in sodium and water absorption are particularly important. Active Na+/substrate transporters, Na+/H+ exchangers (NHE), and passive Na+ channels facilitate sodium absorption and the subsequent passage of water and chloride (21). NHE activity, as well as mRNA and protein levels of the NHE-3 isoform, are increased in the residual intestine after resection (21). The Na+/glucose cotransporter is the primary mechanism for sodium transport in the small bowel (22). Schulzke et al. found a 2.5-fold increase in glucose-dependent sodium absorption per centimeter of intestine following 70% SBR in rats (23). Multiple mechanisms and mediators have been proposed to be required for the initiation and maintenance of the postresection adaptation response. There is strong evidence to implicate a role for luminal nutrients, gastrointestinal secretions, and humoral factors in the genesis of adaptation. Further, the nutritional status of the host, as well as neural, bacterial, and mechanical factors, have become more clear recently.
- Pharmacologic agents can also be used to slow intestinal transit, thus improving nutritional absorption and potentially allowing the patient to wean from parenteral nutrition. The mainstay of pharmacologic therapy to slow intestinal transit has been the use of opioid substances. Opioid agents most commonly used to treat diarrhea include codeine, diphenoxylate, and loperamide. Loperamide has been demonstrated to be more effective than diphenoxylate in the control of diarrhea in several clinical trials (48,49). Codeine, although quite effective for the control of diarrhea, has significant central nervous system side effects and a clear abuse potential glutamine contributes to the proliferative effects of several different endogenous growth factors such as epidermal growth factor (EGF) (31) and insulinlike growth factor-1.
- a novel enteral feeding technique for short bowel syndrome in low-income countries Schematic diagram showing the methodology of triple tube technique. Catheter A is inflated into the enterostomy to collect small bowel effluent, Catheter B is inflated into the mucous fistula and used to feed the colon, and catheter C is inflated into the rectum. In this small series of SBS, resection of ICV did not influence survival. We believe that in these patients the balloon Foley catheter A in the enterostomy acted as an artificial valve allowing the residual small bowel to adapt normally. Second, in patients with SBS, the colon adopts important digestive functions of absorption of short-chain fatty acids, water, and electrolytes so that extensive small bowel losses can be better tolerated Third, the balloon catheter C in the rectum helps in retarding rapid transit of colonic contents, allowing near-complete absorption of feeds in the colon
- 10 neonates underwent extensive bowel resection with creation of an enterostomy and mucous fistula for NEC or MGV In the postoperative period, with return of stoma function, enteral feeding was started using the triple tube technique Briefly, a No. 8 Foley catheter (A) was inserted into the proximal stoma and clamped. At a regular interval (4 to 6 hours) the clamp was released to collect the effluent, which was emptied into an appropriately sized Luer syringe. The energy requirement was calculated for each patient The stoma output was mixed with a semi elemental diet containing medium-chain triglycerides (Pregestimil, Mead Johnson, USA) and reinfused into the mucous fistula over the same period through a No. 8 Foley catheter (B) that was advanced into the stoma for a distance of about 5 cm. Continuous infusion was carried out by microdrip infusion and increased as tolerated to 0.67 kcal/mL. A third Foley catheter (C) was placed in the rectum and clamped. The clamp was released once in 6 hours or earlier if there was abdominal distention. Tolerance was determined according to the stool volume and stool pH. For those patients who developed abdominal distention or diarrhea, feeding was discontinued for 6 hours and than restarted at a slow rate. The feeding protocol was 25% of the calculated feed with breast milk orally and 75% through catheter B. Daily weight charting and arterial blood gas analysis for metabolic acidosis was done during the first 2 weeks and weekly thereafter. If there was pericatheter leak from any of the Foley catheters the balloon was deflated for 4 hours and again inflated. The feeding protocol was continued by the parents at home, using a microdrip set. Intestinal continuity was reestablished after ensuring distal bowel patency by a contrast study. It was accomplished by a single-layer, end-to-end anastomosis with an absorbable suture. After resumption of bowel movement, the babies were fed orally and the rectal catheter C was inflated and deflated in the same manner until the neonates passed formed stools. Follow-up consisted of monthly weight charting and arterial blood gas analysis
- Prevention of SBS is the earliest and best approach to this problem. Timely surgical intervention in patients with NEC and/or volvulus and a conservative approach to intestinal resection are important principles. Once the patient is beyond the initial operation, the ultimate goal is to provide all calories via the enteral route and to discontinue parenteral nutrition. Patients often develop complications related to parenteral nutrition or reach a point where stool output and/or electrolyte losses limit the ability to advance enteral feeding. In addition, some patients may actually worsen and require increasing, rather than decreasing amounts of parenteral nutrition. Those that fall into these groups (complications of parenteral nutrition, failure to advance enteral nutrition, worsening tolerance of enteral feeding) are those in whom operative intervention should be considered. The optimal timing of remedial operative intervention is important. If surgery is performed too early, it might be unnecessary, as the normal postresection adaptation or intestinal lengthening due to normal growth may prevent the need for long-term parenteral nutrition. If offered too late, the patient may suffer complications, as well as the added cost of prolonged parenteral nutritional support. A period of at least 1 year should be the minimum interval that intestinal adaptation should be allowed to improve the ability to tolerate enteral feeding. After this length of time, if a patient is still making progress with regard to tolerance of enteral feeding, operative intervention should be postponed. However, this approach may need to be reconsidered in the context of complications of parenteral nutrition The major goal of surgical intervention is to increase intestinal absorptive capacity. Most surgical procedures have been created to address the specific anatomic and physiologic abnormalities of the intestine in patients with SBS. These abnormalities include rapid intestinal transit, decreased mucosal surface area, ineffective peristalsis, and reduced intestinal length. The various surgical procedures may therefore be categorized based on the primary abnormality they address
- Iowa procedure. A, Approximation of the dilated intestine to the liver and abdominal wall with a row of sutures. The creation of seromyotomies is then followed by a second row of sutures to create a hepatomyoenteropexy. B, Division of the bowel into two hemiloops. C, The newly divided hemiloops. D, Reanastomosis in an isoperistaltic manner. (Reproduced with permission from Kimura K, Soper RT: A new bowel elongation technique for the short-bowel syndrome using the isolated bowel segment Iowa models. J Pediatr Surg 1993;28:792-794 The serial transverse enteroplasty. The dilated bowel is flattened, and the stapler is applied perpendicularly to the long axis of the bowel, from alternating sides. A small defect in the mesentery is created at each point of stapler application. The end result is a zigzag pattern to the lengthened bowel. (Reproduced with permission from Kim HB, Fauza D, Garza J: Serial transverse enteroplasty (STEP): A novel bowel lengthening procedure. J Pediatr Surg 2003;38:425-429.)
- This study identifed cholestasis, enterostomy, an absolute RSBL of &lt;30 cm, and a percentage of expected RSBL of &lt;10% as predictors of survival; and cholestasis, loss of the ICV, an absolute RSBL of &lt;20 cm, and a percentage of expected RSBL of &lt;10% as predictors of weaning off PN