? Duodenal atresia
? Annular pancreas
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? Jejunal and ileal atresia? Malrotation
? Necrotising enterocolitis
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? Meconium ileus
? Duplication cysts
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? Hirschsprung's disease? Anorectal malformation
? Colonic atresia
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Idiopathic hypertrophic pyloric stenosis? A disease of neonatal age group or early infancy.
? Incidence- 1 to 4 per 1000 live births.
? Age of presentation: 2weeks to 8weeks.(most commonly at 4weeks)
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? Boys : girls =4:1
? Usual y seen in first born male.
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? Exact cause is not known.? Seen in children more common who are formula fed than breast feeds.
? Exposure to erythromycin.
? Lack of local deficiency of nitric oxide synthetase.
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Gradual hypertrophy of circle
muscle of the pylorus
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Progressive obstruction of gastric
outlet
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Progressive increase infrequency of vomiting
Loss of gastric contents
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Hyponatraemic, hypokalemic,
metabolic alkalosis with
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paradoxical aciduria? Clinical presentation:
? Progressively worsening non bilious emesis
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? With time, the emesis becomes more frequent, forceful, and projectile in
nature.
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? Visible peristalsis from left to right.? Hungry and dehydrated baby
? Palpation of the pyloric tumor (olive-shaped) in the epigastrium or right upper
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quadrant just below the liver edge.
? If untreated, urine output decreases and acute renal injury due to
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hypovolaemia, hunger stools, hemetemesis Death? Diagnosis:
? USG: The diagnostic criteria for pyloric stenosis is a muscle thickness greater
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than or equal to 4 mm and a pyloric channel length greater than or equal to
16 mm.
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? Upper GI contrast study: String sign--- Content provided by FirstRanker.com ---
? Treatment:? Ramstedt's pylomyotomy: incising pyloric musculature without breaching the
mucosa
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? Both open and laparoscopic approach are available.
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Incision:
Right epigastric incision or
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periumbilical incision? Postoperatively, infants are usually allowed to resume enteral
feedings.
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? Vomiting after surgery occurs frequently but is generally self-limited.? Potential complications include
? incomplete myotomy
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? mucosal perforation
? wound infection
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Duodenal Atresia? The duodenum is one of the most common sites of neonatal
intestinal obstruction.
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? Incidence of duodenal atresia is 1 in 6,000 to 1 in 10,000 live births.
? Commonly associated with Down's syndrome and annular pancreas.
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Associated anomalies
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? In the sixth week, the gut epithelium proliferates rapidly, resulting inobliteration of the intestinal lumen.
? The intestine is then gradually recanalized over the next several
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weeks of development.
? Errors in recanalization are thought to be the primary cause of
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duodenal atresia and stenosis.? Types:
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? Type 1: Type 1 (92% of cases) : There is an obstructing septum(web) formed
49
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from mucosa and submucosa with no defect in the muscularis. Themesentery is intact.
? A variant of type 1 duodenal atresia, a "windsock deformity," can occur if the
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membrane is thin and elongated. The base of the membrane usually lies in
the second portion of the duodenum, but balloons out distally, distending the
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third and fourth portions.? Type 2 (1% of cases): A short fibrous cord connects the two blind ends of the
duodenum. The mesentery is intact
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? Type 3 (7% of cases): There is no connection between the two blind ends of
the duodenum. There is a V-shaped mesenteric defect.
? Most common site of block-near the junction of the first and second
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portions in 85% of cases.
? Site of block is distal to the opening of ampulla of vater.
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Clinical presentation? Antenatal: Polyhydramnios
? Antenatal USG: Double bubble
? Prematurity.
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? Newborn: recurrent bilious vomiting and intolerance to feeds.? Upper abdominal fullness. No gross abdominal distension.
? Treatment:
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? Duodeno-duodenostomy
? Site of abdominal incision : right transverse incision between right costal
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margin and umbilicus? Other bowel distal to obstruction must be checked after surgical correction to
look for any additional blocks or atresia.
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? Usually a transanastomotic nasojejunal tube is placed during surgery so that
feeding may be started early and NJ tube is removed once the aspirates
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become low.? Complications:
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? Leaks? Prolonged dysmotility of duodenum
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Annular pancreas
? Clinical Presentation: Similar to Duodenal atresia
? Management: Nothing is done to pancreas.
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? Two ends of duodenum are joined ? Duodeno-duodenoplasty
Jejuno-ileal atresias
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? Jejunoileal atresia is the most common GI atresia.? Incidence is 1 in 2000 live births.
? Result of an intrauterine mesenteric vascular occlusion.
? Atresias occur slightly more frequently in the jejunum than in the
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ileum.--- Content provided by FirstRanker.com ---
Types? Type I: a mucosal or membranous atresia with intact bowel wal
? Type I : an atretic cord between two blind ends of bowel with intact
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mesentery
? Type I Ia: a complete separation of the blind ends of the bowel by a V-
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shaped mesenteric gap? Type I Ib: an apple peel or Christmas tree deformity with a large mesenteric
gap in which the distal bowel receives a retrograde blood supply from the
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ileocolic or right colic artery.
? This tenuous blood supply has implications for reanastomosis and the potential for
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ischemic necrosis caused by an antenatal volvulus. Thus, many of these infants withthis type of atresia are born with reduced intestinal length.
? Type IV: there are multiple atresias, with a string of sausage appearance.
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Clinical Presentation
? Infants present with bilious emesis, abdominal distention, and failure
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to pass meconium during the early neonatal period.
? Overall clinical presentation is dependent on the level of obstruction.
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? In proximal atresia, abdominal distention is less, but significant biliousemesis is present.
? With distal atresias, abdominal distention is present more frequently.
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Diagnosis
Management
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? Initial resuscitation with IV fluids, IV antibiotics and proximal
segment decompression with placement of the NG tube and
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aspiration? Aim: to establish intestinal continuity and save as much bowel as
possible.
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? Early surgery- delayed surgery causes dysfunction of the proximal
segment due to pressure ischemia.
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? End to end or end to side jejunojejunal/ jejuno-ileal/ ileo-ilealanastomosis
? Peri-operative TPN
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? Problems:? Risk of short bowel syndrome due to loss of significant length of bowel
? Bowel lengthening procedures may be needed later in life.
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? Functional loss of bowel- Poor absorption/ poor peristalsis
? Post-op anastomotic leaks and stenosis
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? Overall survival is 80-90%.Intestinal malrotation
? The incidence of malrotation has been estimated at 1 in 6000 live births.
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? It occurs due to abnormal rotation and fixation of the bowel to theretroperitoneum leading to precariously narrow based mesentery.
? Presence of abnormal mesenteric bands (Ladd's bands)-extend from the
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colon across the anterior duodenum- Predisposing bowel for volvulus and
intestinal obstruction.
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? Up to 75% of patients present during the first month of life, while another15% wil present within the first year
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EmbryologyNormal
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intestinalanatomy
results in fixation of the
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duodenojejunal junction in the
left upper quadrant and the
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cecum in the right lowerquadrant. This allows a wide
breadth to the mesentery of the
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small bowel.
Clinical presentation
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? Recurrent bilious vomiting
? Poor growth and failure to thrive
? Malabsorption
? Chylous ascites
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? Associated volvulus? Recurrent pain abdomen
? Gangrene of the bowel and perforation
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? Peritonitis and shock
? Hemetemesis
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Diagnosis
? Upper GI contrast study:
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? demonstrates an abnormal position of the ligament of Treitz.? Cork screw pattern in case of associated midgut volvulus
? USG:
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? Dilated duodenum
? Alteration in the relation of superior mesenteric vessels
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? Whirl pool sign on colour doppler in case of volvulusManagement
? Aggressive resuscitation especially when midgut volvulus is suspected.
? Emergency laparotomy.
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? Laparoscopic approach is also done in many centres.? Name of the surgery: Ladd's procedure/operation.
? Entry into the abdomen through right supraumbilical incision.
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? Delivery of the bowel.? Derotation of the bowel.
? Excision of the ladd's band.
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? Straightening of the duodenum
? Broadening of the smal intestinal mesentery
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? Appendicectomy? Placement of the smal bowel along the right lateral gutter and large bowel
along the left lateral gutter.
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? Closure of abdomen.
? If gangrenous bowel is present, excision of the gangrenous portion is
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done primarily or after second look re-exploration.
? Aims is to save as much bowel as possible to avoid development of
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short bowel syndrome.? Post-operative bowel function resumes in 3-5days when oral feeds
are started.
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? Complications: Adhesive obstruction later which may require a
surgical management.
Necrotising enterocolitis
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? NEC is the most common GI emergency in the neonatal period.
? Usually seen in premature and low birth weight babies.
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? Usually develops after the starting of feed.? Mortality rate is high.(10-50%)
? Most common cause of short bowel syndrome in children.
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Pathology
? After feeds if excess glucose comes to ileum and colon due to poor
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absorption proximally in the intestines or high glucose content of thefeeds
? Bacterial overgrowth
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? Local acid production by bacteria? Poor gut development of premature baby
? Inflammation and necrosis of bowel due to translocation of bacteria
into the bowel wall and mesentery
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? Bowel gangrene, perforations and severe sepsis
Clinical presentation
? Acute abdominal distention, tenderness, and feeding intolerance with gross or occult
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blood in the stool are hal mark features for NEC.
? Other nonspecific clinical signs include irritability, temperature instability, and episodes
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of apnea or bradycardia.? NEC typical y occurs in the first few days of life with the initiation of enteral feedings.
? In approximately 80% of cases, it occurs within the first month of life.
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? As NEC progresses, systemic sepsis develops, with hemodynamic deterioration and
coagulopathy.
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? The distal ileum and ascending colon are the usual affected areas.? The entire GI tract (NEC totalis) may also be involved.
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Diagnosis? The pathognomonic radiographic feature of NEC is pneumatosis
intestinalis.
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? Pneumatosis is composed of hydrogen gas generated by the bacterial
fermentation of luminal substrates.
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? Other radiographic findings may include portal venous gas, ascites,fixed loops of small bowel, and free air.
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Medical Management
? Initial medical management consists of
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? nasogastric tube decompression? fluid resuscitation
? blood and platelet transfusion
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? administration of broad-spectrum antibiotics.
? In general, NEC can be successfully treated medically in approximately
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50% of cases with a 7- to 10-day course of antibiotics.? The absolute indication for operative management of NEC is the
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presence of intestinal perforation, as revealed by free air on plainabdominal radiographs.
? Other relative indications for surgery include
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? overall clinical deterioration
? abdominal wall cellulitis
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? worsening acidosis? falling white blood cell or platelet count
? a palpable abdominal mass
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? persistent fixed radiographic bowel loop
Surgical management
? The general principles of surgical management of NEC include
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resection of all nonviable segments of intestine with the creation of a
stoma.
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? All efforts need to be made to preserve as much intestinal length aspossible.
? It may be necessary to resect multiple intervening necrotic segments of
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bowel, preserving all viable intestine.
? In cases in which the bowel is ischemic but not frankly necrotic, a second-look
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operation may be performed after 24 hours.? Once the child is stable and started on feeds with increase in the
weight, further surgery may be done to maintain the instestinal
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continuity.
To be continued...
? Meconium ileus
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? Hirschsprung's disease? Anorectal malformation
? Duplication cysts
? Colonic atresia
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MECONIUM ILEUSINTRODUCTION
? Meconium ileus is a subset of CF.
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? It represents a disorder of neonates characterized by intestinalobstruction secondary to the intraluminal accumulation of inspissated
and desiccated meconium.
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? Earliest clinical manifestation of CF.
history
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? First described by Landsteiner(1905)-? Meconium obstructing the small bowel + pathologic changes in pancreas +
pancreatic enzymes deficiency.
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? Term "cystic fibrosis" of the pancreas by Fanconi(1936)
? Association of chronic pulmonary disease of infancy + pancreatic insufficiency
? CF is now recognized as the most common potentially lethal
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autosomal recessive disease in the Caucasian population.
INCIDENCE
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? Incidence of 1 per 1000 to 2500 live births.? Rare in blacks (1/17,000 live births).
? Very rare in in Asians (1/90,000 live births) and Africans.
? Meconium ileus is presenting feature of 20.8% of the CF population in
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the United States.
GENETICS
? Locus for CF- 7q31.
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? codes for the cell membrane protein termed the CF transmembrane
regulator(CFTR).
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? cyclic adenosine monophosphate?induced chloride channel that regulates ionflow across the apical surface of epithelial cells.
? More than 400 mutations known for CFTR.
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Abnormal electrolyte content along the external apical
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environment of epithelial membranesTubular structures lined by affected epithelia
Desiccation and reduced clearance of their secretions
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? Respiratory- recurrent pneumonia? Gastrointestinal- meconium ileus (10% to 20%)
? Biliary- obstructive biliary disease (15% to 20%)
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? Pancreatic- pancreatic insufficiency(90%)
? Reproductive systems- azoospermia(nearly 100%)
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? The F508 mutation- most common CFTR gene
locations.
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? Homozygous individuals nearly always express
pancreatic exocrine insufficiency
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? also present with a higher incidence of meconium ileus.PATHOGENESIS
? Exocrine-eccrine gland dysfunction
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? mucus-secreting and sweat glands.? Intestinal mucosal gland abnormalities caused production of
tenacious material that causes the intraluminal obstruction.
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? Intestinal glandular disease -dominant role
? Pancreatic disease -secondary role
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? Defect in electrolyte resorption of sodium and water
by ductular cells
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? Impermeability of these cells to chloride ionsExcretory product high in both sodium and chloride.
desiccation and reduced clearance of such secretions
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? Analysis of the protein content of abnormal
meconium
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? Most of this protein is albumin.
? In the absence of degrading enzymes, they impart a highly
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viscid rubbery character to the involved secretions.? Thickened meconium accumulates in utero
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obstruct the intestine lumen
complications of meconium ileus
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(i.e., twist of a heavy loop with perforation, peritonitis,and cyst or atresia) and the pattern of abdominal
distention and obstruction seen in the neonate.
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? The proximal ileum dilates, and its wall thickens as it
becomes filled with the tenacious and tarry
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meconium.
? The narrowed distal small bowel and the colon
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contains beaded or "boxcar" concretions of gray-white, putty-like inspissated meconium.
? The more distal colon is small or unused, a microcolon.
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clinical Features
? Presents in the neonate as:
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? uncomplicated or simple? Complicated
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? Uncomplicated meconium ileus? typically presents immediately at birth with abdominal
distention.
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? In utero genetic diagnosis of CF and an ultrasound
suggestive of intestinal obstruction with echogenic bowel.
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? Bilious vomiting and failure to stool.? Complicated meconium ileus
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? will present either in utero or postnatally with evidence of
bowel obstruction complicated by evidence of previous
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intestinal perforation and/or necrosis? Crescents or speckles of intra-abdominal calcification may be
present
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? On clinical assessment there may be evidence of peritonitis
including an erythematous or edematous abdominal wall
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? +/- demonstrable abdominal tenderness.Meconium peritonitis
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? Meconium pseudocyst:? meconium accumulating in the peritoneal cavity for weeks to months.
? Calcified pseudocyst
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? fibrous wall forms around an accumulation of meconium
? spared bowel loops are peripheral to this cyst
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? Adhesive meconium peritonitis:
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? Meconium contamination of the peritoneal cavity? Dense and vascular adhesions
? Scattered calcifications
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? Meconium ascitis:
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? When intestinal perforation occurs only a few days before
delivery
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? an abdomen filled with meconium? calcification is absent
? Bacterially infected ascites
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? colonized intestinal organisms penetrate from the
perforated intestine into the peritoneal cavity.
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CLINICAL HISTORY? A family history of CF is present in 10% to 33%.
? This history + in utero amniocentesis permits the
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accurate diagnosis of the fetus afflicted with CF.
? Above + the results of serial in utero sonography
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permits the accurate predication of which infants areat risk for the development of the intestinal
obstruction of meconium ileus.
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? Maternal polyhydramnios may be a feature of in
utero meconium ileus due to high-grade intestinal
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obstruction.
? In utero growth retardation is common, prematurity
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and other associated anomalies are rare.PHYSICAL EXAMINATION
? Abdominal distention
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? Visible peristaltic waves and palpable, doughy bowel loops are often
present.
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? Finger pressure over a firm loop of bowel may hold the indentation-putty sign.
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? In the presence of an in-utero perforation withmeconium peritonitis and "cyst" formation
? a palpable abdominal mass, discoloration of the abdominal
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wall, and signs of peritoneal irritation are often observed.
? Physical evidence of hypovolemia may rapidly develop in
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infants with peritonitis.? On passage of a nasogastric tube, a quantity of bile-stained
gastric fluid usually exceeds 20 mL.
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RADIOLOGIC STUDY
? In utero, meconium ileus bowel may be distended but an echogenic
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bowel wall in the third trimester may be diagnostic as well.--- Content provided by FirstRanker.com ---
? Uncomplicated meconium ileus is? supine and erect films appearing remarkably similar
1. great disparity in the size of the intestinal loops
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2. no or few air-fluid levels on the erect film because
swallowed air cannot layer above the thickened
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inspissated meconium3. a granular, "soap bubble," or "ground-glass"
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? A contrast enema will outline a normally positioned
unused colon
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? It will be empty or will contain pellets of inspissated
meconium.
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? The colon will be the typical "unused" colon or"microcolon."
? If reflux of contrast agent into the terminal ileum occurs, it
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will outline pellets of inspissated meconium.
Laboratory testing
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? Sweat test:
? The minimum amount of sweat to be collected is 100 mg, and a measured
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concentration of sweat chloride in excess of 60 mEq/L is diagnostic of CF.? Genetic testing for CF:
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? can be done by analyzing cellular DNA for CFTR,? If a family has known CFTR mutations, then amniocentesis
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with fetal DNA analysis may predict a fetal CF diagnosis.? Increased albumin concentration in meconium may be
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useful as a diagnostic screening tool for meconium ileus.
? Meconium from normal neonates has an albumin
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concentration of less than 5 mg/g of stool, whereasmeconium from neonates with CF has values at times in
excess of 80 mg/g.
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? Operative tissue specimens that aid in the diagnosis
of CF include intestinal (rectal) or appendiceal
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pathognomonic changes
? goblet cell hyperplasia
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? Accumulation of secretions within the crypts or within thelumen.
nonoperative management
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? Constitutes dissolution of the inspissated intraluminal meconium in
an otherwise patent and uncompromised ileocolon.
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Prerequisites:
? intravenous antibiotics should be administered
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? the patient should have a full fluid resuscitation with fluidsgiven aggressively (one to three times maintenance) during
the procedure
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? exclude other causes of distal intestinal obstruction
? the complications of volvulus, atresia, perforation, or
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peritonitis must be excluded? The patient should be prepared for imminent operation
should complications develop.
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? A Gastrografin enema has been the standard of
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nonoperative treatment.? Meglumine diatrizoate is a hyperosmolar, water-soluble,
radiopaque solution containing 0.1% polysorbate 80
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? 40%sodium diatrizoate
? 5 mL of a 10% N-acetylcysteine solution should be administered every
6 hours through a nasogastric tube to liquefy upper gastrointestinal
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secretions.
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? After successful conservative management:? supplemental pancreatic enzymes must be administered
with each feeding when feeding is started.
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? If the evacuation of meconium is incomplete and
obstruction persists, the enema may be repeated.
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? If either no evacuation of meconium, then this
technique should be abandoned and operative
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intervention planned.? Similarly, signs of worsening obstruction, clinical
distention, greater distention of loops on radiograph,
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or signs of peritonitis resulting from a possible
perforation are also indications for operative
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intervention.Operative management
? Indications:
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? Simple:(1/3rd to ?)
? Failed to respond to conservative management
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? Complicated(2/3rd to ?)? Intestinal atresia
? Volvulus
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? Perforation
? Meconium cyst formation with peritonitis
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? Intestinal gangrene, or combinations of these events.SIMPLE MECONIUM ILEUS
? Goal of operation is the relief of intraluminal ileocolonic obstruction
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? evacuation of the adherent intraluminal meconium? resection of the portion of bowel filled with inspissated material.
? Enterotomies with irrigation coupled with a limited resection.
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? Irrigating solutions:
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? warmed saline? 50% diatrizoate solution
? 1% solution of pancreatic enzymes
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? hydrogen peroxide
? Most commonly, either a 2% or a 4% solution of N-
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acetylcysteine--- Content provided by FirstRanker.com ---
? Three options:? The enterotomy and the abdomen may then be closed
? enterostomy may be created
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? the site can be controlled by insertion of a T tube.
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? Appendectomy with appendicostomy, with meconium
evacuation or irrigation through this route.
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? Relieving stoma with or without an associated partial
resection.
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? Mikulicz double-barreled enterostomy
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? Primary resection and anastomosis
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? Distal chimney enterostomy (Bishop-Koop procedure):--- Content provided by FirstRanker.com ---
? Santulli proximal chimney enterostomy
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COMPLICATED MECONIUM ILEUS
? Operative indications include:
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? Persisting intestinal obstruction
? Enlarging abdominal mass
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? Signs of peritonitis.Rehbein's two-stage operation
? After initial resuscitation, a proximal dilated loop stoma is created to
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the obstructive site.? Once the general condition of baby improves, further laparotomy and
resection and anastomosis of distal segment is done.
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? Meconium cyst-
? Decortication of the cyst.
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? Ultrasound guided drainage.Postoperative Management
? Instillation of 2% or 4% acetylcysteine (Mucomyst) delivered through
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a nasogastric tube, which will solubilize the residual meconium.? When enteral feedings begin, supplemental pancreatic enzymes must
also be started.
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? If postoperative ileus is prolonged or if short-bowel syndrome total
parenteral nutrition.
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