neonatal intestinal obstruction•“a neonate with bilious vomiting or aspirate is considered to...
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Neonatal intestinal obstruction
HASHEM AL-MOMANISENIOR CONSULTANT PEDIATRIC
SURGEONJORDAN UNIVERSITY HOSPITAL
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Introduction
• Neonatal intestinal obstruction is one of the common pediatric emergencies.
• Incidence : 1 in 2000 live births.
• A wide range of congenital anomalies may result in neonatal bowel obstruction.
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Causes of intestinal obstruction in the neonatal period
• Congenital atresia and stenosis constitute the majority of cases.
• Other causes include
– Malrotation
– Volvulus
– Meconium ileus
– Hirschsprung disease
– Anorectal malformations
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Causes of intestinal obstruction in the neonatal period
• Gastric – Early pyloric stenosis– Pyloric web or atresia– Epidermolysis bullosa pyloric atresia
syndrome
• Duodenum – Stenosis– Atresia– Malrotation– Annular pancreas
• Jejunum-Ileum – Stenosis– Atresia – Malrotation– Meconium ileus– Vitello-intestinal duct remnant– Intussusception– Milk curd obstruction
• Colonic – Stenosis– Atresia– Imperforate anus– Poorly developed colon e.g.– megacystis microcolon intestinal– hypoperistalsis syndrome
• Global – Duplication anomalies– Internal hernia or inguinal hernia– Volvulus with or without (e.g. about a– Meckel’s band) malrotation– Neoplasm
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Etiology113 etiologies in 106 patients
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Presentation
• “A neonate with bilious vomiting or aspirate is considered to have intestinal obstruction until proved otherwise.”
• The presenting symptoms could be any combination of the following:– Bilious vomiting
– Abdominal distension
– Delayed passage of meconium
– Sepsis
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Bilious vomiting
• Bilious vomiting is synonymous with intestinal obstruction, be it functional or mechanical.
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Examination
• dehydration
• abdominal distension
• Visible and palpable bowel loops
• Erythema and tenderness of abdominal wall
• The presence of a normal anus
• Associated anomaly
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A newborn withmarked abdominal distension
• suggesting
– distal obstruction
– necrotizing enterocolitis
– sepsis
• The more marked the abdominal distension, the more distal is the obstruction
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Perineal Examination
• Absent anus
• Rectal stimulation
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Vomiting
• Vomiting :
– Non-bilious vomiting: Colorless or milky if a feed has been taken.
– Bilious vomit:
• Neonatal bilious vomiting should be considered to be a surgical emergency until proved otherwise.
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Constipation
• A term neonate should pass meconium within 24 h of life.
• Delayed passage of meconium: beyond 48 hours
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Passage of meconium
• is absent in complete duodenal and small intestinal obstruction or in anorectal malformations
• meconium passage may be:
– present in malrotation
– delayed in Hirschsprung’s disease
– Occurs by an abnormal route (by a fistula )
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Abdominal Radiology
• The simplest and most informative radiological procedure is the plain abdominal X-ray.
• confirmation of bowel obstruction with some information about the level of the obstruction.
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Imaging Studies
• Plain x-ray abdomen:
– supine film
– lateral decubitus
– Invertogram or prone cross-table lateral film for anorectal malformations
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Plain abdominal X-ray
• The extent and position of bowel gas
• Presence or absence of gas in the rectum
• Degree and level of distended loops
• Air fluid levels
• Evidence of free gas would confirm perforation.– “Football sign”,
– The rigler sign, also known as the double wall sign
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Intestinal air progression
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Abdominal x-ray showing dilatation of bowel loops with air–fluid level
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single air bubble
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Plain abdominalx-ray
• Dilated stomach with air distally suggesting partial duodenalobstruction
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Complete duodenalobstruction
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Triple bubbles
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Pneumo-peritoneum
• Free air due to perforation from any cause is suspected on supine film when
– “football sign” : a large pocket of air overlying liver and the ligamentum teres
– Rigler sign: the bowel wall is sharply delineated (pencil lining).
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Football sign
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Rigler sign
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Lateral decubitus
• Lateral decubitus film with the right side uppermost should be used to see air above the liver.
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Free air above the liver
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Ileal atresia with volvulus
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Calcification
• Calcification of meconium implies long-standing stasis and may be identifiable outside of the bowel loops, which would suggest previous perforation.
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Diffuse calcifications
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Contrast Studies
• The first enema a neonate receives should be a contrast enema.
• The contrast enema acts not just a diagnostic tool but works as a therapeutic measure in cases of:
– meconium plug
– meconium ileus
– Hirschsprung’s disease
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Lower contrast study showing small left colon syndrome
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Lower contrast study showing Hirschsprung’s disease
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Lower contrast study showing meconium plug syndrome
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A lower contrast study:small unused colon suggesting small bowel obstruction or total
colonic hirschprung’s disease.
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Contrast upper gastrointestinal (GI) studies
• If malrotation is suspected
• Sometimes to characterize the duodenal obstruction
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Malrotation
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Malrotation
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Volvulus neonatorum- spiral twist of the bowel
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Upper contraststudy showing congenital
duodenal obstruction.
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HIRSCHSPRUNG DISEASE
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definitions
• Congenital megacolon
• HD is characterized by the absence of myenteric and submucosal ganglion cells in the distal alimentary tract; resulting in decreased motility in the affected bowel segment
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Pathophysiology
• Hirschsprung disease results from the absence of parasympathetic ganglion cells in the myenteric and submucosal plexus of the rectum and/or colon.
• Ganglion cells, which are derived from the neural crest, migrate caudally with the vagal nerve fibers along the intestine.
• These ganglion cells arrive in the proximal colon by 8 weeks of gestational age and in the rectum by 12 weeks of gestational age.
• Arrest in migration leads to an aganglionic segment.
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transitional zone
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Frequency
• Hirschsprung disease occurs in approximately 1 per 5000 live births.
• Sex: 4 times more common in males than females. • Age:
– Nearly all children with Hirschsprung disease are diagnosed during the first 2 years of life.
– one half are diagnosed before they are aged 1 year. – Minority not recognized until later in childhood or adulthood.
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HD can be classified by the extension of the aganglionosis as follows:
• Classical HD (75% of cases): Rectosegmoid
• Long segment HD (20% of cases)
• Total colonic aganglionosis (3-12% of cases)
• rare variants include the following: – Total intestinal aganglionosis
– Ultra-short-segment HD (involving the distal rectum below the pelvic floor and the anus)
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Clinical presentation:
• Newborns : – Failure to pass meconium within the first 48 hours of life
– Abdominal distension that is relieved by rectal stimulation or enemas
– Vomiting
– Neonatal enterocolitis
• Symptoms in older children and adults include the following: – Severe constipation
– Abdominal distension
– Bilious vomiting
– Failure to thrive
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Differential Dx
• Intestinal atresias or stenosis
• Small left colon syndrome
• Meconium plug syndrome
• Intestinal malrotation
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diagnostic workup
• Plain abdominal radiography
• Contrast enema
• Biopsy
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Abdomenal X-Ray
• Dilated bowel
• Air-fluid levels.
• Empty rectum
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AXR
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AXR
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barium enema
• Transition zone
• Abnormal, irregular contractions of aganglionic segment
• Delayed evacuation of barium
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Ba-enema
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Ba-enema - TZ
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Ba-enema- delayed emptying
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Biopsy
• Types:– rectal suction biopsy – full-thickness rectal biopsy.
• In HD, the biopsy reveals:– absence of ganglion cells– hypertrophy and hyperplasia of nerve fibers, – increase in acetylcholinesterase-positive nerve fibers in the
lamina propria and muscularis mucosa.
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treatment
• The treatment is surgical removal or bypass of the aganglionic bowel,
• This can be performed by means of:– preliminary colostomy followed by a definitive pull-through
procedure or,
– primary definitive procedure.
• Examples include:– Soave pull-through procedure,
– Duhamel procedure,
– Swenson procedure.
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The three most commonly performed operations A, Soave. B, Swenson. C, Duhamel
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Enterocolitis
• Enterocolitis accounts for significant morbidity and mortality in patients with Hirschsprung disease.– Patients typically present with explosive diarrhea, abdominal
distention, fever, vomiting, and lethargy.
– Approximately 10-30% of patients with Hirschsprung disease develop enterocolitis. Long-segment disease is associated with an increased incidence of enterocolitis.
– Treatment consists of rehydration, intravenous antibiotics and colonic irrigations.
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Post operative complications
• anastomotic leak
• anastomotic stricture
• intestinal obstruction
• pelvic abscess
• wound infection
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Prognosis
• The long-term outcome is difficult to determine because of conflicting reports in the literature.
• Some investigators report a high degree of satisfaction, while others report a significant incidence of constipation and incontinence.
• approximately 1% of patients with Hirschsprung disease require a permanent colostomy to correct incontinence.
• patients with associated trisomy 21 have poorer clinical outcomes.
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THANK YOU