Rosen & Barkin's 5-Minute Emergency Medicine Consult (348 page)

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See Also (Topic, Algorithm, Electronic Media Element)

• Femur Fracture
  
• Pelvic Fracture
  

CODES

• 820.8 Closed fracture of unspecified part of neck of femur
  
• 835.00 Closed dislocation of hip, unspecified site
  
• 959.6 Hip and thigh injury
  

BASICS

• Described in 1886 by Harold Hirschsprung as a cause of constipation in early infancy
  
• Congenital aganglionosis megacolon
  
• 1:5,000 live births
  
• Overall mortality of Hirschsprung enterocolitis is 35–50%
  

• Absence of enteric ganglia in the distal bowel
  
• Normally, ganglia are derived from neural crest and migrate along the intestine, arriving at the proximal colon by 8 wk of gestation and in the rectum by 12 wk of gestation.
  
• Failure of neural crest cells to migrate into parasympathetic Meissner (submucosal) and Auerbach (myeneteric) ganglions results in an aganglionic segment and clinical disease
  
• Affected bowel classically presents at the internal anal sphincter and involves the rectosigmoid colon (75% of cases)
  
• May extend the entire length of the GI tract (often fatal)
  
• Aganglionic segment chronically contracts, forming an obstruction to the passage of stool, and proximal bowel distends to hold the stool that has not passed
  
• Stimulation of the anus allows passage of stool
  
• Genetic and other causes
  
  • Mutations of the
    ret
    proto-oncogene found in both familial and spontaneous forms
    
  • Male-to-female ratio 4:1
    
    • 8% with positive family history; 5–12% of siblings are affected
      
    • Associated chromosomal abnormality in 5–15%, most commonly trisomy 21 (Down syndrome)
      
    • Other congenital anomalies in 18%—GI, cardiac, craniofacial, cleft palate, congenital deafness
      

DIAGNOSIS

Children with Hirschsprung disease are usually diagnosed by the age of 2 yr

• Three presentations, varying with age:
  
  • Neonatal
    
    • Abdominal distension
      
    • Failure of passage of meconium within the 1st 48 hr of life
      
    • Vomiting
      
    • Enterocolitis (abdominal pain, fever, foul-smelling and/or bloody diarrhea, vomiting, leading to sepsis and potential intestinal perforation)
      
    • Sepsis
      
  • Infancy
    
    • Constipation
      
    • Chronic abdominal distension
      
    • Vomiting
      
    • Failure to thrive
      
    • Enterocolitis (abdominal pain, fever, foul-smelling and/or bloody diarrhea, vomiting, leading to sepsis and potential intestinal perforation)
      
    • Toxic megacolon
      
  • Later childhood and adulthood
    
    • Chronic constipation with obstruction and rare history of overflow incontinence and often refractory to normal treatment protocols
      
    • Enterocolitis (abdominal pain, fever, foul-smelling and/or bloody diarrhea, vomiting, leading to sepsis and potential intestinal perforation)
      
    • Abdominal distension
      
    • Bloody, foul-smelling diarrhea
      
    • Malnutrition
      

• Sepsis
  
• Bowel movements frequently require rectal stimulation or enemas
  
• Narrow caliber stools
  
• Encopresis and diarrhea are uncommon
  
• Absence of inciting factors associated with functional constipation (i.e., fissures, toilet training, diet)
  

Possible palpable colon on the left

• Abdominal distension and symptoms of intestinal obstruction
  
• Findings consistent with malnutrition, failure to thrive
  
• Rectal exam commonly reveals an empty rectal vault
  

Plain abdominal radiographs:

• Distended small bowel and proximal colon (megacolon) with an empty rectum are common findings
  
• Transition zone into a narrowed rectosigmoid segment
  
• In neonates, films will commonly show a distal obstructive pattern
  
• In children with chronic constipation, films may show only large amounts of stool
  
• In children with enterocolitis, bowel wall edema or pneumatosis intestinalis may be present
  

• CBC, electrolytes, glucose, BUN, Cr
  
• Urinalysis
  
• Blood culture, if toxic
  

Barium enema:

• Obtain after stabilization; contraindicated if perforation and enterocolitis are suspected
  
• Dilated colon proximal to the contracted aganglionic colon with uncoordinated peristalsis, and transition zone between the narrowed aganglionic segment and the dilated and normally innervated segment
  
  • A transition zone may not be apparent in neonates, because of insufficient time to develop colonic dilatation, or in infants who have undergone rectal exams and enemas
    
  • May demonstrate a nondistensible rectum, which is a classic sign of Hirschsprung disease
    
• Delayed barium evacuation
  

• Rectal manometry may assist in diagnosis but is often abnormal in long-standing constipation; children with Hirschsprung disease fail to demonstrate reflex relaxation of the internal anal sphincter in response to inflation of a rectal balloon
  
• Full-thickness rectal biopsy confirms diagnosis by the lack of ganglion cells
  
  • The definite diagnosis of Hirschsprung disease rests on histologic review of rectal tissue
    

• Neonates
  
  • Meconium ileus or meconium plug syndrome from cystic fibrosis
    
  • Intestinal or anal atresia or hypoplasia
    
  • Malrotation or duplication with volvulus
    
  • Necrotizing enterocolitis
    
  • Sepsis
    
• Infants and children
  
  • Functional constipation
    
  • Toxic—opiates, anticholinergics
    
• Infectious—botulism
  
• Acquired aganglionic colon
  
  • Metabolic or endocrine—hypothyroid/parathyroid, adrenal insufficiency, electrolyte abnormality
    
    • Spinal cord defects
      
• Abdominal masses
  

TREATMENT

• Airway, breathing, and circulation management with monitoring as appropriate
  
• Initial bolus of isotonic IV fluids (20 cc/kg) for shock, dehydration, sepsis
  

• Infants should be managed for bowel obstruction; in the presence of vomiting, an NG tube may be necessary
  
• Consultation with a pediatric surgeon
  
  • Triple antibiotics are indicated in toxic patients or those with enterocolitis
    
• Unstable patients may require decompression by emergent loop colostomy
  
  • Stable children may have their workup done as an outpatient
    
  • Definitive treatment is resection of the aganglionic section of bowel to achieve normal ganglion containing bowel within 1 cm of anal opening
    
• Enterocolitis may occur at any time, even after operative intervention