Indications for Gastrostomy Feeding and Oesophageal Dilatation in Children with Severe EB

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Indications for Gastrostomy Feeding and
Oesophageal Dilatation in Children with Severe EB

• Jacqueline Denyer Senior EB CNS
• (Paediatric)
• Great Ormond Street Hospital, London
• And DebRA UK

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Gastrostomy

• Preferred device button gastrostomy avoids
  introduction via friable oesophagus.
• Methods of insertion
• Open procedure via laparotomy
• Laparoscopic
• Endoscopic (Interventional Radiologist)

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Button Gastrostomy

• Low profile device.
• Held in place by balloon reservoir of water for
  ease of change

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Indications for Gastrostomy Feeding

• Failure to thrive
• Dysphagia
• Aspiration
• Constipation
• Large volume of medication
• Stress

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Failure to thrive

• Reduced oral intake microstomia, oral
  ulceration, dental overcrowding and decay.
• Oesphageal stricture(s)
• Increased nutrient requirement, competition
  between wound healing and growth
• Reduced appetite from constipation

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Alternative Route for Medications

• Child unable/unwilling to swallow medicines

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Anxiety

• Parents aware of increased need for nutrients
• Prolonged meal times
• Intake greatly reduced at times of acute
  oesophageal obstruction, risk of dehydration

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Number of Children with Gastrostomy by Type of
EB G.O.S.H. (1989 -2005)

• Recessive dystrophic 58
• Herlitz junctional 1
• Non- Herlitz junctional 2
• Recessive simplex - 1

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Number of gastrostomies inserted

• 1989 2001
• Average 6.3 children per year
• 2002 - 2005
• Average 1 child per year
• Introduction of oesophageal dilatation (2001)

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Advantages of gastrostomy feeding

• Greatly reduced anxieties re feeding
• Healthier, happier child
• Reduction in constipation
• Alternative route for medications

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Disadvantages of Gastrostomy Feeding

• Leakage/soreness around site
• Reduced oral intake
• Increased G.O.R
• Childs dislike of button change
• Not always accepted by older children
• Incontinence with over- night feeding
• Fat deposited centrally, children remain thin
  limbed and of short stature

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90 of parents said they would recommend
gastrostomy placement to another family
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Oesophageal Dilatation

• Indication
• Acute obstruction
• Dysphagia
• Food getting stuck
• Inability to swallow saliva
• Odour from pooled food/saliva above stricture

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Balloon Dilatation (G.O.S.H.)

• Interventional Radiologist
• general anaesthesia
• - endotracheal intubation
• - muscle relaxant
• fluoroscopic guidance
• - endoscopy
• - water-soluble contrast oesophagogram
• - pulsed fluoroscopy

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Balloon dilatation of the oesophagus

• Pathophysiology of strictures may be
  multifactorial
• Location is typical
• - cervical
• - mid thoracic
• Severity is variable

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Balloon dilatation

• 1.7-mm (5-Fr) BMC catheter
• floppy-tip hydrophilic guidewire
• 0.89-mm (0.035-in)

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Standard techniqueM/11 y. Dystrophic
epidermolysis bullosa
catheter
guidewire
8 mm balloon
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Patients and procedures 1999-2003

• 31 patients
• median age at first dilatation 7 years
• range 22 months to 16 years
• 118 procedures
• range 1 to 9 per patient
• 0/118 perforations
• median follow-up 33 months

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Interval between dilatationsPatients with 4 or
more procedures (n 13)

• interval between last two procedures
• range 15 days (patient 10) to 3.4 years
• median 10 months
• 11/13 (85) more than 7 months
• interval since last procedure
• median 7 months
• patient 10 gt10 months

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Parents audited comments about oesophageal
dilatation

• Child feels better in himself, confidence is
  boosted
• Takes away pain for the whole family
• Mealtimes once again fun for the whole family
• Change in eating pattern is unbelievable
• its lovely to see her enjoying her food
• Childs social life has improved greatly
• Fewer foods get stuck in oesophagus and
• cause distress
• The risk of perforation is worrying

(Haynes 2005)
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With thanks to.

• Lesley Haynes, EB Dietitian, G.O.S.H.
• and
• Derek Roebuck Interventional Radiologist,
  G.O.S.H. UK
• for their help in preparing this presentation and
  provision of some slides