Table 4.

Treatment of Manifestations in Individuals with BOS

Manifestation/ConcernTreatmentConsiderations/Other
Poor growth & feeding due to chronic severe emesis 1
  • For cyclic vomiting: identification & avoidance of triggers, 2 daily maintenance medication, 3 & early abortive treatment at episode onset 4
  • G-tubes or GJ-tubes often decrease aspiration & improve nutrition; 5 consider thickened feeds.
Fundoplication & traditional antireflux management w/acid blockers are typically not beneficial if gastroesophageal reflux is not the underlying etiology.
Cleft lip &/or palate
  • Primary closure of cleft lip along standard timeline
  • Consider leaving cleft palates unrepaired when speech is lacking.
  • Palate repair in a child at risk for obstructive apnea may ↑ risk.
  • Assess language & mobility to determine plan for palate closure & alveolar bone grafting.
  • Palate repair may be warranted in individuals w/language skills.
Frequent infections &/or aspiration pneumonia 4 Aggressive management of chronic emesis
Fever or increase in emesis
  • Urinalysis & urine culture for possible urinary tract infection
  • Evaluation for other possible sources of infection, pain, or exposure
Seizures Standard antiepileptic medicationsMost individuals respond to monotherapy.
Congenital heart defects Standard management
Respiratory
symptoms 		Tracheostomy may be effective in:
  • Creating a safe airway
  • Treating aspiration → lung disease
  • Treating severe obstructive sleep apnea not controlled by noninvasive pressure support (e.g., CPAP, BiPAP) or surgical management (e.g., adenoidectomy, mandibular distraction)
Aspiration may also be amenable to treatment w/postpyloric feedings (e.g., by gastrojejunostomy).
  • Inhaled albuterol & inhaled steroids have improved respiratory status in some, although typical findings for reactive airway disease were lacking [Russell et al 2015].
  • Descending aspiration of saliva can often be managed w/glycopyrrolate or salivary Botox injections.
Sleep disturbances Melatonin, treatment of anemia
Myopia Corrective lenses, often first prescribed in infancy
Urinary retention, urinary tract infections, renal stones Standard treatmentsAppropriate management of these conditions can improve emesis & hospitalization rate.
1.

If vomiting is well controlled, growth typically improves, hospitalizations for dehydration and aspiration decrease, and overall health and well-being improve, although the rate of linear growth and weight gain remains poor. Lifelong feeding interventions may not be required, so periodic reexamination is appropriate.

2.

Triggers such as vaccines, infections, and anesthesia have been reported, although this is not a reason to avoid vaccination and anesthesia: all children with BOS should receive the full course of standard vaccinations as recommended by the local authorities.

3.

Cyproheptadine has been used for daily maintenance therapy.

4.

Prophylactic treatment prior to a trigger exposure with antiemetics has been beneficial. Abortive treatment includes lorazepam, ondansetron, and acetaminophen or some combination of an antiemetic, pain reliever, and sedative.

5.

Most affected individuals with feeding difficulties require a permanent feeding tube (G-tube or GJ-tube). Thickened feeds can help with emesis, and while G-tubes often do not stop emesis completely, they can limit the amount of nutrition lost through vomiting.

From: Bohring-Opitz Syndrome

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