Robin Sequence and Osteopathia Striata With Cranial Sclerosis (OSCS): A Case Series.
Arhana Chattopadhyay, Liara S Ortiz-Ocasio, Natasha Shur et al.
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Case in brief
Two infants presented with Robin sequence (RS) alongside osteopathia striata with cranial sclerosis (OSCS), an uncommon combination. Both underwent mandibular distraction osteogenesis (MDO) for upper airway obstruction. In the infant with more severe OSCS, apneic episodes persisted after MDO and tracheostomy was required. In the other infant, MDO successfully managed the obstruction. The contrasting outcomes suggest that OSCS severity may be relevant when assessing the likely response to MDO in this clinical setting.
What made this case unusual
The two infants had different airway outcomes after MDO: persistent apnea requiring tracheostomy in the infant with more severe OSCS, versus successful obstruction management in the other.
Diagnostic / clinical pearl
In infants with RS and OSCS, consider OSCS severity when assessing airway management and the potential outcome of MDO; persistent apnea may require further intervention.