A child with Russell–Silver syndrome (RSS) undergoing surgery for congenital cataract: Case report

ABSTRACT

Background

Russell–Silver syndrome (RSS) is an uncommon but well-known imprinting condition primarily characterized by postnatal development failure and idiopathic intrauterine growth retardation (IUGR) and an inverted triangular face and a prominent forehead with relative macrocephaly that distinguish it from idiopathic IUGR and other causes of postnatal growth failure. Few case reports of RSS with cleft palate have been published and those who have perioperative issue such difficult intubation owing to trismus and difficulty to use a mouthpiece due to mandibular development failure.

Case presentation

Female child with RSS was subjected to cataract surgery performed under general anesthesia. Despite limited mouth opening and short thyro-mental distance, the intubation was relatively easy. The patient was extubated and moved to the postoperative care area. Postoperative interval passed uneventful.

Conclusion

In RSS patients, anesthetic considerations of difficult airway and pharyngeal edema must be avoided as a perioperative complication using pediatric fibro-optic device.

SUMMARY OF THE CASE

The rare condition Russell–Silver syndrome (RSS) is characterized by intrauterine growth restriction, poor postnatal growth, a big head, triangular-shaped face, and a prominent forehead (seeing the face from the side), body asymmetry, and major issues in feeding. It can be diagnosed clinically and by genetic mapping in about 60% of cases. The severity and frequency of the wide range of symptoms vary from one affected person to another. Although most individuals with RSS have normal IQs, common symptoms include movement and/or speech difficulties. This condition affects the anesthesia in many ways including difficult in airway and difficult cannulation.

KEYWORDS:

• Russel–Silver syndrome
• congenital cataract surgery

Disclosure statement

No potential conflict of interest was reported by the author(s).