Frontal Encephalocele Associated With a Bilateral Tessier Number Three Cleft and Fraser Syndrome.

Oblique craniofacial clefts and encephaloceles are each rare conditions, and only a few instances of these findings in combination have been described. Each pathologic entity presents a unique reconstructive challenge. The authors report the case of a male infant who presented with a large right frontoencephalocele and bilateral Tessier number 3 clefts. A review of the pertinent literature, including specific considerations related to perioperative management and technical aspects of the surgical approach, is discussed.

Delivery of anesthesia and complications for children with Fraser syndrome: a review of 125 anesthetics.

OBJECTIVES: To perform a retrospective, anesthesia case note review of patients with Fraser syndrome. AIM: To identify the perioperative and postoperative anesthetic management and complications in this patient group. BACKGROUND: Fraser syndrome is a rare, autosomal recessively inherited disorder characterized by cryptophthalmos, cutaneous syndactyly, and ambiguous genitalia. It also has variable association with cardiac, laryngeal, tracheal, and gastrointestinal abnormalities. Children with Fraser syndrome present for a variety of surgical and radiological procedures, and there are a number of single case reports in the literature. METHODS: A retrospective case note review was undertaken on 10 children with Fraser syndrome who presented to our institution over a 30-year period. Analysis of the medical notes and general anesthetic records was undertaken, and the conduct of anesthesia, spectrum of disease, genetic markers, and perioperative complications were recorded. There were a total of 125 procedures performed under general anesthesia during this period. RESULTS: There were a total of ten anesthetic complications in the review, all related to management of the airway. There were two accidental extubations, five recorded incidents of airway obstruction (which were relieved with continuous positive airway pressure (CPAP)) and one posttracheostomy airway bleed. One child was a difficult intubation requiring an ID 2.5 mm oral endotracheal tube (ETT), and one child was an impossible intubation that required an emergency tracheostomy to secure the airway. CONCLUSIONS: There was a low incidence of complications with this group of patients. However, there is a relatively high incidence of difficult or impossible tracheal intubation (20%) due to glottic stenosis and one patient required an emergency tracheostomy despite no previous clinical evidence of airway narrowing.

Two-stage reconstruction for eyelid deformities in partial cryptophthalmos.

PURPOSE: Cryptophthalmos was first described in 1872 as a rare syndrome consisting of congenital upper eyelid and eyebrow coloboma and a rudimentary ocular bulb. Later, Fraser described it as being part of a complex syndrome with multiple anomalies. Many cases have been described since then, but very few surgical techniques have been proposed for its correction. In this paper, the authors present a surgical procedure for correction of the upper eyelid and ocular bulb anomalies in cryptophthalmos. METHODS: Five patients with Fraser syndrome were seen from 1993 to 2008. They were in the age group of 18 months to 24 years. Of these, 4 patients underwent reconstruction of the upper eyelid. RESULTS: The large upper eyelid coloboma and the cutaneous pterygium were treated with a 2-stage procedure, with good results obtained with the adoption of Mustardé's technique. CONCLUSIONS: The complexity of the surgical treatment increases when there is no possibility of rudimentary eye bulb preservation. In upper eyelid reconstruction, the authors adopted Mustardé's technique with fixation of the lower eyelid transposed flap to the levator muscle, leading this way to relatively good upper eyelid motility. The authors consider imperative that the upper fornix be reconstructed with adequate depth to allow perfect positioning of an aesthetic prosthesis, in this way improving the final aesthetic and functional results.

[Bilateral anophthalmia and left-sided orbital tumor : case of an eight-month-old infant]. / Beidseitiger Anophthalmus und linksseitiger Orbitatumor : Fall eines acht Monate alten Säuglings.

An 8-month-old infant from Russia with bilateral anophthalmia presented with an expanding orbital tumor. The tumor was extirpated and the histological examination revealed a non-malignant pseudocystic process with residual neuro-ectodermal structures.