A Case of Cleft Lip and Palate Associated with Seckel Syndrome

Abstract

We report an unusual association of complete cleft of the primary and secondary palate with Seckel syndrome. Seckel syndrome is a very rare syndrome, with only 60 reported cases in the medical literature. It is an autosomal recessive disorder characterized by birdlike face, intrauterine growth retardation, dwarfism, and microcephaly. This young child of 5 years had a successful cleft lip repair under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed.

Palatoplasty in a patient with Seckel syndrome

“…The hypoplastic palatal tissue was difficult to handle during surgery. The patient had no postoperative complications such as apneic spells or intraoral bleed as reported in literature,[11] and the palatal healing was normal on follow-up.…”

“…[9] Kjaer[10] discussed that more pronounced orthopedic and dental abnormality is found in female children when compared with male. Till date only two adults[7] and one child[11] of Seckel syndrome have been administered GA for abdominal, intracranial, and cleft lip surgery, respectively. Although search of the literature did not reveal any such child undergoing cleft palate surgery, cleft lip surgeries have been reported in the past.…”

“…He had borderline intelligent quotient with nearly normal comprehensive and nonverbal expressive language. He had no strabismus or cataract of eyes and also had normal hematological and nutritional profile[11] because of which palatoplasty could be planned. The veins were very fragile causing difficulty in intravenous canula insertion.…”

Anesthesia in a child with Seckel syndrome

“…A literature review reveals that Seckel syndrome and anesthesia management have been reported as several case reports. All describe difficult mask ventilation, difficult airway management and difficult venous cannulation (4–8). Although our experience with this patient did not require tracheal intubation or venous access, review of her previous anesthesia records reveal no difficulty either with intubation or venous access.…”

Anesthesia for Seckel syndrome

“…S ir –Seckel syndrome is an extremely rare, inherited autosomal recessive disorder characterized by growth retardation, microcephaly with mental retardation, and a characteristic ‘bird‐headed’ facial appearance (1). Approximately 60 cases have been reported in the medical literature but only four cases have been associated with anesthesia (2–5).…”