Anesthesia for Seckel syndrome

Abstract

intubation may accentuate any unrecognized preload deficit and further depress the cardiac output. It is plausible that in our patient the use of propofol as induction agent and the addition of positive pressure ventilation may have provided the setting for peripheral vasodilatation, alterations in preload, and finally, the decrease in cardiac output, enough to decrease coronary perfusion pressure. Unrecognized delayed dilated cardiomyopathy has important anesthetic implications, awareness of the potential for this pathophysiology to occur, recognition of its existence and a well-thought anesthetic plan is warranted. Luis M . Zab ala M D M D T ar i q Pa r r ay M D M D

Nasotracheal Intubation of a Patient with Seckel Syndrome by C-Mac Videolaryngoscope

“…Seckel sendromu (SS) ilk olarak 1960 yılında tanımlanan (1), ağır gelişme geriliği, mental retardasyon, retrognati, mikrosefali, cücelik ve tipik yüz görünümü (bird head-kuş kafası) ile karakterize, otozomal resesif, nadir görülen bir hastalıktır ve görülme sıklığı 10.000 canlı doğumda 1' dir (2). Şimdiye kadar genel anestezi uygulanan birçok vaka literatürde bildirilmiştir ve genelde zor havayolu ve zor damar yolu erişimleri sunulmuştur (3,4). Bunlara ek olarak perioperatif ve postoperatif birçok zorlukla da karşılaşılabilir.…”

Anesthetic management of a child with Seckel syndrome for multiple extractions and restoration of teeth

“…[ 1 6 ] Although many cases of Seckel syndrome are described in literature only a few have been reported who required anesthesia. [ 7 – 11 ] We report the anesthetic management of a patient of Seckel syndrome scheduled for multiple extractions and restorations of teeth.…”

“…A dysmorphic face with a receding chin and a relatively big nose could also render mask ventilation difficult. [ 11 ] Dental malocclusion, receding chin, high palatal arch could be the warning signs for the possibility of difficult intubation. [ 11 ] Although we were able to maintain mask ventilation without any difficulty we were prepared to handle the reported difficulty of mask fitting with an appropriate size laryngeal mask airway and the fiberoptic bronchoscope was kept ready in case intubation failed with direct laryngoscopy using stylet.…”

Anesthetic management of a patient with Seckel syndrome and implanted pacemaker

“…S ir —Seckel syndrome is an autozomal recessive inherited disorder, with an incidence of <1 in 10,000. It has characterized with significant intrauterine and postnatal growth retardation, microcephalia with mental retardation, a beak‐like triangular nose and ‘bird headed’ face appearance (1,2). We present our anesthetic approach during a cochlear implant operation in a patient who had Seckel syndrome with an implanted pacemaker.…”