A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.

Tetra-amelia is extremely rare with an incidence of 2.4 per 10,000,000 births. It describes the absence of all 4 outer extremities and can be associated with other malformations. The boy presented here was diagnosed at 22 1/7 weeks of gestation by sonography in 2D and 3D mode. The parents decided to continue the pregnancy; vaginal birth occurred after external rotation at 38 1/7 weeks of pregnancy. Postnatally, surgical closure of a cleft of the soft palate was performed. External abnormalities manifested themselves increasingly in the area of the spine and the face. The anatomically limited psychomotor development of the child is supported by physiotherapy, occupational therapy, and speech therapy. Various aids enable the child to participate in activities appropriate to his age. Eine Tetraamelie ist mit einer Prävalenz von 2,4 bezogen auf 10 000 000 Geburten extrem selten. Sie beschreibt das Fehlen aller 4 äußeren Extremitäten und kann mit anderen Fehlbildungen assoziiert sein. Bei dem hier vorgestellten Jungen wurde die Diagnose mit 22+1 Schwangerschaftswochen mittels Sonografie im 2D und 3D Modus gestellt. Die Eltern entschieden sich zur Fortsetzung der Schwangerschaft, der Spontanpartus erfolgte nach äußerer Wendung in 38+1 SSW. Postnatal erfolgte der operative Verschluss einer Spalte des weichen Gaumens. Im Bereich der Wirbelsäule und des Gesichts manifestierten sich zunehmend äußere Auffälligkeiten. Die anatomisch bedingt eingeschränkte psychomotorische Entwicklung des Kindes wird physiotherapeutisch, ergotherapeutisch und logopädisch gefördert. Verschiedene Hilfsmittel ermöglichen die Teilhabe an altersgerechten Aktivitäten.

Tetra-amelia is a rare congenital disorder characterized by the absence of limbs. We describe the anesthetic management of a 29-year-old woman with tetra-amelia who underwent general anesthesia for tympanomastoidectomy with meatoplasty for an extensive right ear cholesteatoma. Anesthetic challenges related to tetra-amelia include difficult intravenous access, lack of sites for blood pressure monitoring, and possible difficult airway management due to craniofacial anomalies. Our case report focuses on the complex establishment of iliac artery access for invasive blood pressure monitoring by initially cannulating the carotid artery.

We report the novel application of photoplethysmographic technology with the Nexfin HD monitor for real-time measurement of blood pressure (BP) in a patient with tetraamelia. The patient was a 58-year-old man with tetraamelia secondary to thalidomide exposure in utero, who presented for surgical excision of a maxillary schwannoma. Because difficulty of cuff use on rudimentary limbs and failure to gain invasive arterial access due to abnormalities of limb vasculature, this population is known to pose some unique challenges for BP measurement. Nexfin may offer an alternative noninvasive method to detect BP in patients with phocomelia during the perioperative period.