Postoperative Massive Macroglossia in Klippel–Feil Syndrome After Posterior Occipitocervical Fixation Surgery in the Sitting Position

Abstract

Postoperative macroglossia is a very rare complication associated with neurosurgical procedures. There have only been 16 cases reported in the literature. With regard to spine surgery, however, only two cases have been reported. The first report was a case where the complication of postoperative macroglossia occurred during occipitocervical spine surgery in a sitting position. In this second documented incident, we report a case of massive macroglossia, in a 44-year-old man, following posterior craniocervical fixation and fusion surgery performed in a sitting position. The patient had Klippel-Feil syndrome with basilar invagination preoperatively. Postoperative macroglossia was treated by partial glossectomy, because medical management (anti-edema therapy) failed. This complication is a life-threatening complication and must be treated promptly. Diverse etiologic mechanisms are discussed along with preventive and therapeutic measures.

A Reminder for a Very Rare Entity: Massive Tongue Swelling after Posterior Fossa Surgery

“…13 Many treatments are proposed for macroglossia, ranging from oral toilet, bite blocks, pain relievers, to a partial glossectomy. 1 The surgical techniques may be subdivided into two groups: glossectomy along the median line and peripheral glossectomy, both techniques include the resection of a tissue portion and the subsequent suture of the margins. 14 The use of corticosteroids did not prove to be of value according to the literature, and did not improve the situation in our patient.…”

“…Macroglossia is a very rare complication following intracranial procedures. [1][2][3] Its evolution can be dramatic, even potentially lethal. Various hypotheses have been suggested, and the sitting position was favorised as being implicated in the development of macroglossia after surgery.…”

Delayed Lateral Rectus Palsy Following Resection of a Pineal Cyst in Sitting Position: Direct or Indirect Compressive Phenomenon?

“…1,7 Over the years, the popularity of this position for various neurosurgical and spinal approaches has dwindled because of untoward and potentially devastating complications. [3][4][5][6][7][8] Pneumocephalus is seen in virtually all cases following craniotomy in the sitting position. 2,3 However, it is the rare but dreaded sequelae of a tension pneumocephalus that can be particularly troubling for the patient and surgeon alike.…”

“…1,2 Among the numerous reported complications are pneumocephalus, macroglossia, air embolism, and (even more devastating) spinal cord infarction. [3][4][5][6][7][8] Pneumocephalus is seen in virtually all cases following surgery in the sitting position. 2,8 Patients frequently present with worsening headaches; however, neurologic manifestations are rare.…”

Macroglossia in a child undergoing posterior fossa surgery in sitting position

“…[12] Lingual obstruction may occur after use of airway, endotracheal tube, supraglottis device, esophageal stethoscope, and transesophageal echocardiography. [34] Macroglossia may occur in conditions without neck flexion due to abnormal brain stem signaling. [5] In our patient, soft airway (cotton made) was used and adequate (two fingers) distance between the chin and the sternum was maintained to prevent the consequences of severe neck flexion.…”