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Simona Mihaela Florea, Alice Faure, Hervé Brunel, Nadine Girard and Didier Scavarda

The embryological development of the central nervous system takes place during the neurulation process, which includes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD.

This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5–S1 level, with a thickened filum terminale.

The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale.

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Simona Mihaela Florea, Thomas Graillon, Thomas Cuny, Regis Gras, Thierry Brue and Henry Dufour

OBJECTIVE

Ophthalmoplegia is a rare complication of transsphenoidal surgery, only noted in a few studies. The purpose of this study was to analyze the complications of cranial nerve III, IV, or VI palsy after transsphenoidal surgery for pituitary adenoma and understand its physiopathology and outcome.

METHODS

The authors retrospectively analyzed 24 cases of postoperative ophthalmoplegia selected from the 1694 patients operated via a transsphenoidal route in their department.

RESULTS

Two patients were operated on via microscopy and 22 via endoscopy. Patients operated on endoscopically had a greater risk of presenting with an extraocular nerve deficit postoperatively (p = 0.0115). It was found that an extension into or an invasion of the cavernous sinus (Knosp grade 3 or 4 on MRI, 18/24 patients) was correlated with a higher risk of postoperative ophthalmoplegia (p < 0.0001). The deficit was apparent immediately after surgery in 2 patients. For these 2 patients, the mechanisms of ophthalmoplegia were compression or intraoperative nerve lesion. The other 22 patients became symptomatic in the 12–72 hours following the surgery. The mechanisms implied in these cases were intrasellar compressive hematoma (4/22 cases), intracavernous hemorrhagic suffusion, or incomplete resection of the intracavernous portion of the tumor. All patients who did not present with oculomotor palsy immediately after surgery completely recovered their deficits in the 3 months that followed, while the other 2 experienced permanent damage.

CONCLUSIONS

Extraocular nerve dysfunction after transsphenoidal pituitary surgery is a rare complication that occurs more frequently in the case of the invasion or an important extension into the cavernous sinus. In this series, it also appears to be significantly more frequent in patients operated on via an endoscopic approach. Most patients have deficits that appear with a delay of 12–72 hours postoperatively and they are most likely to completely recover.