✓ Retrieval of free abdominal ventriculoperitoneal shunt catheters usually requires laparotomy. The authors describe successful removal of free peritoneal catheters by means of commonly available adult laparoscopic equipment in four pediatric patients aged 2 to 12 years.
Report of four cases
Gay M. Guzinski, William J. Meyer and John D. Loeser
A mouse model
William A. Roy, Robert J. Iorio and Glenn A. Meyer
✓ Craniosynostosis and associated craniofacial deformities, such as frontal bossing, often occur as symptoms of vitamin D-resistant rickets in children. Similar skull deformities develop in mice with X-linked dominant hypophosphatemia, the most common form of vitamin D-resistant rickets. These mice have a short, wide, high neurocranium, which suggested an inhibition of coronal suture growth. To study this question, we compared histologically the postnatal development of the coronal sutures in normal and hypophosphatemic mice between 1 and 13 weeks of age. Premature fusion of the coronal suture occurred in hypophosphatemic mice by 4 weeks of age. The proportion of the suture obliterated by bone varied among individual animals, but craniosynostosis was present in all animals studied at 4 weeks and older. Fusion of the coronal suture did not occur through 13 weeks of age in any of the normal mice studied. The X-linked hypophosphatemic mouse is an animal model that can be used to study the role of vitamin Dresistant rickets in the development of craniosynostosis, to relate craniosynostosis to the development of associated skull deformities, and to test new treatment procedures.
Russell Meyers, William J. Fry, Frank J. Fry, Leroy L. Dreyer, Donald F. Schultz and Robert F. Noyes
Report of two cases
Camilo E. Fadul, Andrew L. Kominsky, Louise P. Meyer, Linda S. Kingman, William B. Kinlaw, C. Harker Rhodes, Clifford J. Eskey and Nathan E. Simmons
✓Pituitary carcinoma is a rare tumor characterized by poor responsiveness to therapy, leading to early death. Reported responses to standard chemotherapy have only been anecdotal, with no single agent or combination demonstrating consistent efficacy in the treatment of patients with this disease. The authors report rare examples of a persistent response to cytotoxic chemotherapy in two patients with pituitary carcinoma.
One patient was a 38-year-old man with visual field loss caused by a luteinizing hormone–secreting pituitary carcinoma that had recurred despite multiple surgeries and radiation therapy. Intradural metastases to the spine that had failed to respond to radiation therapy were pathologically confirmed. The second patient was a 26-year-old man with hyperprolactinemia from a prolactin-secreting pituitary tumor. Spine magnetic resonance images obtained to search for causes of neck pain showed a vertebral tumor, which was later confirmed through pathological analysis to be a metastatic pituitary carcinoma. His disease progressed despite radiation therapy, high-dose bromocriptine, and chemotherapy.
Both patients were treated monthly with temozolomide, which was administered orally on the first 5 days of a 28-day cycle. The patient in the first case underwent all 12 treatment cycles without serious side effects, and his visual field deficits improved. The patient in the second case had undergone only 10 cycles when the drug was stopped because of his severe fatigue. Nonetheless, his pain disappeared and his serum prolactin concentration decreased. Both patients continue to have partial responses and have been employed full-time for more than 1 year after discontinuing temozolomide therapy. These two examples demonstrate that temozolomide may be effective in treating pituitary carcinomas and thus should be considered in the treatment algorithm for these difficult cases.
Benjamin T. Himes, Grant W. Mallory, Arnoley S. Abcejo, Jeffrey Pasternak, John L. D. Atkinson, Fredric B. Meyer, W. Richard Marsh, Michael J. Link, Michelle J. Clarke, William Perkins and Jamie J. Van Gompel
Historically, performing neurosurgery with the patient in the sitting position offered advantages such as improved visualization and gravity-assisted retraction. However, this position fell out of favor at many centers due to the perceived risk of venous air embolism (VAE) and other position-related complications. Some neurosurgical centers continue to perform sitting-position cases in select patients, often using modern monitoring techniques that may improve procedural safety. Therefore, this paper reports the risks associated with neurosurgical procedures performed in the sitting position in a modern series.
The authors reviewed the anesthesia records for instances of clinically significant VAE and other complications for all neurosurgical procedures performed in the sitting position between January 1, 2000, and October 8, 2013. In addition, a prospectively maintained morbidity and mortality log of these procedures was reviewed for instances of subdural or intracerebral hemorrhage, tension pneumocephalus, and quadriplegia. Both overall and specific complication rates were calculated in relation to the specific type of procedure.
In a series of 1792 procedures, the overall complication rate related to the sitting position was 1.45%, which included clinically significant VAE, tension pneumocephalus, and subdural hemorrhage. The rate of any detected VAE was 4.7%, but the rate of VAE requiring clinical intervention was 1.06%. The risk of clinically significant VAE was highest in patients undergoing suboccipital craniotomy/craniectomy with a rate of 2.7% and an odds ratio (OR) of 2.8 relative to deep brain stimulator cases (95% confidence interval [CI] 1.2–70, p = 0.04). Sitting cervical spine cases had a comparatively lower complication rate of 0.7% and an OR of 0.28 as compared with all cranial procedures (95% CI 0.12–0.67, p < 0.01). Sitting cervical cases were further subdivided into extradural and intradural procedures. The rate of complications in intradural cases was significantly higher (OR 7.3, 95% CI 1.4–39, p = 0.02) than for extradural cases. The risk of VAE in intradural spine procedures did not differ significantly from sitting suboccipital craniotomy/craniectomy cases (OR 0.69, 95% CI 0.09–5.4, p = 0.7). Two cases (0.1%) had to be aborted intraoperatively due to complications. There were no instances of intraoperative deaths, although there was a single death within 30 days of surgery.
In this large, modern series of cases performed in the sitting position, the complication rate was low. Suboccipital craniotomy/craniectomy was associated with the highest risk of complications. When appropriately used with modern anesthesia techniques, the sitting position provides a safe means of surgical access.
Panagiotis Kerezoudis, Mohammed Ali Alvi, Daniel S. Ubl, Kristine T. Hanson, William E. Krauss, Fredric B. Meyer, Robert J. Spinner, Elizabeth B. Habermann and Mohamad Bydon
Patient-reported outcomes have been increasingly mandated by regulators and payers to evaluate hospital and physician performance. The purpose of this study is to delineate the differences in patient-reported experience of hospital care for cranial and spinal operations.
The authors selected all patients who underwent inpatient, elective cranial or spinal procedures and completed the Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS) survey at a single, high-volume, tertiary care institution between October 2012 and September 2015. The association of the surgical procedure and diagnosis with various HCAHPS composite measures, calculated across 9 domains using standard top-box methodology, was investigated. Multivariable logistic regression models were fitted for outcomes that were significant with procedure type and diagnosis group on univariate analysis, adjusting for age, sex, case complexity, overall health rating, and education level.
A total of 1484 patients met criteria and returned an HCAHPS survey. Overall, patients undergoing a cranial procedure gave top-box (most favorable) scores more often in pain management measure (66.3% vs 59.6%, p = 0.01) compared with those undergoing spine surgery. Furthermore, despite better discharge scores (93.1% vs 87.1%, p < 0.001), spinal patients were less likely to report excellent health (7.4% vs 12.7%). Lastly, patients with a primary diagnosis of brain or spinal tumor compared with those with degenerative spinal disease and those with other neurosurgical diagnoses provided top-box scores more often regarding communication with doctors (82.7% vs 76.4% vs 75.2%, p = 0.04), pain management (71.8% vs 60.9% vs 59.1%, p = 0.002), and global rating (90.4% vs 84.0% vs 87.3%, p = 0.02). On multivariable analysis, spinal patients had significantly lower odds of reporting top-box scores in pain management (OR 0.67, 95% CI 0.52–0.85; p = 0.001), staff responsiveness (OR 0.68, 95% CI 0.53–0.87; p = 0.002), and global rating (OR 0.59, 95% CI 0.42–0.82; p = 0.002), and significantly higher odds of top-box scoring in discharge information (OR 2.15, 95% CI 1.45–3.18; p < 0.001) than cranial patients. Similarly, brain tumor cases were associated with significantly higher odds of top-box scoring in communication with doctors (OR 1.46, 95% CI 1.01–2.12; p = 0.04), pain management (OR 1.81, 95% CI 1.29–2.55; p < 0.001), staff responsiveness (OR 1.88, 95% CI 1.33–2.66; p < 0.001), and global rating (OR 2.00, 95% CI 1.26–3.17; p = 0.003) compared with degenerative spine cases.
Significant differences in patient-reported experience with hospital care exist across different cranial and spine surgery patient populations. Overall, spinal patients, particularly those with degenerative spine disease, rated their health and their hospital experience lower relative to cranial patients. Identifying weaker areas of hospital performance in target populations can stimulate quality initiatives that aim to increase the overall hospital score.
Christopher S. Graffeo, Avital Perry, Lucas P. Carlstrom, Fredric B. Meyer, John L. D. Atkinson, Dana Erickson, Todd B. Nippoldt, William F. Young Jr., Bruce E. Pollock and Jamie J. Van Gompel
Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)–secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability.
The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Statistical tests included Student's t-test, chi-square test, Fisher's exact test, multivariate analysis, and derived receiver operating characteristic curves.
Between 1956 and 2015, 302 patients underwent bilateral ADX for the treatment of hypercortisolism caused by CD; 88 had requisite imaging and follow-up (mean 16 years). Forty-seven patients (53%) had radiographic progression of pituitary disease and were diagnosed with NSS. Compared with patients who did not experience progression, those who developed NSS were significantly younger at the time of CD diagnosis (33 vs 44 years, p = 0.007) and at the time of bilateral ADX (35 vs 49 years, p = 0.007), had larger tumors at the time of CD diagnosis (6 mm vs 1 mm, p = 0.03), and were more likely to have undergone external-beam radiation therapy (EBRT, 43% vs 12%, p = 0.005). Among NSS patients, the mean tumor growth was 7 mm/yr (SE 6 mm/yr); the median tumor growth was 3 mm/yr. Prevalence of pathognomonic symptoms was low; the classic triad occurred in 9%, while hyperpigmentation without visual field deficit was observed in 23%, and 68% remained asymptomatic despite radiographic disease progression. NSS required treatment in 14 patients (30%).
NSS is a prevalent sequela of CD after bilateral ADX and affects more than 50% of patients. However, although radiological evidence of NSS is common, it is most often clinically indolent, with only a small minority of patients developing the more aggressive disease phenotype characterized by clinically meaningful symptoms and indications for treatment. Young age at the time of CD diagnosis or treatment with bilateral ADX, large tumor size at CD diagnosis, and EBRT are associated with progression to NSS and may be markers of aggressiveness.