Gorham-Stout disease of skull base

Gorham-Stout disease of skull base

Lesions of the skull base are extremely rare and entail an even more devastating prognosis due to cervical instability and cerebrospinal fluid fistula. Due to the scarcity of this condition, the aim of a study was to give an overview of skull base Gorham-Stout disease and review the cases with such conditions reported in the literature.

In this case-based review, different aspects of skull base GSD are discussed, and a sample clinical case of GSD leading to cranial settling and rhinorrhea is presented. The characteristics, symptoms, and management of all English-language PubMed-reported cases were reviewed, and different features of presentation and methods of treatments were analyzed.

Based on the literature review, most of the cases encountered serious problems in the course of the disease. Meningitis/CSF leakage was detected in 12 of 26 collected cases, followed by hearing loss/tinnitus/otitis media in 10 cases, headache in 8, and neck pain/stiffness in 8 patients. Despite a variety of treatments, improvement was only observed in 8 of 26 collected cases. The reminders showed either stable condition or worsening and death.

All cases of GSD of the skull base should be evaluated for rhinorrhea/otorrhea and cranial settling, both of them being among the most life-threatening conditions. Since definite treatment, in order to stop disease progression, is sometimes impossible, symptomatic and supportive treatment should be started as possible. 1).


6 patients (5 males, 1 female) were included. The mean age at diagnosis was 3.5 years (range 0-10). Follow-up was of 5.2 years. Patients were divided into Naso-temporal (NT) and Vertebro-temporal (VT) groups following anatomical location. NT patients (4 patients) all had petrous defects extending anteriorly, including sphenoid, ethmoidal, and mandibular defects. They all had cerebrospinal fluid fistula (CSF) and recurrent meningitis (range from 3 to 7). Two of those patients had sequelae including deafness, paralysis, and epilepsy. VT patients (2 patients) all had temporal, occipital bone and cervical vertebrae defects. None had CSF leaks but both died from medullar compression (preceded by tetraparesis in one case). Overall, five out of six patients had type I Chiari malformation. Interferon seemed to be the most efficient medical treatment. Surgery included petrectomy, endonasal surgery for CSF leak management, and neurosurgery for medullar management but could not guarantee long-term effects.

The main issues in skull base defects are cerebrospinal fluid fistulas leaks and medullacompressions. Surgical treatment is necessary in both cases but can only be satisfactory if general medical treatment can stabilize the disease 2).

A 27-year-old man was diagnosed with GSD with the involvement of the maxillofacial bones and skull base. The patient developed SBO; LMS resulted from progressive osteolysis, and the patient died of an associated brainstem stroke. Careful follow-up with special emphasis on the early detection of intracranial complications is critical in patients presenting with progressive GSD with involvement of the skull base 3)


A two-year-old female child with radiological signs mimicking those of raised intracranial pressure is discussed. The differential diagnosis consists of skull base tumors, meningitisosteomyelitis of the skull basecongenital hydrocephalus, and congenital syndromes involving the skull base. Pathologically it can be very difficult to differentiate it from lymphangioma of the bone. Difficulty in establishing the diagnosis is discussed along with the failure of radiotherapy and palmidronate therapy to cause the arrest of the disease process and failure of surgery to provide stabilization. Girn et al. described the course of the disease in this child over the period of last eight years. This was the youngest case of Gorham’s described so far 4).


A 25-year-old woman with Chiari I malformation associated with Gorham’s syndrome presented with aggressive paresthesia following bacterial meningitis. Axial magnetic resonance imaging (MRI) and computed tomography (CT) cisternography revealed CSF leakage in the right petrous apex. A presyrinx state was diagnosed based on the clinical symptoms and MRI findings. With the resolution of bacterial meningitis, the spinal edema and tonsillar ectopia also improved. Surgical repair of the CSF leakage was performed by an endoscopic endonasal transsphenoidal approach to prevent recurrence of meningitis. The postoperative course was uneventful.

Skull base osteolysis in Gorham’s syndrome may induce Chiari I malformation and CSF leakage. We should pay attention to the acute progression of clinical symptoms because Gorham’s syndrome may predispose to the development of Chiari I malformation and may be complicated by CSF leakage 5).


A case of spinal and skull base Gorham’s disease that was reversed by radiation therapy administered while the spine was supported by a halo-vest 6).


1)

Maroufi SF, Habibi Z, Dabbagh Ohadi MA, Mohammadi E, Nejat F. Gorham-Stout disease of skull base leading to cranial settling and rhinorrhea: a case-based review. Childs Nerv Syst. 2022 Feb 25. doi: 10.1007/s00381-021-05394-3. Epub ahead of print. PMID: 35217940.
2)

Simon F, Luscan R, Khonsari RH, Toubiana J, Belhous K, James S, Blauwblomme T, Zerah M, Denoyelle F, Donadieu J, Couloigner V. Management of Gorham Stout disease with skull-base defects: Case series of six children and literature review. Int J Pediatr Otorhinolaryngol. 2019 Sep;124:152-156. doi: 10.1016/j.ijporl.2019.06.002. Epub 2019 Jun 5. PMID: 31195309.
3)

Nozawa A, Ozeki M, Hori T, Kato H, Ohe N, Fukao T. Fatal Progression of Gorham-Stout Disease with Skull Base Osteomyelitis and Lateral Medullary Syndrome. Intern Med. 2019 Jul 1;58(13):1929-1933. doi: 10.2169/internalmedicine.2118-18. Epub 2019 Feb 25. PMID: 30799352; PMCID: PMC6663530.
4)

Girn HR, Towns G, Chumas P, Holland P, Chakrabarty A. Gorham’s disease of skull base and cervical spine–confusing picture in a two year old. Acta Neurochir (Wien). 2006 Aug;148(8):909-13; discussion 913. doi: 10.1007/s00701-005-0806-x. Epub 2006 Jun 23. PMID: 16791440.
5)

Nagashima H, Mizukawa K, Taniguchi M, Yamamoto Y, Kohmura E. Cerebrospinal fluid leakage and Chiari I malformation with Gorham’s disease of the skull base: A case report. Neurol Neurochir Pol. 2017 Sep-Oct;51(5):427-431. doi: 10.1016/j.pjnns.2017.06.007. Epub 2017 Jul 13. PMID: 28743389.
6)

Mawk JR, Obukhov SK, Nichols WD, Wynne TD, Odell JM, Urman SM. Successful conservative management of Gorham disease of the skull base and cervical spine. Childs Nerv Syst. 1997 Nov-Dec;13(11-12):622-5. doi: 10.1007/s003810050155. PMID: 9454981.

Skull base meningioma outcome

Skull base meningioma outcome

Peritumoral edema (PTE) in skull base meningiomas correlates to the absence of an arachnoid plane and difference in outcome.

A subset of benign (WHO grade I) skull base meningiomas shows early progression/recurrence (P/R) in the first years after surgical resection.


Though various predictors of adverse postoperative outcomes among meningioma patients have been established, research has yet to develop a method for consolidating these findings to allow for predictions of adverse healthcare outcomes for patients diagnosed with skull base meningiomas.


The objective of a study was to develop three predictive algorithms that can be used to estimate an individual patient’s probability of extended length of stay (LOS), experiencing a nonroutine discharge disposition, or incurring high hospital charges following surgical resection of a skull base meningioma.

The study utilized data from patients who underwent surgical resection for skull base meningiomas at a single academic institution between 2017-2019. Multivariate logistic regression analysis was used to predict extended LOS, nonroutine discharge, and high hospital charges, and 2000 bootstrapped samples were used to calculate an optimism-corrected c-statistic. The Hosmer-Lemeshow test was used to assess model calibration, and p<0.05 was considered statistically significant.

A total of 245 patients were included in our analysis. Our cohort was majority female (77.6%) and Caucasian (62.4%). Our models predicting extended LOS, nonroutine discharge, and high hospital charges had optimism-corrected c-statistics of 0.768, 0.784, and 0.783, respectively. All models demonstrated adequate calibration (p>0.05), and were deployed an open-access, online calculator: https://neurooncsurgery3.shinyapps.io/high_value_skull_base_calc/.

Following external validation, these predictive models have the potential to aid clinicians in providing patients with individualized risk-estimation for healthcare outcomes following meningioma surgery 1).


Ko et al. retrospectively investigated the preoperative CT and MR imaging features for the prediction of P/R in skull base meningiomas, with emphasis on quantitative ADC values. Only patients had postoperative MRI follow-ups for more than 1 year (at least every 6 months) were included. From October 2006 to December 2015, total 73 patients diagnosed with benign (WHO grade I) skull base meningiomas were included (median follow-up time 41 months), and 17 (23.3%) patients had P/R (median time to P/R 28 months). Skull base meningiomas with spheno-orbital location, adjacent bone invasion, high DWI, and lower ADC value/ratio were significantly associated with P/R (P < 0.05). The cut-off points of ADC value and ADC ratio for prediction of P/R are 0.83 × 10- 3 mm2/s and 1.09 respectively, with excellent area under curve (AUC) values (0.86 and 0.91) (P < 0.05). In multivariate logistic regression, low ADC values (< 0.83 × 10- 3 mm2/s) and adjacent bone invasion are high-risk factors of P/R (P < 0.05), with odds ratios of 31.53 and 17.59 respectively. The preoperative CT and MRI features for prediction of P/R offered clinically vital information for the planning of treatment in skull base meningiomas 2).


1)

Jimenez AE, Khalafallah AM, Lam S, Horowitz MA, Azmeh O, Rakovec M, Patel P, Porras JL, Mukherjee D. Predicting High-Value Care Outcomes Following Surgery for Skull Base Meningiomas. World Neurosurg. 2021 Feb 7:S1878-8750(21)00188-1. doi: 10.1016/j.wneu.2021.02.007. Epub ahead of print. PMID: 33567369.
2)

Ko CC, Lim SW, Chen TY, Chen JH, Li CF, Shiue YL. Prediction of progression in skull base meningiomas: additional benefits of apparent diffusion coefficient value. J Neurooncol. 2018 Jan 20. doi: 10.1007/s11060-018-2769-9. [Epub ahead of print] PubMed PMID: 29353434.

Cerebrospinal fluid leak after endoscopic skull base surgery

Cerebrospinal fluid leak after endoscopic skull base surgery

Although rates of postoperative morbidity and mortality have become relatively low in patients undergoing transnasal transsphenoidal surgery (TSS) for pituitary adenomacerebrospinal fluid fistulas remain a major driver of postoperative morbidity. Persistent CSF fistulas harbor the potential for headache and meningitis.

Staartjes et al., trained and internally validated a robust deep neural network-based prediction model that identifies patients at high risk for intraoperative CSF. Machine learning algorithms may predict outcomes and adverse events that were previously nearly unpredictable, thus enabling safer and improved patient care and better patient counseling 1).


The objective of a study of Umamaheswaran et al., was to assess the incidence of CSF leak following pituitary surgery and the methods of effective skull base repair. This retrospective observational study conducted in a tertiary care hospital after obtaining due clearance from the Institutional ethics committee. The charts of patients who underwent endonasal pituitary surgery between 2013 and 2018 were studied and details noted. Patients undergoing revision surgery or with history of preoperative radiotherapy were excluded from the study. 52 patients were included in the study. Based on the type of CSF leak, the patients were grouped into four. 19 patients (36.5%) had an intraoperative CSF leak. 3 patients developed a postoperative CSF leak. Based on the histopathology, 4 patients had ACTH secreting tumor. 8 patients had growth hormone secreting tumor, 22 had gonadotropin secreting tumor, 9 patients had a non-functioning tumour and 9 patients had prolactinoma. The type of skull base repair performed in these patients were grouped into 4.18 patients underwent type I repair, 21 patients underwent type II repair, 8 patients underwent type III repair and 5 patients underwent type IV repair. They observed that the pedicled nasoseptal flap is particularly advantageous over other repair techniques, especially in low pressure leaks. The strategy for skull base repair should be tailored to suit each patient to minimise the occurrence of morbidity and the duration of hospital stay 2).


Cerebrospinal fluid leakage is always the primary complication during the endoscopic endonasal skull base surgery.

Dural suturing technique may supply a rescue method. However, suturing and knotting in such a deep and narrow space are difficult. Training in the model can improve skills and setting a stepwise curriculum can increase trainers’ interest and confidence.

Xie et al. constructed an easy model using silicone and acrylic as sphenoid sinus and using the egg-shell membrane as skull base dura. The training is divided into three steps: Step 1: extracorporeal knot-tying suture on the silicone of sphenoid sinus, Step 2: intra-nasal knot-tying suture on the same silicone, and Step 3: intra-nasal egg-shell membrane knot-tying suture. Fifteen experienced microneurosurgical neurosurgeons (Group A) and ten inexperienced PGY residents (Group B) were recruited to perform the tasks. Performance measures were time, suturing and knotting errors, and needle and thread manipulations. The third step was assessed through the injection of full water into the other side of the egg to verify the watertight suture. The results were compared between two groups.

Group A finishes the first and second tasks in significantly less time (total time, 125.1 ± 10.8 vs 195.8 ± 15.9 min) and fewer error points (2.4 ± 1.3 vs 5.3 ± 1.0) than group B. There are five trainers in group A who passed the third step, this number in group B was only one.

This low cost and stepwise training model improved the suture and knot skills for skull base repair during endoscopic endonasal surgery. Experienced microneurosurgical neurosurgeons perform this technique more competent 3).

In-Hospital Costs

All endoscopic transsphenoidal approach for pituitary surgeries performed from January 1, 2015, to October 24, 2017, with complete data were evaluated in a retrospective single-institution study. The electronic medical record was reviewed for patient factors, tumor characteristics, and cost variables during each hospital stay. Multivariate linear regression was performed using Stata software.

The analysis included 190 patients and average length of stay was 4.71 days. Average total in-hospital cost was $28,624 (95% confidence interval $25,094-$32,155) with average total direct cost of $19,444 ($17,136-$21,752) and total indirect cost of $9181 ($7592-$10,409). On multivariate regression, post-operative cerebrospinal fluid (CSF) leak was associated with a significant increase in all cost variables, including a total cost increase of $40,981 ($15,474-$66,489, P = .002). Current smoking status was associated with an increased total cost of $20,189 ($6,638-$33,740, P = .004). Self-reported Caucasian ethnicity was associated with a significant decrease in total cost of $6646 (-$12,760 to -$532, P = .033). Post-operative DI was associated with increased costs across all variables that were not statistically significant.

Post-operative CSF leak, current smoking status, and non-Caucasian ethnicity were associated with significantly increased costs. Understanding of cost drivers of endoscopic transphenoidal pituitary surgery is critical for future cost control and value creation initiatives 4).

Case series

see Cerebrospinal fluid leak after endoscopic skull base surgery case series.

References

1)

Staartjes VE, Zattra CM, Akeret K, Maldaner N, Muscas G, Bas van Niftrik CH, Fierstra J, Regli L, Serra C. Neural network-based identification of patients at high risk for intraoperative cerebrospinal fluid leaks in endoscopic pituitary surgery. J Neurosurg. 2019 Jun 21:1-7. doi: 10.3171/2019.4.JNS19477. [Epub ahead of print] PubMed PMID: 31226693.
2)

Umamaheswaran P, Krishnaswamy V, Krishnamurthy G, Mohanty S. Outcomes of Surgical Repair of Skull Base Defects Following Endonasal Pituitary Surgery: A Retrospective Observational Study. Indian J Otolaryngol Head Neck Surg. 2019 Mar;71(1):66-70. doi: 10.1007/s12070-018-1511-4. Epub 2018 Oct 15. PubMed PMID: 30906716; PubMed Central PMCID: PMC6401034.
3)

Xie T, Zhang X, Gu Y, Sun C, Liu T. A low cost and stepwise training model for skull base repair using a suturing and knotting technique during endoscopic endonasal surgery. Eur Arch Otorhinolaryngol. 2018 Jun 1. doi: 10.1007/s00405-018-5024-2. [Epub ahead of print] PubMed PMID: 29858924.
4)

Parasher AK, Lerner DK, Glicksman JT, et al. Drivers of In-Hospital Costs Following Endoscopic Transphenoidal Pituitary Surgery [published online ahead of print, 2020 Aug 24]. Laryngoscope. 2020;10.1002/lary.29041. doi:10.1002/lary.29041
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