Hemorrhagic Presentation of Rathke Cleft Cysts: A Surgical Case Series

2019 ◽  
Vol 18 (5) ◽  
pp. 470-479 ◽  
Author(s):  
Lauren Schooner ◽  
Michelle A Wedemeyer ◽  
Phillip A Bonney ◽  
Michelle Lin ◽  
Kyle Hurth ◽  
...  

Abstract BACKGROUND Rathke cleft cysts (RCCs) are benign sellar and suprasellar lesions commonly presenting as asymptomatic incidental findings. Rarely, RCCs hemorrhage and mimic pituitary apoplexy on presentation. OBJECTIVE To review a series of hemorrhagic RCCs for physicians encountering this rare presentation. METHODS A database review of >1700 transsphenoidal pituitary operations was performed at the USC Pituitary Center to identify patients with pathologically confirmed RCCs presenting with acute symptoms and evidence of hemorrhage at the time of surgery. Surgical treatment involved transsphenoidal RCC fenestration and drainage. Clinical, endocrine, and imaging outcomes were reviewed. RESULTS A total of 119 RCCs were identified, and 6 (5.0%) presented with hemorrhage mimicking pituitary apoplexy. Presenting symptoms included acute onset headaches (5/6), vision loss (2/6), and oculomotor nerve palsy (n = 1). Endocrine disturbances at presentation included pre-existing amenorrhea in all female patients (3/3), hypothyroidism (n = 2), panhypopituitarism (n = 2), and one with profound hyponatremia (Na 116 meq/L). All patients underwent endonasal transsphenoidal fenestration and drainage with no major complications. Over mean follow-up of 38.4 mo, 2/2 patients with vision loss reported improvement, and 2/5 patients with headaches reported improvement. Although all women resumed menses, patients with preoperative hypopituitarism did not experience pituitary axis improvement. Follow-up magnetic resonance imaging showed no instances of RCC recurrence with a mean imaging follow-up of 38.6 mo. CONCLUSION RCCs occasionally present with hemorrhage and clinical symptoms that may be confused with apoplexy. Outcomes following hemorrhagic RCC treatment are excellent when treated at tertiary pituitary centers. Although hyperprolactinemia often improves following surgery, other pituitary axis deficits typically do not.

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.


2015 ◽  
Vol 123 (3) ◽  
pp. 808-812 ◽  
Author(s):  
Avetis Azizyan ◽  
Joseph M. Miller ◽  
Ramzi I. Azzam ◽  
Marcel M. Maya ◽  
Pouyan Famini ◽  
...  

OBJECT Pituitary apoplexy is a rare and potentially life-threatening disorder that is most commonly characterized by a combination of sudden headache, visual disturbance, and hypothalamic/hormonal dysfunction. In many cases, there is hemorrhagic infarction of an underlying pituitary adenoma. The resulting clinical symptoms are due to compression of the remaining pituitary, cavernous sinuses, or cranial nerves. However, there are only 2 case reports in the literature describing spontaneous retroclival expansion of hemorrhage secondary to pituitary apoplexy. Ten cases of this entity with a review of the literature are presented here. METHODS This is a single-institution retrospective review of 2598 patients with sellar and parasellar masses during the 10-year period between 1999 and 2009. The pituitary and brain MRI and MRI studies were reviewed by 2 neuroradiologists for evidence of apoplexy, with particular attention given to retroclival extension. RESULTS Eighteen patients (13 men and 5 women; mean age 54 years) were identified with presenting symptoms of sudden onset of headache and ophthalmoplegia, and laboratory findings consistent with pituitary apoplexy. Ten of these patients (8 men and 2 women; mean age 55 years) had imaging findings consistent with retroclival hematoma. CONCLUSIONS Retroclival hemorrhage was seen in the majority of cases of pituitary apoplexy (56%), suggesting that it is more common than previously thought.


2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.


2018 ◽  
Vol 31 (3) ◽  
pp. 146 ◽  
Author(s):  
Luís Ramos dos Santos ◽  
Magna Alves-Correia ◽  
Margarida Câmara ◽  
Manuela Lélis ◽  
Carmo Caldeira ◽  
...  

Introduction: Carbon monoxide poisoning may occur in several contexts.Material and Methods: Retrospective of 37 carbon monoxide poisoning cases that underwent hyperbaric oxygen during wildfires in Funchal in August 2016.Results: The studied sample included 37 patients, mean age of 38 years, 78% males. Ten were firefighters, four children and two pregnant victims. Neurological symptoms were the most reported. Median carboxyhemoglobin level was 3.7% (IQR 2.7). All received high-flow oxygen from admission to delivery of hyperbaric oxygen. Persistence of symptoms was the main indication for hyperbaric oxygen. Median time to hyperbaric oxygen was 4.8 hours (IQR 9.5), at 2.5 ATA for 90 minutes, without major complications. Discharge in less than 24 hours occurred in 92% of the cases. Thirty days follow-up: five patients presented clinical symptoms of late neurological syndrome; twelve patients were lost to follow-up. Carboxyhemoglobin levels on admission and mean time to hyperbaric oxygen were no different between those who did and did not develop the syndrome at 30 days (p = 0.44 and p = 0.58, respectively).Discussion: Late neurological syndrome at 30 days occurred in 20% and no new cases were reported at 12 months.Conclusion: Use of hyperbaric oxygen appears to have reduced the incidence of the syndrome. This seems to be the first Portuguese series reporting use of hyperbaric oxygen in carbon monoxide poisoning due to wildfires. The authors intend to alert to the importance of referral of these patients because the indications and benefits of this treatment are well documented. This is especially important given the ever-growing issue of wildfires in Portugal.


Author(s):  
Mohammed Alahmari ◽  
Fahad Alkherayf ◽  
Andrea Lasso ◽  
Fatmahalzahra Banaz ◽  
Sepideh Mohajeri ◽  
...  

Abstract Background Pituitary apoplexy (PA) is a rare complication of pituitary tumors that can present with a myriad of symptoms, including sudden onset cranial nerve deficits. After patient stabilization and hormone replacement, surgical decompression is often recommended. The timing of surgical decompression remains controversial. In this case series, we describe our institutional experience pertaining to the cranial nerve recovery in patients who underwent endoscopic endonasal transsphenoidal (EETS) surgery for PA while evaluating outcome based on tumor stage using the suprasellar infrasellar parasellar anterior posterior (SIPAP) classification. Design Present study is a single-institution retrospective cohort. Methods A retrospective review of all EETS cases for pituitary tumor resection between November 2009 and August 2018. Queries of the hospital database were completed by trained personnel to identify cases of PA treated using the EETS approach. Baseline characteristics, tumor type, endocrine data, and SIPAP classification based on preoperative magnetic resonance imaging (MRI) and operation characteristics were extracted from medical records. Postoperative results were extracted for the duration of the follow-up period available for each patient. Results Fifteen cases of PA were identified. Patient follow-up period was a mean of 30 months. The cranial nerve deficits present at admission were visual deficit (33%); unilateral third nerve palsy (47%) and unilateral sixth nerve palsy (27%). No fourth nerve palsies were observed. Following EETS, 60% of patients with preoperative visual deficit had normal visual fields. For those with third and sixth nerve palsies preoperatively, 43 and 75%, respectively, had return to normal function postoperatively. SIPAP tumor characteristics were not related to postoperative cranial nerve recovery. Conclusion In this series of surgically treated patients with pituitary apoplexy, all cranial nerve deficits normalized or improved following surgery. The tumor SIPAP classification was not associated with patient outcome. Though in a small series, the presented results suggest surgical treatment is beneficial for these patients.


2018 ◽  
Vol 130 (1) ◽  
pp. 136-144 ◽  
Author(s):  
Michael Karsy ◽  
Hussam Abou-Al-Shaar ◽  
Christian A. Bowers ◽  
Richard H. Schmidt

OBJECTIVEIdiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a complex and difficult-to-manage condition that can lead to permanent vision loss and refractory headaches if untreated. Traditional treatment options, such as unilateral ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt placement, have high complication and failure rates and often require multiple revisions. The use of bilateral proximal catheters has been hypothesized as a method to improve shunt survival. The use of stereotactic technology has improved the accuracy of catheter placement and may improve treatment of IIH, with fewer complications and greater shunt patency time.METHODSThe authors performed a retrospective chart review for all patients with IIH who underwent stereotactic placement of biventriculoperitoneal (BVP) shunt catheters from 2008 to 2016 at their institution. Bilateral proximal catheters were Y-connected to a Strata valve with a single distal catheter. We evaluated clinical, surgical, and ophthalmological variables and outcomes.RESULTSMost patients in this series of 34 patients (mean age 34.4 ± 8.2 years, mean body mass index 38.7 ± 8.3 kg/m2; 91.2% were women) undergoing 41 shunt procedures presented with headache (94.1%) and visual deficits (85.3%). The mean opening pressure was 39.6 ± 9.0 cm H2O. In addition, 50.0% had undergone previous unilateral shunt placement, and 20.6% had undergone prior optic nerve sheath fenestration. After BVP shunt placement, there were no cases of proximal catheter obstruction and only a single case of valve obstruction at 41.9 months, with a mean follow-up of 24.8 ± 20.0 months. Most patients showed improvement in their headache (82.4%), subjective vision (70.6%), and papilledema (61.5% preoperatively vs 20.0% postoperatively, p = 0.02) at follow-up. Additional primary complications included 4 patients with migration of their distal catheters out of the peritoneum (twice in 1 patient), and an infection of the distal catheter after catheter dislodgment. The proximal obstructive shunt complication rate in this series (2.9%) was lower than that with LP (53.5%) or unilateral VP (37.8%) shunts seen in the literature.CONCLUSIONSThis small series suggests that stereotactic placement of BVP shunt catheters appears to improve shunt survival rates and presenting symptoms in patients with IIH. Compared with unilateral VP or LP shunts, the use of BVP shunts may be a more effective and more functionally sustained method for the treatment of IIH.


2018 ◽  
Vol 79 (02) ◽  
pp. e36-e40 ◽  
Author(s):  
Vincent Anagnos ◽  
Ricardo Hanel ◽  
Iman Naseri

AbstractA pituitary abscess is an often-overlooked diagnosis in the clinical presentation of a sellar mass. Due to its rare incidence and nonspecific presentation, diagnosis and treatment is often delayed. The authors describe a 56-year-old male patient presenting with acute onset of severe headache, visual field deficit, and radiologic findings of an expansile sellar lesion. The presenting symptoms were unremarkable for the diagnosis of meningitis, cavernous sinus thrombosis, and septicemia. Recent medical history included symptoms of rhinosinusitis on the days preceding his acute presentation. The initial clinical presentation was suggestive of a possible pituitary apoplexy. Intraoperative findings revealed purulent output upon surgical entry of the sella. Histopathology confirmed the diagnosis of a pituitary abscess. Review of the clinical and radiologic data revealed evidence of multiple opacifications within the paranasal sinuses, along with dehiscence overlying the sellar bone, supporting a diagnosis of secondary pituitary abscess. This case, along with a review of the available literature, will serve to expand our knowledge of this rare disease process that is often overlooked. Clinicians should be mindful of this condition, and include a primary versus secondary pituitary abscess in the differential workup on such cases.


2017 ◽  
Vol 102 (7) ◽  
pp. 971-976 ◽  
Author(s):  
Thibaud Mathis ◽  
Benoit Delaunay ◽  
Armelle Cahuzac ◽  
Vivien Vasseur ◽  
Martine Mauget-Faÿsse ◽  
...  

BackgroundMultiple evanescent white dot syndrome (MEWDS) is an inflammatory disease that can be associated with choroidalneovascularisation (CNV). However, few studies in the literature have described the occurrence of MEWDS in association with CNV. This paper discusses whether CNV can trigger MEWDS in a predisposed eye.MethodsA retrospective multicentric case series of six eyes in six patients with acute onset of MEWDS and evidence of previous CNV was conducted between January 2015 and January 2017. All patients underwent ophthalmic examination including multimodal imaging at baseline and during follow-up.ResultsThe mean age was 32.2±12.2 years. The majority of patients were women (5/1). In each case, MEWDS was diagnosed during a recurrence or occurrence of CNV secondary to choriocapillaritis, central serous chorioretinopathy or atrophic scar, presumably due to congenital toxoplasmosis. All patients were treated with intravitreal injections of antivascular endothelial growth factor (anti-VEGF) with good anatomical and functional responses (mean gain of 0.3±0.31logMAR). The mean duration of follow-up was 13.5±10.65 months.ConclusionThis study highlights a sequence in the development of MEWDS, following the occurrence or recurrence of CNV. CNV may trigger MEWDS, possibly due to the proinflammatory environment created by the retinal tissue surrounding the CNV.


2019 ◽  
Vol 50 (03) ◽  
pp. 188-192
Author(s):  
Saša Ilovar ◽  
Mirjana Benedik ◽  
Tina Vesnaver ◽  
Damjan Osredkar

Background Brain aneurysms are rare in the pediatric population. The diagnosis of a brain aneurysm in a child may be difficult because of its infrequency and often subtle or nonspecific clinical presentation. Endovascular therapy and microsurgical treatment are increasingly used approaches in treating children, possibly contributing to favorable outcomes if patients are treated in a timely manner. Objective We were interested in the clinical presentation, symptoms, diagnostics, treatment, and follow-up of pediatric patients with brain aneurysms in Slovenia. Methods This was a retrospective review of medical documentation of children with intracranial aneurysms treated at the University Children's Hospital in Ljubljana, Slovenia, from January 1998 to December 2017. Results We identified a cohort of eight children (median age: 14.9 years; range: 2.8–17.7). The estimated incidence of pediatric brain aneurysms in Slovenia is 0.12/100,000 children per year. We observed a male predominance (1.7:1). Half of the patients presented with acute onset of neurologic symptoms and three with subarachnoid hemorrhage. One of the patients had a related stroke. The presenting symptoms were tonic–clonic seizures, hemiparesis, paresthesias, speech disturbance, and cranial nerve palsy. The other half of aneurysms were identified incidentally. Five patients had anterior circulation aneurysms; the most prevalent location was the internal carotid artery. One patient was treated with surgical procedures, four patients were treated with endovascular procedures, and three patients were treated conservatively. Outcome was excellent in all patients. Conclusion Endovascular interventions and microsurgical procedures appear to be safe and effective in the treatment of brain aneurysms in the pediatric population. Asymptomatic patients with brain aneurysms need close follow-up.


2019 ◽  
pp. 193864001989206 ◽  
Author(s):  
Robin T. A. L. de Bot ◽  
Jasper Stevens ◽  
Joris P. S. Hermus ◽  
Heleen M. Staal ◽  
Lodewijk W. van Rhijn ◽  
...  

Background. The purpose of this study is to evaluate functional and radiological outcomes of subtalar arthroereisis in the treatment of symptomatic pediatric flexible flatfeet. Methods. A total of 16 patients (26 feet) were treated with a Kalix II as subtalar motion blocker between 2009 and 2014. Calcaneal pitch (CP) and Meary’s angle (MA) were measured on radiographs preoperatively, directly postoperatively, and at follow-up 47 ± 17 (range 19-79) months. Patient satisfaction surveys were used to assess functional outcome and patient satisfaction. Results. Surgery was performed mostly for pain, walking problems, or a combination of both at a mean age of 12.5 ± 1.5 (range 10-15) years. Symptoms were relieved in 62.5% of patients in the postoperative phase and increased to 68.75% at follow-up. A statistically significant increase in CP of 2.8° and decrease in MA of 14.0° was observed directly postoperatively, which persisted during the follow-up period irrespective of Kalix removal. Revision surgery was necessary in 6 cases (23%) because of arthroereisis migration. Conclusion. Subtalar Kalix II arthroereisis significantly reduced clinical symptoms and improved the CP and MA directly postoperatively, which persisted during follow-up, irrespective of Kalix removal. Therefore, subtalar arthroereisis is a considerable intervention to reduce symptoms in children with symptomatic flexible flatfeet. Levels of Evidence: Level IV: Case series


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