Symptom relief and quality of life after combined partial hepatectomy and cyst fenestration in highly symptomatic polycystic liver disease.

BACKGROUND: Polycystic liver disease can cause severe symptomatic hepatomegaly. Combined partial hepatectomy and cyst fenestration can be performed to reduce liver volume and symptom burden. We aimed to assess change in symptom relief and quality of life 6 months after partial hepatectomy and cyst fenestration in polycystic liver disease patients. METHOD: We established a prospective cohort between 2014 and 2018 at a referral center in the United States. Patients who underwent partial hepatectomy and cyst fenestration for volume-related symptoms were included. Primary outcome was change in polycystic liver disease-related symptoms, measured with Polycystic Liver Disease Questionnaire. Secondary outcomes were change in liver volume (computed tomography/ magnetic resonance imaging) and change in quality of life, measured with the 12-Item Short Form Survey and the EuroQoL Visual Analogue Scale. Questionnaire scores range from 0 to 100 and were assessed before and 6 months after partial hepatectomy and cyst fenestration. Surgical complications were scored according to Clavien-Dindo (grade 1 to 5). RESULTS: We included 18 patients (mean age 52 years, 82% female). Partial hepatectomy and cyst fenestration reduced median liver volume (4,917 to 2,120 mL). Symptoms, measured with Polycystic Liver Disease Questionnaire, decreased (76.9 to 34.8 points; P < .001) 6 months after surgery; 15/16 symptoms declined after treatment, with the most impact seen on early satiety and dyspnea. Quality of life also improved after surgery: median physical and mental component scales of the 12-Item Short Form Survey and EuroQoL Visual Analog Scale increased (24.9 to 45.7, P = .004; 40.5 to 55.4, P = .02; and 40.0 to 72.5, P = .003). Major complications (grade 4) occurred in 2 patients. There was no procedure-related mortality. CONCLUSION: Partial hepatectomy and cyst fenestration substantially improves symptom burden and quality of life in highly symptomatic polycystic liver disease patients.

Surgical treatment for non-parasitic liver cysts improves quality of life.

BACKGROUND&PURPOSE: Liver cysts occur frequently. Most are harmless, however some carry a significant patient burden. Optimizing treatment strategy is complicated as needs differ between patients. The current study assesses the effect of surgery on quality of life (QoL) of patients with non-parasitic liver cysts. METHODS: A retrospective cohort study of all patients who underwent surgery for non-parasitic liver cysts in three major Dutch medical centers from 1993 to 2017. Patient characteristics and surgery related variables were collected from the electronic patient file. QoL was measured before and after surgery using the EORTC QLQ-C30. Summary scores (SumSc) were calculated and compared to reference values of the general population. Multivariate analysis using logistic regression was performed for identifying outcome related factors. Increase of ≥ 10% in SumSc was defined as clinically relevant. MAIN FINDINGS: Eighty-eight of 132 eligible patients (67%) completed two QoL assessments. Respondents demonstrated significant improvement in the global health status, on all 5 functional scales (all p ≤ 0.005), on all 9 symptom scales after surgery (all p < 0.05), and on SumSc (p < 0.001) to levels similar or better than the general population. Patients with complications demonstrated a significant QoL gain (p < 0.05), and reported a similar postoperative status compared to patients without complications (p = 0.74). QoL gain for patients who underwent open and laparoscopic cyst fenestration were similar (p = 0.08). Multivariate analysis of SumSc found mechanical complaints as significant factor for ≥ 10% SumSc increase (OR 0.11, 95% CI (0.02-0.55). CONCLUSIONS: Surgery is a safe and effective strategy to significantly improve QoL in patients with symptomatic liver cysts.

Quality of Life related to Eruption Hematoma in a Twenty Months Old Infant.

AIM: This paper describes a case of an eruption hematoma in a 20-month-old boy and the impact of this hematoma on the quality of life (QoL) related to oral health of this infant and his family. BACKGROUND: Eruption hematoma is a soft benign cyst that contains blood and overlie a tooth that are about to erupt. Oral health conditions can affect the QoL and bring psychological impacts. CASE REPORT: The proposed treatment was based on oral hygiene instruction, normal diet and massage on the lesion area. A weekly follow-up visits up to the spontaneous regression at the 6 weeks of the lesion was conducted. The impact on QoL was assessed though the Brazilian version of the early childhood Oral Health Impact Scale (B-ECOHIS) questionnaire before and after 2 months of the hematoma regression. CONCLUSION: The presence of eruption hematoma impacted, physically and emotionally, the QoL related to oral health of the child and his family, and this impact decreased when the eruption hematoma disappeared. CLINICAL SIGNIFICANCE: It is important the knowledge of the dentists about eruption cyst/hematoma to make the correct decisions to improve the QoL of their patients and families.

Somatostatin analogues improve health-related quality of life in polycystic liver disease: a pooled analysis of two randomised, placebo-controlled trials.

BACKGROUND: Polycystic liver disease is associated with impaired health-related quality of life (HRQL). Somatostatin analogues reduce hepatomegaly in polycystic liver disease. AIM: To determine whether somatostatin analogues improve HRQL and to identify factors associated with change in HRQL in polycystic liver disease. METHODS: We pooled data from two randomized, double-blind, placebo-controlled trials that evaluated HRQL using the Short-Form 36 (SF-36) in 96 polycystic liver disease patients treated 6-12 months with somatostatin analogues or placebo. The SF-36 contains a summarizing physical and mental component score and was administered at baseline and at the end of treatment. We used random effect models to delineate the effect of somatostatin analogues on HRQL. We determined the effect of demographics, height-adjusted liver volume, change in liver volume, somatostatin analogue-associated side effects with change in HRQL. In patients with autosomal dominant polycystic kidney disease, we estimated the effect of height-adjusted kidney volume and change in kidney volume in relation to HRQL. RESULTS: Physical component scores improved with somatostatin analogues, but remained unchanged with placebo (3.41 ± 1.29 vs. -0.71 ± 1.54, P = 0.044). Treatment had no impact on the mental component score. Large liver volume was independently associated with larger HRQL decline during follow up (-4.04 ± 2.02 points per logarithm liver volume, P = 0.049). In autosomal dominant polycystic kidney disease, patients with large liver and kidney volumes had larger decline in HRQL (5.36 ± 2.54 points per logarithm liver volume; P = 0.040 and -4.00 ± 1.88 per logarithm kidney volume; P = 0.039). CONCLUSION: Somatostatin analogues improve HRQL in symptomatic polycystic liver disease. Halting the progressive nature of polycystic liver disease is necessary to prevent further decline of HRQL in severe hepatomegaly.

Evaluating health-related quality of life in patients with polycystic liver disease and determining the impact of symptoms and liver volume.

BACKGROUND & AIMS: Polycystic liver disease (PLD) follows a progressive course ultimately leading to severe hepatomegaly and mechanical complaints in a subset of patients. It is still unknown to what extent this compromises health-related quality of life (HRQL). Our aim was to determine HRQL in PLD patients and investigate its association with concurrent abdominal symptoms and liver volume. METHODS: Pooled data of 92 severe PLD patients from two randomized clinical trials were used for our cross-sectional analysis. HRQL was assessed using the generic short-form health survey (SF-36) resulting in eight scale scores and the summarizing physical (PCS) and mental component score (MCS). Subsequently, these were compared with the general population. Abdominal symptoms were measured with a standardized, 7-point scale questionnaire in 54 patients. We dichotomized symptoms for absence or presence and compared them with the component scores. Finally, a possible correlation between liver volume and HRQL was explored. RESULTS: Demographics showed severe polycystic livers (mean 4906 ± 2315 ml). PCS was significantly lower compared with the general population (P < 0.001), in contrast with a similar MCS (P = 0.82). PLD patients had statistically significant (P < 0.05) diminished physical functioning, role physical, general health, vitality and social functioning scores. Upper- and lower abdominal pain and dyspnoea were significantly associated with a reduced PCS (P < 0.01). No correlation was found between liver volume and HRQL. CONCLUSION: Polycystic liver disease patients had significantly lower HRQL in the physical dimension compared with the general population. Abdominal pain and dyspnoea had a significant impact on this physical dimension of HRQL.

Sudden death caused by aortic dissection in a patient with polycystic kidney disease.

A 43-year-old man presented at the emergency medical unit with chest pain. The results of a clinical examination were normal, apart from sternum pain (without radiation) on palpation. The patient had no respiratory problems and the pain was relieved by paracetamol. The electrocardiogram, laboratory tests and chest X-ray were normal. However, the man was found dead the next morning. In the autopsy, we noted the presence of haemopericardium, aortic dissection (starting from the vessel's origin and extended to the aortic arch and on through the diaphragm), polycystic kidney disease and liver cysts. In adult autosomal dominant polycystic kidney disease (ADPKD) patients, the main causes of death are ruptured intracerebral aneurysms, coronary artery disease, congestive heart failure, valvular heart disease and ruptured abdominal aortic aneurysms. Aortic dissection is considered to be rare cause of sudden death in ADPKD sufferers. ADPKD can have serious consequences for the vascular system. The families of confirmed ADPKD sufferers must be informed and screened as early as possible, in order to prevent renal and cardiovascular complications.

Idiopathic pigmented free-floating posterior vitreous cyst.

A 28-year-old female who complained of visual symptoms in the right eye was admitted to the ophthalmology clinic. She had been suffering from visual disturbance for about 5 years and her symptoms had become worse during the last 2 months. There was no previous history of trauma, infection or inflammatory disorders. A pigmented vitreous cyst was detected in her ophthalmologic examination. As the patient refused, laser cystotomy could not be performed. The pigmented vitreous cyst was possibly congenital in origin and described as a cystic choristoma from the primitive hyaloid system in the literature. The movements of the cyst, causing only transient blurring of vision, may rarely affect the visual axis. The visual acuity was 20/20 in the affected eye so it was suggested that she should be followed-up periodically for her cyst.

Giant cystic widening of Virchow-Robin spaces: an anatomofunctional study.

We describe 2 patients with unusual white matter cystic dilations, which could correspond to widening of the perivascular spaces. They underwent morphologic MR imaging with tractography, functional MR imaging (fMRI), and neuropsychological evaluation. fMRI examination showed no functional reorganization of cortical areas. Tractography showed an apparent decrease of white matter tract vectors into the regions of concern. Findings of the neuropsychological examination were normal. It seems that even an extensive cystic dilation of white matter does not deteriorate brain function.

Exaggerated crying and tremor with a cerebellar cyst.

The authors describe clinical, neuropsychological, and neuroimaging findings in a 70-year-old man with a cystic lesion in the midline cerebellum. He presented with pathological crying in parallel to a worsening of his ataxia. This report suggests a role for the cerebellum in the regulation of emotional expression.

Dissociating task-set selection from task-set inhibition in the prefrontal cortex.

Patients with focal lesions in the left (n=7) and right (n=4) prefrontal cortex were compared with controls (n=16) in a task-switching experiment using four different, simple spatial tasks. Each of these tasks involved a left-right decision, either regarding an arrow, the word "left" or "right," a circle position, or the direction of a moving line. We compared performance on trials that required rule switches versus rule repetitions (local switch costs) and we compared performance between blocks with bivalent stimuli (two dimensions present) and blocks with univalent stimuli (only one dimension present) to assess global switch costs. Patients with left prefrontal lesions, but not patients with right prefrontal lesions, exhibited increased costs on trials in which the relevant dimension switched (local switch costs), but also on no-switch trials with bivalent stimuli (global costs). We also assessed task-set inhibition in the form of the backward-inhibition effect [increased response times to recently abandoned tasks; Mayr, U., & Keele, S. Changing internal constraints on action: The role of backward inhibition. Journal of Experimental Psychology: General, 129, 4-26, 2000]. Although left frontal patients showed normal inhibition, right frontal patients showed no evidence for inhibition. These results suggest a neurocognitive dissociation between task-set selection and inhibition.

Neurocognitive assessment before and after cranioplasty.

OBJECTIVE: This paper addresses the possible value of neurocognitive tests on the evaluation of patients before and after cranioplasty for large cranial defects. METHODS: In a single patient with a large corrected cranial defect a detailed neurocognitive analysis was performed utilizing the EXIT interview and Cognistat before and after surgery. Planning and development of the surgical prosthesis were based on the pre-operative reconstruction of the head CT and on pre-operative pictures of the patient. Xenon-CT anatomical and quantitative cerebral blood flow (CBF) analysis was performed before and periodically after cranioplasty. FINDINGS: A significant improvement in major cognitive functions was observed after surgery. INTERPRETATION: Cognistat is a detailed neuropsychological battery that permits a better assessment of patients in diverse neurological conditions. The EXIT interview gives a better rapid assessment of cognition not provided by other methods, such as the mini-mental status examination (MMSE). The utilization of these techniques permits a better understanding of long-term outcomes for patients with diverse neurological conditions, including post-cranioplasty patients.

Differential cognitive effects of colloid cysts in the third ventricle that spare or compromise the fornix.

A series of twelve cases, all of whom had received surgery for the removal of a colloid cyst in the third ventricle, was examined on a series of memory tests. The only consistent predictor of poor memory performance that could be detected from MRIs was the presence of bilateral interruption of the fornix, which occurred in three of the subjects. Although these three cases were poor on tests of learning and recall, there was evidence that recognition was less impaired. The subjects were also tested on a set of recognition and concurrent discriminations that closely matched tests given to non-human primates. Clear parallels were found between the apparent effects of fornix damage in these clinical cases and those observed following more selective surgery in non-human primates. These findings not only indicate that fornix damage is sufficient to induce anterograde amnesia but also support the validity of recent animal tests that are thought to capture aspects of episodic memory.

A case of small cerebral cyst and pericentric inversion of chromosome 9 that developed schizophrenia-like psychosis.

A case of schizophrenia-like psychosis (psychotic disorder not otherwise specified according to the DSM-IV criteria) with pericentric inversion on chromosome 9 [inv.(9) (p11; q13)] is reported. In this case, a minor brain anomaly, a small cyst in the left subcortex, was observed on magnetic resonance imaging of the brain. In the clinical course, prominent chronic hallucinations were observed; however, there was no evidence of the disorganization of personality, delusion, and deterioration in level of functioning that are usually seen in schizophrenia. This case and a review of the literature indicate that the pericentric region of chromosome 9 might be a potential areas of interest for the aetiology of psychiatric disorders. The phenotype-karyotype relationship of pericentric inversion on chromosome 9 and its relationship to psychosis are discussed.

L-dopa resistant parkinsonism in an adult woman with a cyst in the posterior fossa.

We report a case of a woman with L-Dopa resistant asymmetrical parkinsonism with a posterior fossa cyst compressing the lower brainstem on MR. She did not show improvement in any of her symptoms after cysto-cardiac derivation. It was not possible to delineate if this was a case of a new malformative syndrome or the coincidence of two different disorders.

Immune responses associated with early survival after peroral infection with Toxoplasma gondii.

After peroral infection with cysts of Toxoplasma gondii, C57BL/6 mice died and A/J mice survived. To better understand the reasons for this difference in survival, host defenses during acute infection were studied: initial portal of entry of T. gondii contributed to susceptibility as more C57BL/6 mice survived after i.p. than peroral infection (p less than 0.001). Susceptible (C57BL/6) mice had more necrosis and inflammation in their brains, livers, and mesenteric lymph nodes than resistant (A/J) mice. Susceptible mice had less IgM antibody to T. gondii (p less than 0.0005) than resistant mice 7 days after infection, but amounts of IgG antibody to T. gondii were similar. Infection reduced percentages of spleen cells with the Lyt-2+ phenotype in susceptible (p less than 0.02) but not resistant mice; infection decreased percentages of spleen cells with the L3T4+ phenotype similarly in both strains of mice. Spleen cells from infected susceptible mice had greater depression in their blastogenic response to Con A (p less than 0.05) and produced significantly more IFN-gamma in culture with (p = 0.009) or without (p less than 0.05) Toxoplasma Ag than spleen cells from infected resistant mice. Infection increased serum levels of IFN-gamma substantially in susceptible but not resistant mice. Lymphocyte IL-2 production was similar in both groups of mice. Peritoneal macrophages from both strains of mice became activated to inhibit or kill T. gondii by 7 days after infection, but Kupffer cells became activated only in susceptible mice. These results indicate that increased resistance to peroral Toxoplasma infection is likely to be mediated by a number of immune responses acting together. They suggest that increased susceptibility may result from inadequately regulated inflammatory responses that increase tissue destruction.

Clinical and neuropsychological results after operative and conservative treatment of arachnoidal cysts of the perisylvian region.

Most supratentorial arachnoid cysts are found in the perisylvian region. They are thin extracerebral fluid filled pouches and occasionally symptoms are caused by space occupation. We have studied 28 cases with these cysts, with follow-up time varying from one to ten years. 8 of the cases presented with a sudden onset of seizures, while 5 cases had a history of seizures dating from childhood. 14 cases underwent surgery, of whom most had histological evidence of previous haemorrhage and some had evidence of subdural haematoma. Surgery was uneventful in all cases regardless of the method used and all had satisfactory outcome. All 28 cases were followed up systematically including intelligence testing and specific testing of memory and other temporal lobe functions. The group scored normal in standardized tests with a distribution similar to the normal population. Results did not show any deficit of function related to the localization of the cyst. Despite of the severity of their initial symptoms, postoperative cases performed just as well at psychological testing. Those cases who continued to experience seizures showed a memory disturbance and seemed to lack initiative. Most cases studied had normal error scores in the dichotic listening examinations. There was no connection between the side of the cyst and a unilateral deficit. The results are discussed.

Asymptomatic large arachnoid cyst of the middle cranial fossa. A clinical and neuropsychological study.

The authors studied a 26-year-old healthy subject in whom a large arachnoid cyst of the right middle cranial fossa was revealed by chance. As there were no subjective or objective neurological findings, we checked the existence of any neuropsychological impairment. In spite of this, the patient obtained a good general performance level and sometimes the hemisphere containing the cyst performed better, in accordance with the patient's handedness.

Neuropsychological and neuroendocrinological disturbances associated with extracerebral cysts of the anterior and middle cranial fossa.

In ten adult patients various clinical signs and symptoms led to the diagnosis of an arachnoid cyst in the anterior and middle cranial fossa. In this study a functional relationship between these cysts and disturbances of higher cognitive processes is described. In addition, neuroendocrinological impairment were caused by arachnoid cysts reaching into the suprasellar cistern. Considering the short medical history of our patients we presumed the disturbances not to be primary, but rather secondary, caused by an expansion of the cysts' volume.