Spontaneous Regression of Tumor in an Optic Pathway Glioma Patient With Diencephalic Syndrome: Case Report and Literature Review

Author(s):  
Rutao Luo ◽  
Tianlei Zhang ◽  
Wei Yang ◽  
Yuanqi Ji ◽  
Hailang Sun ◽  
...  

Abstract Background: Diencephalic syndrome (DS) can cause failure to thrive in pediatrics, which is mostly found in optic pathway gliomas (OPGs) patients. OPGs patients with DS always show a poor outcome. Case presentation: We present the case of an OPG patient with DS who got a spontaneous regression without any treatment. A 6-month-old girl presented with failure to thrive for two months and visual dysfunction with bilateral horizontal nystagmus. MRI demonstrated a 42mm*37mm*36mm enhanced and lobulated lesion located in the sellar region with a clear boundary with surrounding tissues. OPG and DS was diagnosed according to her radiological examination and manifestation. Conservative follow-up was given. There was a spontaneous decrease in tumor size during follow-up. Symptom improves and the patient continues to have a good quality of life despite a moderate dysfunction of her left eye.Conclusions: Conservative observation can be used as a treatment for some OPG patients, body weight may be a marker of the growth of tumor in OPG patients with DS.

PEDIATRICS ◽  
1995 ◽  
Vol 95 (6) ◽  
pp. 807-814 ◽  
Author(s):  
Maureen M. Black ◽  
Howard Dubowitz ◽  
Jacqueline Hutcheson ◽  
Julie Berenson-Howard ◽  
Raymond H. Starr

Objective. To evaluate the efficacy of a home-based intervention on the growth and development of children with nonorganic failure to thrive (NOFTT). Design. Randomized clinical trial. Participants. The NOFTT sample included 130 children (mean age, 12.7 months; SD, 6.4) recruited from urban pediatric primary care clinics serving low income families. All children were younger than 25 months with weight for age below the fifth percentile. Eligibility criteria included gestational age of at least 36 weeks, birth weight appropriate for gestational age, and no significant history of perinatal complications, congenital disorders, chronic illnesses, or developmental disabilities. Children were randomized into two groups: clinic plus home intervention (HI) (n = 64) or clinic only (n = 66). There were no group differences in children's age, gender, race, or growth parameters, or on any of the family back-ground variables. Most children were raised by single, African-American mothers who received public assistance. Eighty-nine percent of the families (116 of 130) completed the 1-year evaluation. Interventions. All children received services in a multidisciplinary growth and nutrition clinic. A community-based agency provided the home intervention. Families in the HI group were scheduled to receive weekly home visits for 1 year by lay home visitors, supervised by a community health nurse. The intervention provided maternal support and promoted parenting, child development, use of informal and formal resources, and parent advocacy. Measurements. Growth was measured by standard procedures and converted to z scores for weight for height and height for age to assess wasting and stunting. Cognitive and motor development were measured with the Bayley Scales of Infant Development, and language development was measured by the Receptive/Expressive Emergent Language Scale. Both scales were administered at recruitment and at the 12-month follow-up. Parent-child interaction was measured by observing mothers and children during feeding at recruitment and at the 12-month follow-up, and the quality of the home was measured by the Home Observation Measure of the Environment 18 months after recruitment. Analyses. Repeated-measures multivariate analyses of covariance were used to examine changes in children's growth and development and parent-child interaction. Analyses of covariance were used to examine the quality of the home. Independent variables were intervention status and age at recruitment (1.0 to 12.0 vs 12.1 to 24.9 months). Maternal education was a covariate in all analyses. When changes in developmental status and parentchild interaction were examined, weight for height and height for age at recruitment were included as covariates. Results. Children's weight for age, weight for height, and height for age improved significantly during the 12-month study period, regardless of intervention status. Children in the HI group had better receptive language over time and more child-oriented home environments than children in the clinic-only group. The impact of intervention status on cognitive development varied as a function of children's ages at recruitment, with younger children showing beneficial effects of home intervention. There were no changes in motor development associated with intervention status. During the study period, children gained skills in interactive competence during feeding, and their parents became more controlling during feeding, but differences were not associated with intervention status. Conclusions. Findings support a cautious optimism regarding home intervention during the first year of life provided by trained lay home visitors. Early home intervention can promote a nurturant home environment effectively and can reduce the developmental delays often experienced by low income, urban infants with NOFTT. Subsequent investigations of home intervention should consider alternative options for toddlers with NOFTT.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Lauren Doig ◽  
Kevin Solverson

Introduction. Delusional and fearful memories after critical illness are observed in up to 70% of patients post critical illness. However, they often go unrecognized after patients leave the intensive care unit (ICU). Case Presentation. A 40-year-old male was admitted to the ICU with community-acquired pneumonia and multiorgan failure requiring mechanical ventilation and renal replacement therapy. He developed protracted delirium and severe ICU-acquired weakness but was eventually discharged home. The patient returned to a follow-up clinic two months post-ICU discharge and revealed that he was suffering anxiety from memories in the ICU of different staff trying to harm and kill him, including being repeatedly suffocated. By providing context to the memories, the patient had significant relief in his anxiety. Conclusions. Intrusive memories contribute to psychological morbidity post critical illness, including posttraumatic stress disorder (PTSD) and reduced health-related quality of life. The majority of critical illness survivors do not share their intrusive or frightening memories, and therefore, most healthcare professionals are unaware of the problems they can pose. Assessment of patients’ memories from the ICU is essential and may create the opportunity to help patients place memories into context and improve psychological morbidities.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 20024-20024
Author(s):  
R. Kebudi ◽  
O. Ayrancı ◽  
S. Tuncer ◽  
I. Ayan ◽  
G. Peksayar ◽  
...  

20024 Background: Optic pathway gliomas (OPG) constitute 1–5% of childhood brain tumors, they can occur in patients with NF1. The aim of this study is to evaluate our patients with OPG through a 16 year period. Methods: We reviewed the medical records of 22 patiens treated between Dec. 1990- Dec. 2006. Results: 12 girls and 10 boys (6 mo-19 yrs, median 5yrs) were diagnosed with OPG. 13 patients had NF1.Two had diencephalic syndrome. In 10 the tumor extended to the hypothalamus.Two had leptomeningeal disease, both died despite therapy.Median follow up was 56 months (6–147 mo). 3 patients had stable disease and were followed without treatment, 6 underwent surgery (4 total tumor resection, 2 ventriculoperitoneal shunt; 5 had no additional therapy). 2 patients (14 and 19 years old ) were treated with radiotherapy and 12 (6 mo-9 yrs) received chemotherapy. 3 patients, with stable disease, remained stable. 4 patients, who had total tumor resection, an one with VPS were followed more than 26 months. In two patients , treated with radiotherapy because of progressive disease, clinical response was attained and remained stable. 12 patients recieved chemotherapy, 11 had vincristine 1.5 mg/m2 and carboplatinum 560 mg/m2 in one day every 28 days. Of these 11 patients, 10 patients had clinical response/stable disease after chemotherapy, 1 died of progressive disease. Two patients had hypersensitivity to carboplatinum after 8 courses. There were no other major side effects of treatment. In a patient who had vincristine, CCNU,CTX and PCZ, 7 months after achievement of stable disease with chemotherapy, progressive disease occured, and died. Conclusion: OPG are rare childhood central nervous system tumors. The rarity ot these tumors and their variable course make assesment and standardization of treatment methods difficult. Conservative management can be suggested for asymptomatic or stable cases. Based on the primary location of the tumor and age of the patient; surgery, radiotherapy and chemotherapy can be used as a single treatment modality or in combination. Treatment should be planned on an individual basis. In young children with progressive disease chemotherapy with vincristine, carboplatinum once a month seems to be effective and provides a good quality of life during treatment. No significant financial relationships to disclose.


2018 ◽  
Vol 31 (9) ◽  
pp. 1053-1056
Author(s):  
Sorin Ioacara ◽  
Elisabeta Sava ◽  
Alexandra Barosan ◽  
Florentina Cojocaru ◽  
Adelina Gutan ◽  
...  

Abstract Background Neonatal diabetes mellitus (NDM) is defined as a monogenic form of diabetes that occurs in the first 6 months of life. As information on diet in NDM patients successfully treated with sulfonylurea is not yet available, we aimed to investigate the hypothesis that a carb-restricted diet is not needed in such cases. Case presentation In this case report, we present a successful implementation of a completely liberalized diet in a young patient with NDM, developmental delay and epilepsy (DEND syndrome), who was also switched to sulfonylurea treatment. The excellent metabolic control during follow-up despite completely ignoring any diet suggests that at least in some patients this approach might work. Conclusions If our proposed hypothesis is also confirmed by other reports, it might add significantly to the quality of life of these patients and broaden the knowledge in this medical field.


2015 ◽  
Vol 29 (4) ◽  
pp. 381-384 ◽  
Author(s):  
A. Chiriac ◽  
Giorgiana Ion ◽  
Z. Faiyad ◽  
I. Poeata

Abstract Intervertebral disc herniation is a common disease that usually requires surgical intervention. However, in some cases, neurological symptoms may improve with conservative treatment. In this article, we present a case with spontaneous regression of extruded lumbar herniated disc correlated with clinical improvement and documented with follow up MRI studies.


2021 ◽  
Vol 3 (3(September-December)) ◽  
pp. e912021
Author(s):  
Aldo José F Da Silva

Hydrocephalus caused by optic pathway glioma (OPG) is most often treated with a ventriculoperitoneal shunt (VPS), but this treatment may present complications such as ascites. Hence, ventriculocisternal shunt is an option worth considering. This article reports a case in which it was decided to place a ventriculocisternal or Torkildsen shunt in a patient with hydrocephalus caused by OPG. Case Report: A 12-year-old girl, amaurotic, with a VPS due to hydrocephalus caused by an OPG, reported to the emergency room with abdominal distension and pain and difficulty in walking. Computed tomography of the abdomen was performed, and a large amount of fluid was evidenced in the peritoneal cavity (ascites). Liver and kidney functions were normal. In the surgical procedure, the distal extremity of the ventriculoperitoneal catheter was exposed, and 3 L of ascites fluid of cerebrospinal origin was drained. After 10 days of antibiotic therapy, a ventriculocisternal or Torkildsen shunt was placed. After 4 years of follow-up, the patient has no complaints. Discussion: Arne Torkildsen was the first to perform a ventriculocisternal shunt in 1937; it is indicated in cases of hydrocephalus with obstruction of the aqueduct or third ventricle. In OPG, increased protein levels in CSF and the use of platinum-based chemotherapy agents would explain the development of ascites after VPS placement. In these cases of hydrocephalus with third ventricle tumor infiltrating the hypothalamus, the ventriculocisternal shunt can be used as an option in specific cases.


2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Martin M. I. Sabandal ◽  
Till Dammaschke ◽  
Edgar Schäfer

Abstract Background Amelogenesis imperfecta is a hereditary malformation showing various manifestations regarding enamel dysplasia. This case report shows a 9-year follow-up after restorative treatment of a 16-year old female patient affected by a hypoplastic type of amelogenesis imperfecta. The caries-free, hypersensitive teeth of the patient were restored by direct dentin adhesive composite restorations performed in total etch technique. Case presentation After rehabilitation the patient reported a marked improvement of the mastication ability and quality of life especially during food intake. Accumulation of plaque was reduced and the ability to perform adequate oral hygiene was improved. During follow-up of 9 years recurring secondary caries and debonding of fillings were recognized and retreated. Conclusions The retrospective assessment exhibits that the performed restorative treatment prolonged the time until further treatment has to be considered, such as prosthetic treatment.


2021 ◽  
Vol 20 (1) ◽  
pp. 34-45
Author(s):  
A. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
L. I. Papusha ◽  
O. I. Bydanov ◽  
E. M. Tarasova ◽  
...  

Diencephalic cachexia (DC ) is a metabolic disorder characterized by a decrease in body weight. DC usually occurs in the presence of glioma brain tumors extended into the optic pathway. These tumors are very aggressive and have poor prognosis.Objective: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .Material and Methods. The study included 264 patients aged 0 to 18 years with an initial diagnosis of OPG s registered in the N.N. Burdenko National Medical Research Center of neurosurgery from 01/01/2003 to 12/31/2015. Patients were divided into two groups: without DC (204 people) and with DC (60 children). Results: neurofibromatosis type I (NFI) was much more common in children without DC , and pilomyxoid histology was much more prevalent in children with DC . Five-year overall survival (OS ) and event-free survival EFS were significantly lower in children with DC than in children without DC (82 ± 5 % and 96 ± 1 %, respectively versus 37 ± 7 % and 62 ± 3 %, respectively). It was found that in the DC group, the OS and EFS rates were significantly lower in girls, in children without NFI, in children without histological verification and in children with pilocytic astrocytomas. It was also found that in the DC group, OS rates were significantly lower in children under 1 year, and EFS rates were significantly lower in children aged more than 12 months. The number of patients without events were significantly higher in the group without DC (p=0.001). The number of deaths in the postoperative period was significantly higher in children with DC (p<0.001). Diabetes insipidus and hyponatremia were significantly more common in patients with diencephalic cachexia, and vision improvement after treatment was significantly more likely to occur in patients without DC .Conclusion. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation. 


2013 ◽  
Vol 29 (10) ◽  
pp. 1941-1945 ◽  
Author(s):  
Maria Elena Cavicchiolo ◽  
Enrico Opocher ◽  
Marco Daverio ◽  
Matteo Bendini ◽  
Elisabetta Viscardi ◽  
...  

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