LGG-22. TREATMENT OUTCOME AND LONG-TERM HEALTH DEFICITS OF PATIENTS WITH LOW-GRADE GLIOMA DIAGNOSED DURING THE FIRST YEAR OF LIFE

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

Download Full-text

Comparing Long‐Term Outcomes in Tracheostomy Placed in the First Year of Life

The Laryngoscope ◽

10.1002/lary.29440 ◽

2021 ◽

Author(s):

Jordan R. Salley ◽

Yann‐Fuu Kou ◽

Gopi B. Shah ◽

Romaine F. Johnson

Keyword(s):

First Year ◽

Long Term Outcomes ◽

First Year Of Life

Download Full-text

LGG-06. LONG-TERM OUTCOME OF NEWLY DIAGNOSED LOW GRADE GLIOMA

Neuro-Oncology ◽

10.1093/neuonc/noaa222.391 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii367-iii367

Author(s):

Nongnuch Sirachainan ◽

Attaporn Boongerd ◽

Samart Pakakasama ◽

Usanarat Anurathapan ◽

Ake Hansasuta ◽

...

Keyword(s):

Surgical Management ◽

Progression Free Survival ◽

Low Grade Glioma ◽

Low Grade ◽

Newly Diagnosed ◽

Term Outcome ◽

Long Term Outcome ◽

Common Location ◽

Suprasellar Region

Abstract INTRODUCTION Low grade glioma (LGG) is the most common central nervous system (CNS) tumor in children accounted for 30–50%. Regarding benign characteristic of disease, surgical management remains the mainstay of treatment. However, surgical approach is limited in some conditions such as location at brainstem or infiltrative tumor. Chemotherapy and radiation treatments have been included in order to control tumor progression. The 5-years survival rate is approach 90% especially in patients who receive complete resection. However, the outcome of children with LGG in low to middle income is limited. Therefore, the aim of the study was to determine long-term outcome of children with newly diagnosed LGG. METHODS A retrospective study enrolled children aged <18 years who were newly diagnosed LGG during January 2006- December 2019. Diagnosis of LGG was confirmed by histological findings of grade I and II according to WHO criteria. RESULTS A total of 40 patients, female to male ratio was 1:1.35 and mean (SD) for age was 6.7 (4.0) years. The most common location was optic chiasmatic pathway (42.5%), followed by suprasellar region (25.0%). Sixty percent of patients received at least partial tumor removal. Chemotherapy and radiation had been used in 70% and 10.0% respectively. The 10-year progression free survival was 74.1±11.4% and overall survival was 96.2±3.8%. SUMMARY: Treatment of Pediatric LGG mainly required surgical management, however, chemotherapy and radiation had been used in progressive disease. The outcome was excellent.

Download Full-text

THE NEPHROTIC SYNDROME IN THE FIRST YEAR OF LIFE

PEDIATRICS ◽

10.1542/peds.25.6.967 ◽

1960 ◽

Vol 25 (6) ◽

pp. 967-976

Author(s):

R. A. Parker ◽

Carolyn F. Piel

Keyword(s):

Nephrotic Syndrome ◽

Cortical Area ◽

Clinical Course ◽

Renal Pathology ◽

Electrolyte Imbalance ◽

First Year ◽

Renal Lesions ◽

Susceptibility To Infection ◽

First Year Of Life

The clinical course of nephrosis in five infants with onset of disease before 7 months of age is presented, together with evaluation of renal lesions seen at necropsy. The problems of the management of nephrosis susceptibility to infection and water and electrolyte imbalance were found to be exaggerated by the young age of the patients. The renal pathology observed in these five infants consisted of persistence of immature glomeruli and dilatation of the tubules in the cortical area. Later, the immature glomeruli and associated tubules appear to atrophy and the remaining glomeruli to hypertrophy. Long-term adrenocorticosteroid therapy seems to be contraindicated, not only on the basis of the pathologic changes, but because it greatly exaggerates the problems of management and does not effect a remission of the disease.

Download Full-text