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The study reviews 77 MMA-related hydrocephalus cases among 1671 hospitalized hydrocephalus patients (June 2012 to March 2022), including clinical characteristics, surgical details, and PedsQL 4.0 assessments. Results identify common manifestations, mutation patterns, and VPS revision risks. Findings define timing cutoffs to reduce complications and support better quality of life.",{"@graph":69,"@context":126},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/hydrocephalus-caused-by-methylmalonic-acidemia-clinical-characteristics-optimal-timing-of-surgical-intervention-and-health-related-quality-of-life/452595/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/hydrocephalus-caused-by-methylmalonic-acidemia-clinical-characteristics-optimal-timing-of-surgical-intervention-and-health-related-quality-of-life/452595.png","ImageObject",300,407,{"name":92,"@type":93},"OmBimo","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-05","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118,122],{"name":109,"@type":110,"acceptedAnswer":111},"What was the purpose of the study?","Question",{"text":112,"@type":113},"To evaluate the clinical characteristics and health-related quality of life of infants with hydrocephalus caused by MMA and to determine the optimal timing of surgery.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How was health-related quality of life assessed?",{"text":117,"@type":113},"Health-related quality of life was measured using the Pediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales.",{"name":119,"@type":110,"acceptedAnswer":120},"Which findings relate to surgical timing and revision risk?",{"text":121,"@type":113},"The study identifies an Evans index cutoff of 0.45 and suggests optimal cutoffs for duration of drug treatment and age at surgery; VPS revision cases had shorter drug treatment duration and occurred at younger ages, with lower psychosocial and school functional scores.",{"name":123,"@type":110,"acceptedAnswer":124},"What is the study’s conclusion about optimal surgical timing?",{"text":125,"@type":113},"For patients with hydrocephalus caused by MMA, surgery is suggested after Evans index exceeds 0.45, drug treatment lasts more than 1 month, and surgical treatment occurs after age 4.5 months to reduce complications and improve quality of life.","https://schema.org",{"og:url":83,"og:type":128,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":130,"canonical":83},"index,follow",{"doc_id":132,"site_id":62},452595,1791382977,{"code":4,"msg":5,"data":135},{"doc_id":132,"user_id":136,"nickname":92,"user_avatar":137,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":138,"file_id":139,"file_url":140,"file_type":141,"file_size":142,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":52,"language":143,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":144,"faqs":145,"seo_title":146,"seo_description":67,"update_tm":147,"read_time":148,"preview_page_count":4},962090893581,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","CLINICAL RESEARCH  \nHydrocephalus Caused by Methylmalonic Acidemia: Clinical Characteristics, Optimal Timing of Surgical Intervention and Health-Related Quality of Life  \nFaliang Zhou, MD ‡*, Yixuan Huo, MD‡*, Yu Li, MD§, Lixue Shen, MD§, Lili Liu, MD‡, Hongxin Yao, MD§, Rui Zhang, MD‡*, Xinlin Hou, MD‡*  \n‡Department of Pediatrics, Peking University First Hospital, Beijing, China; §Department of Pediatric Surgery, Peking University First Hospital, Beijing, China.  \n*Faliang Zhou, Yixuan Huo, Rui Zhang, and Xinlin Hou contributed equally to this work.  \nCorrespondence: Xinlin Hou, MD, Department of Pediatrics, Peking University First Hospital, No.1 courtyard, Xi’anmen St, Xicheng District, Beijing 100034, China.  \nEmail: [xinlin.hou@pkufh.com](xinlin.hou@pkufh.com); Rui Zhang, MD, Department of Pediatrics, Peking University First Hospital, No.1 courtyard, Xi’anmen St, Xicheng District, Beijing 100034, China.  \nEmail: [ansonzr@163.com](ansonzr@163.com)  \nReceived, October 24, 2024; Accepted, March 19, 2025; Published Online, June 20, 2025.  \nNeurosurgery 98:432–441, 2026 [https://doi.org/10.1227/neu.0000000000003584](https://doi.org/10.1227/neu.0000000000003584)  \n[Copyright](Copyright) © [2025 The Author](2025 The Author)([s](s)) [.](.) Published by Wolters Kluwer Health, Inc. on behalf of the Congress of Neurological Surgeons. This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.  \nBACKGROUND AND OBJECTIVES: Hydrocephalus is a rare and potentially fatal complication of methylmalonicacidemia (MMA) and is associated with a poor prognosis, requiring prompt clinical decision making. The aim of our study was to evaluate the clinical characteristics and quality of life of infants with hydrocephalus caused by MMA and to determine the optimal timing of surgery.  \nMETHODS: This is a retrospective observational study. We reviewed 77 MMA-related hydrocephalus cases from 1671 hydrocephalus patients admitted between June 2012 and March 2022. Data collected included clinical characteristics, surgical data, and health-related quality of life assessed using the Pediatric Quality of Life Inventory (PedsQL􀀁) 4.0 generic core scales.  \nRESULTS: The most common clinical manifestations were developmental retardation, poor appetite, and altered consciousness. The most frequent mutation was c.609G>A in MMACHC gene (70%). Sixty-six patients (85.7%) underwent ventriculoperitoneal shunting with higher Evans index than the non-VPS group (P \u003C .001) . An Evans index cutoff point of 0.45 was identified. The non-VPS group scored better on all PedsQL􀀁 domains. Of the 66 cases in the ventriculoperitoneal shunting group, 19 (24.7%) needed revision surgery. They had a shorter duration of drug treatment (P =.002) and underwent surgery at a younger age (P = .049) than those in the nonrevision group. Optimal cutoff points were duration of drug treatment >1 month and age of surgical treatment >4.5 months. The revision group had lower psychosocial health and school functional scores.  \nCONCLUSION: Our study provides insights into the clinical profile and health-related quality of life of patients with hydrocephalus caused by MMA. Those with severe hydrocephalus had lower PedsQL􀀁 scores and need surgery. The optimal timing for surgery in these patients is Evans index >0.45, duration of drug treatment >1 month, and age of surgical treatment >4.5 months after birth to reduce surgical complications and improve quality of life.  \nKEY WORDS: Methylmalonic acidemia, Hydrocephalus, Health-related quality of life, Timing of surgical intervention  \nM  \nethylmalonic acidemia (MMA) is a genetic metabolic disorder that leads to the accumulation of methylcitric acid.1 It is the most common organic aciduria ","cbCaiexJsZEU6CqP","https://ap.wps.com/l/cbCaiexJsZEU6CqP","pdf",1203800,"English","# Background and Objectives\n# Methods\n# Results\n# Conclusion\n# Key Words","[{\"question\":\"What was the purpose of the study?\",\"answer\":\"To evaluate the clinical characteristics and health-related quality of life of infants with hydrocephalus caused by MMA and to determine the optimal timing of surgery.\"},{\"question\":\"How was health-related quality of life assessed?\",\"answer\":\"Health-related quality of life was measured using the Pediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales.\"},{\"question\":\"Which findings relate to surgical timing and revision risk?\",\"answer\":\"The study identifies an Evans index cutoff of 0.45 and suggests optimal cutoffs for duration of drug treatment and age at surgery; VPS revision cases had shorter drug treatment duration and occurred at younger ages, with lower psychosocial and school functional scores.\"},{\"question\":\"What is the study’s conclusion about optimal surgical timing?\",\"answer\":\"For patients with hydrocephalus caused by MMA, surgery is suggested after Evans index exceeds 0.45, drug treatment lasts more than 1 month, and surgical treatment occurs after age 4.5 months to reduce complications and improve quality of life.\"}]","Hydrocephalus Caused by Methylmalonic Acidemia - Clinical Characteristics, Optimal Timing of Surgical Intervention and Health-Related Quality of Life | PDF",1790737456,25]