Recommendations for 46,XX Congenital Adrenal Hyperplasia Across Two Decades: Insights from the North American Differences of Sex Development Clinician Survey.

Several aspects of clinical management of 46,XX congenital adrenal hyperplasia (CAH) remain unsettled and controversial. The North American Disorders/Differences of Sex Development (DSD) Clinician Survey investigated changes, over the last two decades, in clinical recommendations by specialists involved in the management of newborns with DSD. Members of the (Lawson Wilkins) Pediatric Endocrine Society and the Societies for Pediatric Urology participated in a web-based survey at three timepoints: 2003-2004 (T1, n = 432), 2010-2011 (T2, n = 441), and 2020 (T3, n = 272). Participants were presented with two clinical case scenarios-newborns with 46,XX CAH and either mild-to-moderate or severe genital masculinization-and asked for clinical recommendations. Across timepoints, most participants recommended rearing the newborn as a girl, that parents (in consultation with physicians) should make surgical decisions, performing early genitoplasty, and disclosing surgical history at younger ages. Several trends were identified: a small, but significant shift toward recommending a gender other than girl; recommending that adolescent patients serve as the genital surgery decision maker; performing genital surgery at later ages; and disclosing surgical details at younger ages. This is the first study assessing physician recommendations across two decades. Despite variability in the recommendations, most experts followed CAH clinical practice guidelines. The observation that some of the emerging trends do not align with expert opinion or empirical evidence should serve as both a cautionary note and a call for prospective studies examining patient outcomes associated with these changes.

Recommendations for 46,XY Disorders/Differences of Sex Development Across Two Decades: Insights from North American Pediatric Endocrinologists and Urologists.

Clinical decision-making for individuals with 46,XY disorders/differences of sex development (DSD) remains unsettled and controversial. The North American DSD Clinician Survey examines the recommendations of a large group of clinical specialists over the last two decades. Active members of the (Lawson Wilkins) Pediatric Endocrine Society and the Societies for Pediatric Urology were invited to respond to a web-based survey at three different timepoints: 2003-2004 (T1), 2010-2011 (T2), and 2019-2020 (T3). Data from 429 participants in T1, 435 in T2, and 264 in T3 were included in this study. The participants were presented with three XY newborn clinical case scenarios-micropenis, partial androgen insensitivity syndrome, and iatrogenic penile ablation-and asked for clinical management recommendations. The main outcomes assessed included the recommended gender of rearing, surgical decision-maker (parent or patient), timing of genital surgery, and age at which to disclose medical details and surgical history to the patient. For all scenarios, the overwhelming majority recommended rearing as male, including a significant increase across timepoints in those recommending a male gender of rearing for the infant with penile ablation. The proportions recommending female gender of rearing declined significantly across timepoints. In general, most recommended parents (in consultation with the physician) serve as surgical decision-makers, but these proportions declined significantly across timepoints. Recommendations on the timing of surgery varied based on the patient's gender and type of surgery. There has been a shift in recommendations away from the "optimal gender policy" regarding gender of rearing and surgical interventions for patients with XY DSD.

ANTHROPOMETRIC MEASURES AMONG CHILDREN AND TEENS WITH CLASSIC 46,XX CONGENITAL ADRENAL HYPERPLASIA IN RELATION TO IMPROVEMENTS IN DIAGNOSIS AND CARE.

OBJECTIVES: 1) To examine anthropometric changes of patients with classic 46,XX CAH and matched referents; 2) To investigate the impact of improvements in diagnosis and care on growth patterns in these patients by comparing changes in anthropometric parameters before and after CAH consensus guidelines. METHODS: This was a retrospective cohort study nested within three large integrated health-systems. Seventy-six patients with classic 46XX CAH and 1,102 matched referents <21 years of age were identified. Anthropometric measurements including age-specific percentiles for height, weight, and body mass index were examined and compared between groups using linear mixed-effect models. Anthropometric trajectories were explored using latent class analyses (LCA). RESULTS: CAH patients had lower height percentiles than referents at all time points. Differences ranged from 10.7% to 28.4%. After age 5 differences in height were only significant among study participants born before the publication of CAH consensus guidelines. LCA of height detected a "gradual growth increase" pattern in 28% of CAH cases and only 4% of referents, and a "growth stunting" pattern was observed in 13% of CAH cases and 6% of referents. Height percentile measures did not differ in CAH patients with or without evidence of hormonal interventions (growth hormone and/or puberty blockers) used to increase adult height. CONCLUSIONS: There is substantial heterogeneity in growth trajectories of CAH patients. Although stunting may affect CAH patients, advances in diagnosis and care improved anthropometric outcomes in this population. Understanding the disease- and therapy-related mechanisms that explain the different growth patterns requires additional research.

Direct Administration of Chemotherapy and Other Agents into the Fourth Ventricle to Treat Recurrent Malignant Brain Tumors in Children.

Direct administration of chemotherapy and other agents into the fourth ventricle of the brain is a novel approach to treating recurrent malignant posterior fossa brain tumors in children. Candidates for this treatment approach include patients with recurrent medulloblastoma, ependymoma, atypical teratoid/rhabdoid tumor, and potentially other neoplasms that originate in the fourth ventricle or elsewhere in the posterior fossa. In this chapter, the authors first explain the rationale for considering fourth ventricular drug infusions in patients with recurrent malignant posterior fossa tumors. We then summarize the results of translational experiments conducted in piglets and non-human primates that demonstrated safety and favorable pharmacokinetics. These translational experiments led to several pilot human clinical trials, and the results of these trials are reviewed. Finally, currently open clinical trials testing infusion of various agents into the fourth ventricle are discussed, and thoughts about potential future directions are shared.

Progressive vascular tumor in infant: A case report and literature review of PIK3CA vascular malformation.

PURPOSE: Vascular anomalies are classified as either vascular tumors or vascular malformations. Vascular malformations can be difficult to diagnose and treat in the pediatric population and can masquerade as malignant processes. Understanding the genetics behind vascular malformations can lead to identification of specific mutations which can be treated with targeted immunotherapy. METHODS: Our case presents a pediatric patient with progressively enlarging vascular malformation despite multiple surgical resections and systemic medical treatments who underwent genetic evaluation and was found to have PIK3CA mutation. RESULTS: After identification of PIK3CA mutation, our patient was successfully treated with the p110ɑ-specific inhibitor, alpelisib, with both shrinkage of malformation on follow-up imaging as well as gains in her developmental milestones. CONCLUSION: Progressive vascular malformations in the pediatric population can be hard to diagnose and treat and are thought to arise from somatic mutations. Our case highlights a patient with progressive malformation despite multiple surgical resections who was successfully treated with targeted immunotherapy after proper identification of genetic mutation.

Ethnic-Racial Identity and Attitude Change: Assessments of Outgroup and Diversity Attitudes among Adolescents in Sweden.

Outgroup and diversity attitudes are important components of intercultural understanding and well-being. Despite the potential of ethnic-racial identity development as a means to foster positive outgroup and diversity attitudes, little is known about its effectiveness in rapidly diversifying contexts such as Sweden. This pre-registered study filled this gap by examining if adolescents taking part in an intervention focused on ethnic-racial identity exploration, the Identity Project, also reported change in outgroup and diversity attitudes, and whether migration background, education type, and ethnic-racial identity development predicted such change. Twenty-three tenth-grade classes in Sweden (N = 509; Mage = 16.28; SDage = 0.80; 66% female; 51% migration background) participated in the intervention and were assessed in four waves over a period of 26 weeks. Whereas ethnic-racial identity exploration and resolution increased for the intervention group, the adolescents reported no change in outgroup and diversity attitudes when compared to a control group. Increases in ethnic-racial identity exploration and resolution co-varied with increases in attitudes, but only at Time 3. The results do not provide support for the link between ethnic-racial identity development and positive outgroup and diversity attitudes, and challenge the notion of attitude change as a cascading effect of the Identity Project intervention in non-US sociocultural contexts. All aspects of the study were pre-registered on the Open Science Framework platform ( https://osf.io/f5896 ).

Different contexts - different stories: Adolescents' experiences of how ethnicity is addressed in schools and sports and on social media in Sweden.

Ethnicity plays a significant role in adolescents' everyday lives, but there is a limited understanding of adolescents' own experiences with how ethnicity is addressed in different contexts. Three contexts of importance during adolescence are investigated in the present study: schools, social media, and sports. A closer contextual examination has the potential to provide insights into how multiple contexts shape experiences with ethnicity. The aim of the study was to understand more about adolescents' experiences of how ethnicity is addressed in schools, on social media, and in sports. Six focus groups with a total of 21 adolescents (Mage = 14.5, SDage = 0.5, female = 76%) discussed their experiences. Data were transcribed verbatim and analyzed using a close-to-data, inductive thematic analysis. The analysis resulted in three main themes and seven subthemes, indicating that ethnicity was addressed differently in the three studied contexts. For the main theme of how ethnicity was addressed in schools, the subthemes were: Addressing ethnicity is important; Ethnicity is addressed through stereotypes and Everyday racism. The main theme of ethnicity on social media consisted of two subthemes: Sharing ethnic and cultural narratives and Hateful remarks. The main theme of ethnicity in sports also consisted of two subthemes: On equal terms and Clear consequences for racist behaviors. To better understand the multiple contexts, the results are discussed guided by the ecological systems theory. The adolescents highlighted that there are many benefits of addressing ethnicity and that it is important to do so in multiple contexts of adolescent life, just not in the same way. When ethnicity was addressed carelessly, such as through stereotypes or via racism masked as jokes, it had the potential to cause harm. When ethnicity was addressed with reflection, it instead had the potential to build understanding, lead to positive experiences, and provide learning opportunities.

Contrast Enhancement Patterns in Pediatric Glioblastomas.

BACKGROUND AND PURPOSE: Brain tumors are the most common cause of cancer-related deaths among the pediatric population. Among these, pediatric glioblastomas (GBMs) comprise 2.9% of all central nervous system tumors and have a poor prognosis. The purpose of this study is to determine whether the imaging findings can be a prognostic factor for survival in children with GBMs. MATERIALS AND METHODS: The imaging studies and clinical data from 64 pediatric patients with pathology-proven GBMs were evaluated. Contrast enhancement patterns were classified into focal, ring-like, and diffuse, based on preoperative postcontrast T1-weighted magnetic resonance images. We used the Kaplan-Meier method and Cox proportional hazard regression to evaluate the prognostic value of imaging findings. RESULTS: Patients with ring-enhanced GBMs who underwent gross total resection or subtotal resection were found to have a significantly shorter progression-free survival ( P = 0.03) comparing with other enhancing and nonenhancing glioblastomas. CONCLUSIONS: In this study, we analyzed survival factors in children with pediatric glioblastomas. In the group of patients who underwent gross total resection or subtotal resection, those patients with focal-enhanced GBMs had significantly longer progression-free survival ( P = 0.03) than did those with other types of enhancing GBMs (diffuse and ring-like).

Can Craniosynostosis be Diagnosed on Physical Examination? A Retrospective Review.

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures.

The transition to independence and adult care for women with Turner syndrome: Current status and priorities of 1338 women and parents.

Transitioning to adult health care and functioning is crucial for youth and young adults with special health care needs, such as those with Turner syndrome (TS). The International Turner Syndrome Consensus Group developed clinical practice guidelines to improve this transition. This study sought to evaluate how effectively they have been implemented and whether they align with the priorities of women with TS and families of girls and women with TS. A web-based survey was offered to those affiliated with major TS support organizations, with 1338 successfully responding (n = 543 women [≥18 years]; n = 232 parents of a woman [≥18 years]; and n = 563 parents of a girl with TS [<18 years]). Findings demonstrated that while most women transitioned to adult primary care providers, follow-up with key specialists was lacking. Women and families prioritized flexibility in appointments and designating one provider to oversee all their TS-related care. They identified health care coverage as a barrier to receiving care. The transition process to adult care and independence commonly occurred after girls become legal adults. Together, these findings serve to inform strategies to improve the delivery of transitional care for girls and women with TS.

Differences/Disorders of Sex Development: Medical Conditions at the Intersection of Sex and Gender.

Defined as congenital conditions in which development of chromosomal, gonadal, or anatomic sex is atypical, differences or disorders of sex development (DSDs) comprise many discrete diagnoses ranging from those associated with few phenotypic differences between affected and unaffected individuals to those where questions arise regarding gender of rearing, gonadal tumor risk, genital surgery, and fertility. Controversies exist in numerous areas including how DSDs are conceptualized, how to refer to the set of conditions and those affected by them, and aspects of clinical management that extend from social media to legislative bodies, courts of law, medicine, clinical practice, and scholarly research in psychology and sociology. In addition to these aspects, this review covers biological and social influences on psychosocial development and adjustment, the psychosocial and psychosexual adaptation of people born with DSDs, and roles for clinical psychologists in the clinical management of DSDs.

Choroid plexus cauterization to treat inadequate abdominal cerebrospinal fluid absorption: case report.

INTRODUCTION: Inadequate absorption of cerebrospinal fluid (CSF) in the setting of high CSF production is a relatively rare cause of shunt malfunction. CASE REPORT: We present the unique case of a 3-year-old boy who developed sterile ascites and abdominal distension in a delayed fashion after shunt placement. The shunt was externalized, and the patient was noted to have high CSF output. Bilateral choroid plexus cauterization resulted in a significant decrease in CSF production and enabled the shunt to be re-inserted into the abdomen.

Longitudinal associations between digital media use and ADHD symptoms in children and adolescents: a systematic literature review.

Previous reviews have often shown a link between digital media ADHD symptom levels. However, longitudinal studies are needed to find stronger evidence of a causal effect as well as to determine the direction of effects. The aim of the present review (PROSPERO CRD42021262695) was therefore to provide a systematic review of studies meeting the following inclusion criteria: (1) include longitudinal data investigating associations between digital media (i.e., gaming and social media) and later ADHD symptoms or vice versa, (2) be published within the past 10 years (i.e., 2011 until June 2021), (3) be published in a peer-reviewed journal in English, and (4) include children or adolescents (age 0-17 years). After a systematic search in the Web of Science and PsycInfo databases, we included 28 studies, all with adequate or high quality. Results showed support for reciprocal associations between digital media and ADHD symptoms, with associations being more consistent for problematic use of digital media than for screen time. Thus, children with ADHD symptoms appear more vulnerable to developing high or problematic use of digital media (i.e., selection effects), and digital media also have effects on later ADHD symptom levels, either because of specific characteristics of digital media or because of indirect effects on, for example, sleep and social relations (i.e., media effects). However, it should be emphasized that further studies investigating potential moderators and mediators are needed if we are to better understand the complex associations between digital media and ADHD symptom levels.

Back to the Beginning: An Algorithmic Approach to Neonatal Myelomeningocele Repair.

INTRODUCTION: Myelomeningoceles are formed by prenatal failure of neural tube closure and can cause hydrocephalus, motor abnormalities, and developmental delay. Although small defects are amenable to primary closure, larger defects often require complex reconstruction. Our goal was to identify factors associated with postoperative soft tissue complications and develop a systematic approach for myelomeningocele closure. METHODS: A retrospective review was performed at the Children's Memorial Hermann Hospital from January 2013 to January 2019. Patients were identified using International Classification of Diseases, Ninth Revision/Tenth Revision , codes for myelomeningocele. Cohorts were stratified by reconstruction type and defect location. Primary outcomes were incidence of complications including cerebrospinal fluid leak, superficial and deep infection, and wound dehiscence. In addition, we developed an algorithm to standardize closure approach for patients with myelomeningoceles. RESULTS: A total of 172 patients with myelomeningocele were identified with 73 patients undergoing postnatal repair. Overall, 72% of defects were >5 cm. Defects were in the lumbar (9%), sacral (8%), and junctional (83%) regions. Overall, 30.1% patients underwent lumbar myofascial repair with 39.7% requiring fasciocutaneous flaps. Larger defects (>5 cm) were more likely to be closed with complex fasciocutaneous flaps (82.8% vs 66.0%, P = 0.11). No significant differences were observed in complication rates. CONCLUSIONS: In this series, patients with larger myelomeningoceles appear to benefit from complex flap closure. We propose a 5-layer closure for patients with myelomeningocele including the routine use of a myofascial layer. Cutaneous closure technique should be tailored based on specific defect characteristics as outlined in our algorithm. This approach streamlines myelomeningocele repair while optimizing outcomes and decreasing downstream complications.

Changes in Size and Demographic Composition of Transgender and Gender Non-Binary Population Receiving Care at Integrated Health Systems.

OBJECTIVE: To examine temporal changes in the number and demographic composition of transgender/gender non-binary (TGNB) population using data from integrated health care systems. METHODS: Electronic health records from Kaiser Permanente health plans in Georgia and Northern and Southern California were used to identify TGNB individuals, who sought care from January 2006 to December 2014, and the data were analyzed by year, site, age, and sex assigned at birth. RESULTS: In 2006, the number of TGNB people (and corresponding 95% CI) per 100 000 population were 3.5 (1.9, 6.3) in Georgia, 5.5 (4.8, 6.4) in Southern California, and 17 (16, 19) in Northern California. In 2014, these frequencies increased to 38 (32, 45), 44 (42, 46), and 75 (72, 78) per 100 000 population, respectively. When analyzed by age, the most rapid increase was observed among persons 18 to 25 years old, and this increase accelerated after 2010. The ratio of transmasculine to transfeminine persons also changed from 1:1.7 in 2006 to 1:1 in 2014 overall and from 1:1 in 2006 to 1.8:1 in 2014 among persons <18 years of age. CONCLUSION: This analysis confirms previous observations that the proportion of TGNB people is growing, especially among young adults. The composition of the TGNB population is also changing from predominantly transfeminine to roughly 1:1 overall and to predominantly transmasculine in children and adolescents.

Spontaneous resolution of large subdural hematoma with midline shift: a case report.

Subdural hematomas with mass effect and midline shift are typically offered urgent surgical evacuation. The authors report a 6-month-old baby who was referred for macrocephaly and found to have a large subdural hematoma with midline shift. Given her lack of symptoms and normal neurological examination, the patient was observed without neurosurgical intervention. She remained asymptomatic, and serial brain imaging showed progressive decrease of the subdural hemorrhage and complete resolution of midline shift. This case is presented to alert neurosurgeons that observation of a large subdural hematoma may rarely be appropriate in babies with open fontanelles.

"I Tell Them What I Can Feel and How Far My Legs Can Bend": Optimizing Sexual Satisfaction for Women With Spina Bifida.

BACKGROUND: There is a high reported rate of sexual dysfunction among women with spina bifida, but little is known about the etiology of this or how sexual satisfaction could be improved. AIM: To identify, through the words of women with spina bifida, perceived causes of diminished sexual satisfaction and recommendations to optimize partnered sexual encounters. METHODS: In this qualitative study, we conducted semi-structured individual interviews with 22 women with spina bifida (median age 26.5 years, range 16-52 years) who have had a romantic partner. Using Grounded Theory, interviews were independently coded by 3 reviewers. Disagreements were resolved by consensus. OUTCOMES: We identified overlapping themes of issues women experienced during sexual intimacy and strategies they learned to improve sexual encounters. RESULTS: 7 salient themes emerged from the data: (i) fear of rejection with resulting difficulty setting boundaries and the risk of coercion; (ii) conflict between spontaneity and self-care in sexual encounters; (iii) worry about incontinence during sex; (iv) trial and error in learning optimal sexual positions; (v) decreased genital sensation; (vi) safety considerations; and (vii) sharing advice with other women with spina bifida. CLINICAL IMPLICATIONS: As sexual satisfaction is influenced by physical features as well as psychological, interpersonal, and sociocultural factors, optimizing sexual satisfaction of women with spina bifida is best managed with a holistic approach utilizing a biopsychosocial model. STRENGTHS & LIMITATIONS: The sample included women with a diverse range of functional impairments. Women were forthright with their comments and thematic saturation was reached. Recruitment was primarily from a single Midwestern institution, which may have limited sampled perspectives. CONCLUSION: While women with spina bifida encounter challenges during sexual encounters, strategies focused on improving communication with partners and addressing specific physical considerations can potentially enhance their sexual experiences. Streur CS, Schafer CL, Garcia VP, et al. "I Tell Them What I Can Feel and How Far My Legs Can Bend": Optimizing Sexual Satisfaction for Women With Spina Bifida. J Sex Med 2020;17;1694-1704.

Outcomes and prognostic factors of pediatric patients with a Glasgow Coma Score of 3 after blunt head trauma.

PURPOSE: This study aims to assess outcomes of pediatric patients with blunt traumatic brain injury (TBI) with a presenting Glasgow Coma Score (GCS) of 3. METHODS: After local institutional review board approval, we identified patients ages 0 to15 years with blunt TBI and a reported GCS of 3 between 2007 and 2017 from a pediatric level 1 trauma center prospective registry. Exclusion criteria were cardiac death on arrival and penetrating injury. We recorded clinical variables from patients with a non-pharmacologic GCS of 3 and pupillary exam documented by a neurosurgical attending or resident. The original Glasgow Outcome Scale (GOS) was used to compare with other studies. Importance of variables to survival was calculated. RESULTS: A total of 88 patients (mean age 6.9 years) were included with a mortality rate of 68%. Twelve percent had a poor long-term outcome (GOS 2 or 3) while 20% had a good long-term outcome (GOS 4 or 5). Median follow-up was 1.8 years. Initial group comparison revealed patients in group 1 (survivors) had less hypotension on arrival (14% SBP < 90 mmHg vs. 66%, p < 0.0001), higher temperatures on arrival (36.3 °C vs 34.9 °C, p = 0.0002), lower ISS (29.7 vs 39.5, p = 0.003), less serious injury to other major organs (34% vs 61%, p = 0.02), more epidural hematomas (24% vs 7%, p = 0.04), and less evidence of brain ischemia on CT (7% vs 39%, p = 0.002) or brainstem infarct, hemorrhage, or herniation (0% vs 27%, p = 0.002). Differences between the 2 groups in age, sex, race, MOI, AIS score, presence of midline shift > 5 mm, or time from injury to hospital arrival or time to surgery were not statistically significant. Classification tree analysis showed that the most important variable for survival was pupillary exam; mortality was 92% in presence of bilateral, fixed dilated pupils. The relative importance of initial temperature, MOI, and hypotension to survivability was 0.79, 0.75, and 0.47, respectively. CONCLUSION: Twenty percent of our pediatric non-pharmacologic GCS 3 cohort had a good functional outcome. Lack of bilaterally fixed and dilated pupils was the most important factor for survival. Temperature, MOI, and hypotension also correlated with survival. The data support selective aggressive management for these patients.

Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases.

INTRODUCTION: We report 2 cases of medulloblastoma maturing into gangliocytoma after receiving multimodal therapy. Here we present 2 cases of diagnosed medulloblastoma which on re-resection were noted to be gangliocytoma without heterogeneity, which is an extremely rare occurrence. CASE PRESENTATION: The first patient, an 11-year-old boy diagnosed with high-risk (non-WNT, non-SHH) medulloblastoma, was treated with near-total surgical resection followed by craniospinal radiation therapy with weekly vincristine. He then received maintenance chemotherapy with vincristine, cyclophosphamide, and cisplatin. On surveillance MR imaging studies residual tumor in the lateral aspect of the tumor bed was noted to be slowly growing, eliciting gross-total resection of the residual tumor. Histopathology showed benign gangliocytoma without residual medulloblastoma. The second patient, a 3-year-old girl, was diagnosed with medulloblastoma, desmoplastic nodular variant. She was initially treated with gross total resection and chemotherapy with etoposide, carboplatin, and high-dose methotrexate. At 4 months off therapy, she was noted to have local recurrence along the resection cavity. Second-line therapy was started with irinotecan and temozolomide, but MRI assessment during treatment showed further disease progression. She then received craniospinal radiation. Eleven months off therapy, further radiographic progression was noted, and the patient underwent second-look surgery, with pathology showing gangliocytoma and treatment-related gliosis. DISCUSSION/CONCLUSION: The maturation of medulloblastoma into a ganglion cell-rich lesion is very rare, with few well-characterized previous reports. Given the rare nature of this entity, it would be of great value to understand the process of posttreatment maturation and the genetic and treatment factors which contribute to this phenomenon.