Epidemiologic Assessment of Craniosynostosis in Mississippi's Pediatric Population from 2015 to 2020.

OBJECTIVES: This study aimed to report geographic and demographic patterns of patients with craniosynostosis (CS) treated at Children's of Mississippi, the state's only American Cleft Palate-Craniofacial Association-approved craniofacial team. METHODS: Patients with CS were treated at a tertiary pediatric hospital cared for by craniofacial surgeons and neurosurgeons from 2015 to 2020. Demographic, geographic, and CS diagnosis details, including sex, gestational age, race, ethnicity, insurance status, and affected cranial suture type(s), number, and associated syndromic diagnosis were collected, including birth county and total live births from state data. Significant differences between prevalence of CS in four regions of Mississippi were examined using two-tailed t tests (P < 0.05). RESULTS: Among 222,819 live births in Mississippi between 2015 and 2020, 79 pediatric patients presented to Children's of Mississippi with CS, with an overall incidence of 0.355/1000 live births. Most cases were nonsyndromic CS (82%, n = 65) affecting a single major cranial suture (81%, n = 64). The overall incidence of CS was higher in the coastal and central regions compared with northeast Mississippi, at 0.333 and 0.527 vs 0.132/1000 live births (P = 0.012 and P = 0.004), respectively. CONCLUSIONS: Results from this study suggest regional patterns of CS in Mississippi, which may reflect actual incidence patterns or proximity to Children's of Mississippi. Further study could reveal regional differences in risk factors underlying CS incidence or access to specialized CS care for different regions in the state. This will lead to opportunities for institutional outreach to decrease the burden of CS care in Mississippi.

Posterior Vault Distraction in the Acute Setting.

ABSTRACT: Posterior vault distraction osteogenesis (PVDO) has been shown to resolve acute intracranial hypertension (AIH) while carrying an acceptable perioperative morbidity profile. PVDO has been associated with symptomatic improvement and fewer surgeries in those requiring ventriculoperitoneal shunts. The authors' experience using PVDO as an acute intervention is presented, demonstrating its safety and efficacy for management of AIH. Four cases of children with craniosynostosis that underwent PVDO in the acute setting are outlined. All patients presented with papilledema and symptoms of AIH. One patient with slit ventricle syndrome (SVS) presented with a nonfunctioning shunt following multiple shunt revisions. No intraoperative complications during distractor placement or removal were reported. Distraction protocol for all patients began on postoperative day 1 at 1-2 mm per day, resulting in an average total distraction of 30.25 mm. For the 3 cases with no shunt, the average length of stay was 7 days. As part of the planned treatment course, the patient with SVS required externalization of the shunt during distraction followed by early distractor removal and planned shunt replacement. One case of surgical site infection (in an immunocompromised patient) required premature distractor removal during the consolidation period. Computed tomography (CT) in all patients indicated increased intracranial volume following distraction, and symptomatic improvement was reported. Six-month follow-up showed resolution of papilledema in all patients. The authors' experience using PVDO in the acute setting is reported, alongside a review of current literature, in order to provide supporting evidence for the efficacy of PVDO as a tool for resolving AIH.

Mechanical Thrombectomy in Pediatric Large Vessel Occlusions Before Cerebrovascular Maturity: A Case Report and Technical Note.

Pediatric arterial ischemic stroke is a rare but increasingly acknowledged disorder. Large vessel occlusions in this population have been treated off-label with endovascular thrombectomy. However, there is limited evidence to guide management. Small children, before the age of five when the cerebrovasculature reaches adult size, present additional challenges. We report the case of cardioembolic basilar occlusion in a two-year-old and the technical details of endovascular management, currently lacking in published literature. We employed a 5 French slender sheath, typically used for radial access, as a femoral short sheath. We accessed the dominant vertebral artery with a 5 French intermediate catheter, navigated with a typical 0.027-inch microcatheter and 0.014-inch microwire, and performed direct aspiration thrombectomy of the basilar clot. No closure device was employed. The patient had a near-complete and durable recovery. Small children present additional challenges for the endovascular management of stroke. Pre-procedural imaging can be used to design an aspiration-capable system appropriate for the child's size. Endovascular thrombectomy in children is feasible with some modifications to adult protocols.

Urinary Tract Infection and Progression to Pyelonephritis: Group B Streptococcus versus E. coli.

Objective Group B Streptococcus (GBS) colonization of the lower urinary tract in pregnancy is associated with severe infections such as chorioamnionitis, endometritis, and pyelonephritis. The objective of this study was to compare rates of progression to pyelonephritis between GBS and Escherichia coli lower urinary tract infections (LUTIs), as well as compare infectious and obstetric morbidity secondary to these pathogens. Study Design Retrospective cohort of pregnant women with LUTIs (asymptomatic bacteria or acute cystitis [AC]) from a single health system between July 2013 and May 2019. Demographic, infectious, antepartum, and intrapartum data were abstracted from medical records of women with GBS or E. coli LUTI. The primary outcome was progression to pyelonephritis. Secondary outcomes included pyelonephritis-related anemia, sepsis, pyelonephritis length of stay (LOS), median gestational age (GA) at delivery, preterm delivery, and low birth weight (LBW). Logistic regression was used to calculate the adjusted odds of the primary outcome. Results Of 729 pregnant women with urinary colonization, 433 were culture positive for one of the aforementioned bacteria, with 189 (43.6%) having GBS and 244 (56.4%) having E. coli. Women with E. coli were more likely to be younger, use tobacco, have a history of AC, and have a history of preterm birth. Rates of progression to pyelonephritis were markedly higher with E. coli (15.6%) than with GBS (1.1%; p < 0.001). Median LOS for pyelonephritis and pyelonephritis-related morbidities did not differ. Median GA at delivery, preterm delivery, and LBW rates also did not differ. In adjusted analysis, controlling for history of AC, insurance status, tobacco use, prior preterm birth, primary infection type, and maternal age, women with GBS LUTI had markedly decreased odds of developing pyelonephritis in pregnancy compared with those with E. coli (adjusted odds ratio: 0.04, 95% confidence interval: 0.01-0.28). Conclusion Escherichia coli infections progress to pyelonephritis in pregnancy at markedly higher rates than GBS, although obstetric outcomes are similar.

Prenatal Vaccination Patterns among Birthing Individuals with History of Preterm Birth in the Pre- and Post-COVID Era.

OBJECTIVE: This study aims to explore vaccination acceptance among individuals with a history of preterm birth between March and June during the pre-COVID (2019), early-COVID (2020), and late-COVID (2021) periods. STUDY DESIGN: This is a cross-sectional, retrospective cohort study of pregnant individuals with a history of preterm birth (<37 weeks' gestation) who initiated care of a subsequent pregnancy during pre-COVID (March-June 2019), early-COVID (March-June 2020), or late-COVID (March-June 2021). The primary outcome of interest was vaccination status for influenza, Tdap, and COVID-19 vaccines. Fisher's exact and chi-square tests were used to investigate association between vaccination status and time periods, race/ethnicity, and insurance. RESULTS: Among 293 pregnancies, influenza vaccination rate was highest in early-COVID (p < 0.05). There was no statistically significant difference in Tdap or COVID-19 vaccination between time periods. COVID-19 vaccination was highest in individuals with private insurance (p < 0.05). There was no statistically significant difference in vaccination status by race/ethnicity. CONCLUSION: In this study on high-risk pregnant individuals, the majority of our cohort remained unvaccinated against COVID-19 into the late-COVID period. Additionally, their influenza vaccination rates were greater than the national average in early-COVID and substantially lower than the national average in late-COVID. This shift in influenza vaccination acceptance may have been sparked by COVID-19 vaccine distribution beginning in January 2021 leading to overall vaccination hesitancy. Standardized guidelines and counseling concerning prenatal safety in recommended immunizations may serve as important tools of reassurance and health promotion. KEY POINTS: · Maternal infections during pregnancy are a risk factor for preterm birth.. · High-risk cohort had low influenza vaccination post-COVID possibly due to COVID-19 vaccine hesitancy.. · Vaccination education may be a uniquely important tool among high-risk pregnant patients..

Delta variant neutralizing antibody response following maternal COVID-19 vaccination.

The Delta pandemic wave saw increased maternal morbidity and mortality compared to prior viral strains. COVID-19 vaccination in pregnancy elicits detectable levels of neutralizing antibodies to SARS-CoV-2 wild type and SARS-CoV-2 Delta variants in both maternal and neonatal samples at delivery. Our results demonstrate the effectiveness of vaccination which confers an immunological response against newer, and potentially more dangerous, viral variants.

Factors Associated with Guideline Concordant Clinician Counseling about Low-Dose Aspirin to Prevent Preeclampsia in Nulliparous Patients.

OBJECTIVE: The primary aim of this study was to examine the United States Preventative Services Task Force (USPSTF) guidelines concordant low-dose aspirin (LDA) counseling and factors associated with counseling in nulliparous birthing individuals. STUDY DESIGN: We conducted a retrospective cohort study of nulliparous birthing individuals who delivered between January 1, 2019 and June 30, 2020 and received prenatal care at the Duke High Risk Obstetrical Clinics (HROB). All nulliparous patients over 18 years old who established or transferred care to HROB by 16 weeks, 6 days were included in the analysis. We excluded patients with more than two previous first-trimester pregnancy losses, multiple gestation, a known contraindication to LDA, initiation of LDA prior to their prenatal care, or documented medical history of coagulation disorder. Bivariate associations between demographic/medical characteristics and our primary outcome, receipt of counseling (yes/no), were assessed using two-sample t-tests for continuous variables and chi-square or Fisher's exact test for categorical variables. Factors significantly associated with the primary outcome (p < 0.05) were entered into the multivariable logistic regression model. RESULTS: Among 391 birthing individuals included in the final analysis cohort, 51.7% of eligible patients received guideline consistent LDA counseling. Factors associated with increased odds of LDA counseling were advanced maternal age (adjusted odds ratio [aOR]: 1.05, 95% confidence interval [CI]: 1.01-1.09), Black race compared with White race (aOR:1.75, 95% CI: 1.03-2.98), chronic hypertension (aOR: 4.17, 95% CI: 1.82-9.55), and obesity (aOR: 5.02, 95% CI: 3.12-8.08). CONCLUSION: Approximately half of all nulliparous birthing individuals had appropriately documented LDA counseling. The USPSTF guidelines on LDA for preeclampsia risk reduction are complex, which may lead to ineffective provider adherence. Efforts to simplify guidelines and improve LDA counseling are vital to ensuring this low-cost, evidence-based preeclampsia prevention is used in a consistent and equitable manner. KEY POINTS: · A total of 51.7% of eligible patients received guideline consistent LDA counseling.. · Advanced maternal age , body mass index > 30, Black race, and chronic hypertension associated with increased odds of counseling.. · Among patients most likely to be counseled, high numbers did not receive LDA counseling..

Squamosal Craniosynostosis Associated with Rickets.

Rickets results from defective bone mineralization, leading to skeletal deformities. Among those deformities, rickets has been associated with craniosynostosis, the premature closure of cranial sutures. Most of these patients have fusion of major sutures. Rarely, squamosal craniosynostosis in association with rickets has been described. Squamosal craniosynostosis is noted as lacking a definitive head abnormality and difficult visualization on standard imaging modalities, leading to poor recognition. Careful attention should be given to rickets patients to monitor for these unusual suture closures. Additionally, craniosynostosis could be a presenting feature of rickets, and further rickets evaluation of the patient is indicated.

Pregnant individuals' information needs and intention to vaccinate their children with routine and COVID-19 vaccines: Findings from a cross-sectional survey.

OBJECTIVE: The current study assessed pregnant individuals' intention to accept routine and COVID-19 vaccines for their baby after birth, and to identify key demographic (e.g., age) and nondemographic characteristics (e.g., prior birth) associated with vaccination intention. METHODS: The authors conducted a cross-sectional survey with pregnant individuals attending prenatal practices affiliated with a large academic medical center and two rural county health departments in the southeastern United States. The survey included 11 questions and was self-administered by pregnant individuals in clinic settings. The final analysis included responses from 443 pregnant individuals. RESULTS: Only 67% of pregnant individuals planned to vaccinate their baby with all recommended routine vaccines; an additional 22% reported having questions about recommendations. Only 35% of pregnant individuals said they would vaccinate their baby as soon as possible with a COVID-19 vaccine. Younger pregnant individuals and those identifying as Black or African American had lower routine and COVID-19 vaccination intention. Routine vaccination, but not COVID-19 vaccination intention, was associated with familiarity with routine vaccination schedule, trimester of pregnancy, Hispanic ethnicity, history of prior birth, and having access to a computer. CONCLUSION: Study findings support differential tailoring of future interventions targeting pregnant individuals to promote routine and COVID-19 vaccines for children.

A deceptive mass on neonatal ultrasound and a fetal brain MRI-confirmed complex dural arteriovenous fistula postnatally: illustrative case.

BACKGROUND: Dural arteriovenous fistulas (dAVFs) are direct, aberrant connections between dural arteries and cerebral veins. In neonates, delayed diagnosis results in grim outcomes. Treatment involves endovascular management because of its success and tolerability. Here, the authors present a case of a complex dAVF initially recognized with an in utero neurosonogram and fetal magnetic resonance imaging (MRI). OBSERVATIONS: A 21-week fetal ultrasound of a nonspecific brain mass was confirmed with fetal MRI as a 2.7-cm T1-hyperintense posterior fossa mass. Although a large flow void in the left middle cranial fossa was present, postnatal computed tomography angiography ultimately revealed a high-flow dAVF communicating with the left transverse sinus. In the early postnatal period, the patient developed hydrocephalus. After successful partial embolization, 6-week postangiogram brain MRI indicated disease progression with the development of a venous varix causing brainstem compression. Repeat embolization resulted in complete cessation of early venous drainage. LESSONS: Neonatal dAVFs are exceedingly rare and result in futile outcomes; however, detection in utero is possible. Although definitive therapy must be performed postnatally, constant monitoring and early delivery can prevent complications. Attention to fetal ultrasound is essential, and knowledge of fetal MRI in the detection of these complex lesions can significantly improve outcomes.

Symptomatic versus asymptomatic COVID-19: does it impact placental vasculopathy?

This study sought to assess the impact of COVID-19 on placental vasculature in the context of maternal symptomatology - comparing asymptomatic to symptomatic pregnant patients - and disease severity - comparing pregnant patients with mild, moderate, severe, and critical COVID-19 infection. PCR-confirmed COVID-19 positive pregnant patients in a single health system who delivered between 3/2020-5/2021 included. All patients had positive COVID test and delivered during the study period. Primary outcome was incidence of any vascular malperfusion on placental pathology. Secondary outcomes were FVM and MVM on placental pathology. Placental pathology compared between symptomatic (sCOVID) and asymptomatic (aCOVID) patients. Secondary analysis of symptomatic patients, comparing placental pathology between mild disease(mCOVID) and worse disease(moderate, severe, or critical-defined by 2020 NIH guidelines) (dCOVID), also performed. Of 112 patients, 53 (47%) had symptoms. Twenty-seven (24.1%) patients had evidence of vascular malperfusion; 26 (23.2%) had MVM. When comparing aCOVID and sCOVID patients, no difference in rate of vascular malperfusion identified, nor any differences in rates of FVM or MVM. Among sCOVID patients (n = 53), 39 (74%) had mCOVID and 14 (26%) had dCOVID (moderate n = 4, severe n = 9, critical n = 1). Patients with dCOVID had earlier median delivery GA (37.4wks vs 39.2wks, p = .03). No difference in latency from diagnosis to delivery seen between mCOVID and dCOVID groups (4.4 vs 3.0wks, p = .96). Twelve (30.8%) patients had vascular malperfusion on pathology, all had mCOVID (p = .02). Eleven (28.2%) mCOVID patients had MVM; no dCOVID patients had evidence of vascular malperfusion (p = .03). No difference in FVM was found between cohorts. Symptomatic COVID-19 infection did not impact placental vasculature differently than asymptomatic infection, even when stratifying by trimester of infection. Among pregnant patients with symptomatic COVID-19, mild disease was associated with placental vascular changes on the maternal side while severe disease was not. Further studies are needed to understand the implications of these findings.

Lower Urinary Pathogens: Do More Pathogenic Bacteria Increase the Risk of Pyelonephritis?

OBJECTIVE: Outside of pregnancy, urinary pathogens such as Proteus and Klebsiella are considered more pathogenic than E. coli. During pregnancy, the implications of lower urinary tract infection (LUTI) with more pathogenic bacteria are unclear. Thus, we sought to compare the risk of progression from LUTI to pyelonephritis among women infected with these more pathogenic urinary bacteria to those infected with E. coli. STUDY DESIGN: Retrospective cohort of pregnant women with LUTI at single tertiary center from July 2013 to May 2019. Pathogenic infections (PI) were defined as asymptomatic bacteriuria or acute cystitis urinary cultures positive for Proteus, Klebsiella, Enterobacter, Citrobacter, Acinetobacter, Staphylococcus, or Raoultella species. Demographic, infectious, antepartum, and postpartum data abstracted. Pregnant women with PI compared with those with E. coli. Primary outcome was progression to pyelonephritis. Secondary outcomes included pyelonephritis length of stay (LOS) >6 days, preterm birth (PTB), low birthweight (LBW), and measures of pyelonephritis-related morbidity. RESULTS: Of 686 pregnant women with LUTIs, 313 had urine culture growing out either PI or E. coli, with 59 (12%) growing PI and 254 (54%) growing E. coli. Women with PI were more likely to be African American, have chronic hypertension, and have history of preeclampsia. The primary species causing PI were Klebsiella (n = 29) and Proteus (n = 11). PI were not more likely to progress to pyelonephritis than E. coli LUTIs (10.9 vs. 14.5%; p = 0.67). Median LOS for pyelonephritis and other measures of pyelonephritis-related morbidity did not differ nor did PTB or LBW rates. After controlling for race, body mass index, history of preeclampsia, and history of pyelonephritis, PI were not associated with increased odds of progression to pyelonephritis (adjusted odds ratio: 0.69, 95% confidence interval: 0.27-1.80). CONCLUSION: Bacteria traditionally considered to be more pathogenic outside of pregnancy do not progress to pyelonephritis at higher rates than E. coli in pregnancy, and are associated with similar pyelonephritis-related morbidity. Larger studies are needed to confirm these findings. KEY POINTS: · Little is known about impact of uropathogen on progression to pyelonephritis and obstetric outcomes.. · Rates of progression to pyelonephritis from UTI did not vary by uropathogen.. · Pyelonephritis-related morbidities and preterm birth rates were also similar among uropathogens..

Bad bugs: antibiotic-resistant bacteriuria in pregnancy and risk of pyelonephritis.

BACKGROUND: The introduction of antibiotics has significantly reduced morbidity and mortality from microbial infections, but the rise of antibiotic-resistant and multidrug-resistant microbes is of increasing clinical concern. Few studies have examined the prevalence and impact of antibiotic resistance in common antenatal infections. OBJECTIVE: This study aimed to determine whether pregnant women with a urine culture positive for antibiotic-resistant or multidrug-resistant gram-negative bacteria are at increased risk of developing pyelonephritis than pregnant women infected with antibiotic-susceptible organisms. STUDY DESIGN: This was a retrospective cohort study of pregnant women with asymptomatic bacteriuria or acute cystitis from a single health system from July 2013 to May 2019. Women with gram-negative antibiotic-resistant (resistance to 1-2 antibiotic classes) and multidrug-resistant (resistance to ≥3 antibiotic classes) lower urinary tract infections were compared with women with antibiotic-susceptible urinary tract infections in terms of demographic, infectious, antepartum, and intrapartum data. The primary outcome was pyelonephritis, defined as a billing code for pyelonephritis plus fever or flank pain. The secondary outcomes were length of stay in the hospital because of pyelonephritis, a composite of pyelonephritis complications (renal abscess, sepsis, and intensive care unit admission), and preterm delivery. The differences in the primary outcome were analyzed using multivariate logistic regression. RESULTS: A total of 573 women were eligible for inclusion. Of the 573 women, 334 (58%) had gram-negative bacteria on urine culture. Of the 334 cases, 173 (52%) were antibiotic susceptible, 74 (22%) were antibiotic resistant, and 87 (26%) were multidrug resistant. Women with antibiotic-resistant and multidrug-resistant infections were more likely to have hypertension (P=.004), to be Black (P=.03), to have public insurance (P=.002), and to experience more urinary infections (P=.001). Pyelonephritis was more common in women with antibiotic-resistant (adjusted odds ratio, 2.27; 95% confidence interval, 1.08-4.78) and multidrug-resistant (adjusted odds ratio, 3.06; 95% confidence interval, 1.57-5.96) infections than in women with antibiotic-susceptible urinary tract infections. Length of stay, preterm delivery, and pyelonephritis complications did not differ between antibiotic-susceptible and antibiotic-resistant and multidrug-resistant infections. CONCLUSION: In an age of increasing antibiotic resistance, more than one-half of pregnant women with bacteriuria experience at least 1 infection with an antibiotic-resistant organism. These resistance patterns have a real clinical impact as pregnant women with antibiotic-resistant gram-negative lower urinary tract infections have an estimated 2- to 3-fold increased odds of developing pyelonephritis.

The risk for recurrent preterm birth after prior preterm birth complicated by major fetal anomaly.

Major fetal anomalies (MFA) are a known risk factor for preterm birth (PTB), though the etiology of this is not well-studied, making counseling of these patients difficult. Thus, we sought to describe the rate of recurrent PTB in a second-observed pregnancy among persons with a history of PTB of an infant with a MFA in a first observed pregnancy, and to assess the difference in delivery timing between the first- and second-observed pregnancy. This was a retrospective cohort study of patients with a first-observed pregnancy complicated by MFA and a second-observed pregnancy in single healthcare system between 2013 and 2017. The primary outcome was recurrent PTB (PTB in second-observed pregnancy) and secondary outcomes were recurrent spontaneous PTB (SPTB), delivery gestational age (GA) in second-observed pregnancy, and difference in delivery GA between the first- and second-observed pregnancy. Recurrent PTB in the setting of prior PTB complicated by an MFA is common (∼1/4 patients), though median delivery timing for individuals who delivered preterm in the first-observed pregnancy was 6 weeks later in the second-observed pregnancy. These data suggest that PTB in the setting of MFA is a comparable risk factor to PTB in the absence of MFA.

Does Obesity Affect Preoperative Shoulder Balance in Adolescent Idiopathic Scoliosis?

BACKGROUND: Obese patients with adolescent idiopathic scoliosis (AIS) have been shown to present with larger curve magnitudes preoperatively. However, the effect of obesity on shoulder balance in AIS remains unknown. The purpose of our study was to determine if overweight and obese patients with AIS have worse radiographic shoulder balance on initial presentation when compared with normal weight patients. METHODS: AIS patients <18 years old, with Lenke 1 or 2 curves, who underwent a posterior spinal fusion between March 2013 and December 2018 were retrospectively evaluated. BMI-for-age percentiles as defined by the Center for Disease Control and Prevention were used: obese (≥95th percentile), overweight (85th to <95th percentile), and normal weight (5th to <85th percentile). Shoulder height was measured via the radiographic shoulder height (RSH) method, with an RSH ≤ 1 cm considered balanced. The primary outcome was preoperative shoulder balance. Secondary outcomes included postoperative shoulder balance, major curve correction, and UIV selection. RESULTS: One hundred eighty-four patients (116 [63%] normal weight and 68 [37%] overweight/obese) were included. The mean age at surgery was 13.1 ± 2 years, and mean follow-up was 17.4 ± 13 months. Preoperative shoulder imbalance was significantly greater in the overweight/obese group compared to the normal weight group (1.9 ± 1 cm vs. 1.5 ± 1 cm, p = 0.04). The odds ratio of presenting with unbalanced shoulders was 2.0 (95% CI: 1.02-3.83, p = 0.04) for the overweight/obese group. No significant differences were found for postoperative shoulder balance, UIV selection, or major curve correction. CONCLUSIONS: Overweight and obese patients with AIS are twice as likely to present with unbalanced shoulders preoperatively; however, this difference is not clinically relevant with a mean difference of 0.4 cm between cohorts. Finally, the preoperative BMI percentile did not show a significant effect on the chosen UIV or curve magnitude correction. LEVEL OF EVIDENCE: Level III: this is a retrospective case-control study.

Posterior cranial vault distraction osteogenesis in the immunocompromised patient.

PURPOSE: The treatment of patients with multisuture craniosynostosis is complex and patient-dependent. Cranial distraction osteogenesis is a relatively new procedure for treatment of these patients, with its use increasing in many centers. With this increased use comes an expanding range of indications. Surgical management of multisuture craniosynostosis in therapeutically immunosuppressed patients following a solid organ transplant presents unique challenges. We describe our experience with posterior cranial vault distraction in two patients with multisuture craniosynostosis that had previously undergone organ transplantation. METHODS: Two solid-organ transplant recipient patients with multisuture craniosynostosis were identified. A detailed examination of their medical/transplant history and perioperative details were recorded. RESULTS: The first patient was a 3-year-old girl who received a kidney transplantation in infancy and subsequently presented with a symptomatic Chiari malformation and papilledema. Imaging revealed pansynostosis. She underwent posterior cranial vault distraction extending into a Chiari decompression. Her postoperative course was complicated by distractor site infection at the beginning of consolidation, necessitating early removal of distractors. The second patient was a 2-year-old boy who received a heart transplantation at the age of 3 months and subsequently presented with head shape concerns. Imaging revealed bicoronal and sagittal craniosynostosis. He underwent a posterior cranial vault distraction without complication. Following removal of the distractors, he developed an infection at one of the distractor sites with associated fever and leukocytosis, necessitating washout and drain placement. Both patients achieved successful cranial vault expansion with distraction osteogenesis and at a 2-year follow-up do not have evidence of elevated intracranial pressure. CONCLUSIONS: Immunosuppressive therapy has the potential to inhibit wound healing and place patients at risk for wound infection. Although we have demonstrated successful cranial vault expansion with distraction in two immunosuppressed children, extra care must be taken with these patients when placing semi-buried hardware. Specifically, prompt identification and proactive management of potential infectious complications is critical to applying this technique safely in these patients.

Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results.

OBJECTIVE: We sought to compare our large single-institution cohort of postnatal myelomeningocele closure to the 2 arms of the Management of Myelomeningocele Study (MOMS) trial at the designated trial time points, as well as assess outcomes at long-term follow-up among our postnatal cohort. METHODS: A single-institutional retrospective review of myelomeningocele cases presenting from 1995 to 2015 at Children's Hospital of Pittsburgh was performed. We compared outcomes at 12 and 30 months to both arms of the MOMS trial and compared our cohort's outcomes at those designated time points to our long-term outcomes. Univariate statistical analysis was performed as appropriate. RESULTS: One-hundred sixty-three patients were included in this study. All patients had at least 2-year follow-up, with a mean follow-up of 10 years (range 2-20 years). There was no difference in the overall distribution of anatomic level of defect. Compared to our cohort, the prenatal cohort had a higher rate of tethering at 12 months of age, 8 versus 1.8%. Conversely, the Chiari II decompression rate was higher in our cohort (10.4 vs. 1.0%). At 30 months, the prenatal cohort had a higher rate of independent ambulation, but our cohort demonstrated the highest rate of ambulation with or without assistive devices among the 3 groups. When comparing our cohort at these early time points to our long-term follow-up data, our cohort's ambulatory function decreased from 84 to 66%, and the rate of detethering surgery increased almost 10-fold. CONCLUSIONS: This study demonstrated that overall ambulation and anatomic-functional level were significantly better among our large postnatal cohort, as well as having significantly fewer complications to both fetus and mother, when compared to the postnatal cohort of the MOMS trial. Our finding that ambulatory ability declined significantly with age in this patient population is worrisome for the long-term outcomes of the MOMS cohorts, especially given the high rates of cord tethering at early ages within the prenatal cohort. These findings suggest that the perceived benefits of prenatal closure over postnatal closure may not be as substantial as presented in the original trial, with the durability of results still remaining a concern.

Norrie disease with a spontaneously shrinking choroid plexus abnormality: a case report.

Background: Norrie disease is a genetic disorder of the retina characterized by impaired retinal vascular development leading to retinal detachment and blindness. Non-retinal manifestations of the disorder include intellectual disability and seizure disorders. However, to date, no association with neurological mass lesions has been described.Materials and methods: Case reporResults: Here, we report a case of a patient with Norrie disease who presented with an enhancing mass of the choroid plexus that spontaneously diminished in size. Conclusion: This report suggests watchful waiting as a reasonable clinical approach to choroid plexus lesions in patients with Norrie disease.

Beyond Expert Opinion: A Comparison of Antibiotic Regimens for Infectious Urinary Tract Pathology in Pregnancy.

Objective Outside pregnancy, nitrofurantoin, ciprofloxacin and sulfamethoxazole-trimethoprim (SMZ-TMP) are first-line therapy (FLT) for lower urinary tract infections (LUTIs). Optimal antibiotics for LUTI have been extrapolated based on expert opinion. Progression to pyelonephritis and adverse obstetric outcomes were compared between women who received FLT and those given alternative antibiotics. Methods This study includes a retrospective cohort of women with LUTI, including asymptomatic bacteriuria and acute cystitis at single health care system from July 2013 to May 2019. Women receiving FLT, defined as nitrofurantoin or SMZ-TMP, were compared with those receiving nonfirst-line therapy (nFLT). Primary outcome was progression to pyelonephritis. Secondary outcomes included pyelonephritis-related anemia, sepsis, length of stay, preterm birth (PTB), and low birth weight (LBW). Logistic regression was used to calculate odds of outcomes. Results Of 476 women, 336 (70.6%) received FLT and 140 (29.4%) received nFLT. Women receiving FLT were more likely having BMI ≥ 40 ( p = 0.04). Progression to pyelonephritis did not differ (5.8 vs. 8.2%; p = 0.44), nor did other pyelonephritis-related outcomes. After controlling for confounders, no difference in odds of progression to pyelonephritis was seen (adjusted odds ratio [aOR] 1.02, 95% confidence interval [CI] 0.42, 2.49). FLT was not associated with PTB or LBW (aOR 0.60, 95% CI 0.29, 1.26) after controlling for confounders. Conclusion Receipt of antibiotics other than nitrofurantoin or SMZ-TMP for LUTI in pregnancy was not associated with increased risk of progression to pyelonephritis, PTB, or LBW.