Comparison of endoscopic sinus sampling versus intracranial sampling for microbiological diagnosis of intracranial infection in children: a case series and literature review.

INTRODUCTION: Intracranial infection is often associated with contiguous sinus infection, with Streptococcus intermedius being the most common pathogen. Microbiological assessment is possible via sinus or intracranial sampling. While a sinus approach is minimally invasive, it is not clear whether this yields definitive microbiological diagnosis leading to optimized antimicrobial therapy and avoidance of intracranial surgery. METHODS: A retrospective review of a prospectively collected electronic departmental database identified patients between 2019 and 2022. Further demographic and microbiological information was obtained from electronic patient records and laboratory management systems. RESULTS: Thirty-one patients were identified with intracranial subdural and/or epidural empyema and concurrent sinus involvement during the 3-year study period. The median age of onset was 10 years with a slight male predominance (55%). All patients had intracranial sampling with 15 patients undergoing sinus sampling in addition. Only 1 patient (7%) demonstrated identical organism(s) grown from both samples. Streptococcus intermedius was the most common pathogen in intracranial samples. Thirteen patients (42%) had mixed organisms from their intracranial cultures and 57% of samples undergoing bacterial PCR identified additional organisms, predominantly anaerobes. Sinus samples had a significant addition of nasal flora and Staphylococcus aureus which was rarely grown from intracranial samples. Of concern, 7/14 (50%) of sinus samples did not identify the main intracranial pathogen diagnosed on intracranial culture and additional PCR. Literature review identified 21 studies where sinus drainage was used to treat intracranial empyemas, with only 6 authors reporting concurrent microbiology results. This confirmed our cohort to be the largest comparative study in the current literature. No center has observed a greater than 50% concordance in microbiological diagnoses. CONCLUSION: Endoscopic sinus surgery may have therapeutic benefit, but it is not an appropriate approach for microbiological diagnosis in pediatric subdural empyemas. High rates of contaminating nasal flora can lead to misdiagnosis and inappropriate treatment. Routine addition of 16S rRNA PCR to intracranial samples is recommended.

Multiparametric Imaging for Presurgical Planning of Craniopagus Twins: The Experience of Two Tertiary Pediatric Hospitals with Six Sets of Twins.

Conjoined twins are rare and pose a challenge to radiologists and surgeons. Craniopagus twins, where conjunction involves the cranium, are especially rare. Even in large pediatric centers, radiologists are unlikely to encounter more than one such event in their medical careers. This rarity makes it daunting to select a CT and MRI protocol for these infants. Using the experience of two tertiary pediatric hospitals with six sets of craniopagus twins, this multidisciplinary and multimodal integrated imaging approach highlights the key questions that need addressing in the decision-making process for possible surgical intervention.

ERF-related craniosynostosis: The phenotypic and developmental profile of a new craniosynostosis syndrome.

Mutations in the ERF gene, coding for ETS2 repressor factor, a member of the ETS family of transcription factors cause a recently recognized syndromic form of craniosynostosis (CRS4) with facial dysmorphism, Chiari-1 malformation, speech and language delay, and learning difficulties and/or behavioral problems. The overall prevalence of ERF mutations in patients with syndromic craniosynostosis is around 2%, and 0.7% in clinically nonsyndromic craniosynostosis. Here, we present findings from 16 unrelated probands with ERF-related craniosynostosis, with additional data from 20 family members sharing the mutations. Most of the probands exhibited multisutural (including pan-) synostosis but a pattern involving the sagittal and lambdoid sutures (Mercedes-Benz pattern) predominated. Importantly the craniosynostosis was often postnatal in onset, insidious and progressive with subtle effects on head morphology resulting in a median age at presentation of 42 months among the probands and, in some instances, permanent visual impairment due to unsuspected raised intracranial pressure (ICP). Facial dysmorphism (exhibited by all of the probands and many of the affected relatives) took the form of orbital hypertelorism, mild exorbitism and malar hypoplasia resembling Crouzon syndrome but, importantly, a Class I occlusal relationship. Speech delay, poor gross and/or fine motor control, hyperactivity and poor concentration were common. Cranial vault surgery for raised ICP and/or Chiari-1 malformation was expected when multisutural synostosis was observed. Variable expressivity and nonpenetrance among genetically affected relatives was encountered. These observations form the most complete phenotypic and developmental profile of this recently identified craniosynostosis syndrome yet described and have important implications for surgical intervention and follow-up.