Objective  The purpose of this study is to specifically assess pediatric patients with nonpneumatized sphenoid sinuses who have undergone transsphenoidal resections of skull base tumors and assess the complications and outcomes. Methods  Data was collected by a retrospective chart review done on children ages 7 and under who underwent endoscopic tumor resection and had a partially or completely nonpneumatized sphenoid sinus on preoperative computed tomography imaging. Surgical data collected included surgical corridor, gross total versus subtotal resection, repair method, use of septal flap, intraoperative and postoperative cerebrospinal fluid leak, and estimated blood loss. Results  Six patients were identified that fit our inclusion criteria who underwent surgery between November 2015 and April 2018 (3 males, 3 females; average age = 4.28 years). Tumor pathologies include three craniopharyngiomas, Rathke cleft cyst, meningocele, and neuroblastoma. All cases involved varying percentages of sphenoid sinus pneumatization. All cases except one craniopharyngioma and the neuroblastoma required removal of cancellous bone to access pathology. Degree of sphenoid pneumatization tended to be greater with age and resulted in less intraoperative bleeding. The two youngest patients with completely nonpneumatized sphenoid sinuses lost 61.73 and 17.52% of their total blood volume intraoperatively. Procedures were able to be adequately performed and pathology completely addressed with minimal postoperative complications and no postoperative CSF leaks. Conclusions  Intraoperative challenges including hemorrhage are likely in nonpneumatized patients, and thus surgeons should be prepared with adequate vascular access and blood products. Hemorrhage and total blood volume loss are increased in the younger patients with no sphenoid pneumatization. Despite the additional challenges, a nonpneumatized sphenoid sinus is not a contraindication for an endoscopic resection in the pediatric population.

Introduction  The fully endoscopic expanded endonasal approach (EEA) has been shown to be safe and efficacious in pediatric patients. However, in the very young patient (ages six and under), the anatomical challenge of working through a small nasal corridor is problematic. The ability to repair the skull base and use a nasoseptal flap (NSF) has also been called into question. Here, we review skull base resections using EEA in patients aged 6 years and younger. Methods  A retrospective chart review was conducted on pediatric patients age 6 years and younger who underwent EEA skull base resections over a 3-year period (June 2014 through June 2017). Results  Eight children aged 6 and under with seven tumor pathologies underwent an EEA for the resection of their skull base tumors, with a mean follow-up of 1.45 years. Only chordoma cases required multiple-staged resections. There are no tumor recurrences to date. A high-flow intraoperative cerebrospinal fluid (CSF) leak was encountered in three cases, and there were no postoperative CSF leaks. CSF leaks were effectively repaired with NSFs. One patient suffered from postoperative hypopituitarism, one patient experienced recurrent epistaxis secondary to turbinate destruction by the tumor, and one patient expired 8 months postresection secondary to metastatic disease. Conclusion  In our case series, EEA for skull base tumors in children aged 6 and under was a safe and effective surgical approach. Skull base defects are able to be effectively addressed with typical repairs including NSFs. Larger studies are warranted to further investigate this technique in this pediatric population.

Study objectives

The efficacy of portable-monitor (PM) sleep testing in children is not well understood. While most studies have evaluated PM in a lab setting, the utility of PM in the home environment is relatively unknown. We sought to determine whether home PM accurately diagnoses obstructive sleep apnea in adolescents and to assess patient satisfaction with home PM sleep testing.

Methods

We evaluated adolescents (age 12-18 years) with suspected obstructive sleep apnea using a PM device. In addition to in-laboratory polysomnography (PSG), all participants had PM testing performed twice, once in their home and once concurrent to in-laboratory PSG. PM was compared to PSG using 2 primary outcomes: the apnea-hypopnea index and oxygen desaturation index. All participants were approached for interview to evaluate their experience with PM sleep testing.

Results

Twenty adolescents participated. Bland-Altman analysis comparing the apnea-hypopnea index and oxygen desaturation index determined by home or in-laboratory PM to in-laboratory PSG revealed mostly agreement; however, some deviations were observed when either parameter was markedly increased. While PM testing tended to underestimate the apnea-hypopnea index, the diagnostic agreement between home PM and PSG was 80% (by the White-Westbrook method). Most preferred PM to PSG and found PM easy to very easy to set up.

Conclusions

In a small cohort of adolescents, our study supports the application of home PM in the diagnosis of suspected obstructive sleep apnea. Until studies implementing PM using larger cohorts become readily available, the findings from this preliminary study could contribute to adolescents receiving sleep apnea therapy more promptly.

Clinical trial registration

Registry: ClinicalTrials.gov; Identifier: NCT03748771. At the time of issue publication, this registration is not publicly available because the trial includes a device that is not approved or cleared for use in pediatric populations. Once the device is FDA cleared, the registration will become public.

OBJECTIVE: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs. METHODS: We performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022. RESULTS: Overall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSION: Due to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.

Introduction

Nasal chondromesenchymal tumors (NCMT) are rare benign neoplasms that usually present in children <1 year of age. They can display rapid growth and significant local bony remodeling that can mimic a malignant process. Of the ∼50 published cases to date, few have documented the need for neurosurgical intervention. We herein report a NCMT in an infant treated with a staged cranial and transnasal approach, as well as summarize the available literature on this pathology.

Case report

A newborn male with a compromised airway was noted to have a large sinonasal lesion. After stabilization, MRI demonstrated a 4-cm enhancing mass with diffuse sinus involvement and significant extension into the anterior cranial fossa, with displacement of the optic apparatus and hypothalamic pituitary axis. After an initial biopsy, the patient underwent a bifrontal craniofacial approach at 2 months of age, followed by a second-stage transnasal endoscopic approach at 15 months which resulted in a complete resection. There were no neurosurgical complications. Pathology was consistent with a NCMT.

Discussion

Although rare, neurosurgical involvement is critical for the treatment of NCMTs with intracranial extension. Staged cranial and endonasal endoscopic approaches may be needed for complete resection of such lesions.

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria. Here, we categorize these patients as syndrome of undifferentiated recurrent fever (SURF), and identify the unique features which distinguish them from the PFAPA syndrome. SURF patients were more likely to report gastrointestinal symptoms of nausea, vomiting and abdominal pain, and experienced inconsistent responses to on-demand steroid therapy compared to PFAPA patients. For this previously undefined cohort, an optimal course of therapy remains uncertain, with medical and surgical therapies largely driven by parental preference. A subset of patients with SURF underwent tonsillectomy with complete resolution. Flow cytometric evaluation demonstrates leukocytic populations distinct from PFAPA patients, with reduced CD3+ T cell numbers. SURF patient tonsils were predominantly characterized by an IL-1 signature compared to PFAPA, even during the afebrile period. Peripheral blood signatures were similar between groups suggesting that PFAPA and SURF patient tonsils have localized, persistent inflammation, without clinical symptoms. These data suggest that SURF is a heterogenous syndrome on the autoinflammatory disease spectrum.

Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking. In our tertiary care center cohort, 62 patients with PFAPA syndrome had complete resolution of symptoms after surgery (95.3%). Flow cytometric evaluation demonstrates an inflammatory cell population, distinct from patients with infectious pharyngitis, with increased numbers of CD8+ T cells (5.9% vs. 3.8%, p < 0.01), CD19+ B cells (51% vs. 35%, p < 0.05), and CD19+CD20+CD27+CD38-memory B cells (14% vs. 7.7%, p < 0.01). Cells are primed at baseline with increased percentage of IL-1β positive cells compared to control tonsil-derived cells, which require exogenous LPS stimulation. Gene expression analysis demonstrates a fivefold upregulation in IL1RN and TNF expression in whole tonsil compared to control tonsils, with persistent activation of the NF-κB signaling pathway, and differential microbial signatures, even in the afebrile period. Our data indicates that PFAPA patient tonsils have localized, persistent inflammation, in the absence of clinical symptoms, which may explain the success of tonsillectomy as an effective surgical treatment option. The differential expression of several genes and microbial signatures suggests the potential for a diagnostic biomarker for PFAPA syndrome.