Δευτέρα 30 Νοεμβρίου 2020

Acute mastoiditis complicated by cerebral venous sinus thrombosis in children.

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Acute mastoiditis complicated by cerebral venous sinus thrombosis in children.

Int J Pediatr Otorhinolaryngol. 2020 Nov 17;:110508

Authors: Vergadi E, Velegrakis S, Raissaki M, Bitsori M, Karatzanis A, Galanakis E

Abstract
AIM: Diagnosis and management of complicated mastoiditis in childhood are still controversial. We investigated the clinical manifestations, evaluation and management of children with mastoiditis complicated with cerebral venous sinus thrombosis.
METHODS: Retrospective cohort study that included all children admitted for acute mastoiditis over the last 5 years. Children were divided in two groups based on the presence or not of venous sinus thrombosis. Clinical, laboratory, imaging and management data were retrieved and compared.
RESULTS: Overall, 20 children with acute mastoiditis were included, of whom 5 had magnetic resonance imaging-confirmed cerebral venous sinus thrombosis and elevated intracranial pressure (ICP). In all complicated cases, neurological signs rather than mastoiditis signs, prevailed. The more prominent neurologic signs observed were lethargy (60%), nuchal rigidity (60%), abducens nerve palsy (60%) and ataxic gait (20%). Treatment consisted of intravenous antibiotics combined with anticoagulation. Surgery was performed in four children (4/5). Complicated cases had prolonged symptoms prior to admission (p 0.002), presented with neurologic signs and symptoms (p < 0.001), underwent more often lumbar puncture (p < 0.001) and brain imaging (p < 0.001), and were treated with prolonged courses of antibiotics and surgery (<0.001), compared to children with uncomplicated mastoiditis.
CONCLUSION: Neurological signs and symptoms and elevated ICP dominate in children with mastoiditis complicated with thrombosis. Brain imaging is essential for early diagnosis of cerebral venous sinus complications and appropriate management.

PMID: 33234334 [PubMed - as supplied by publisher]

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Bipolar loop device versus bipolar diathermy for tonsillectomy: A pilot randomized controlled trial.

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Bipolar loop device versus bipolar diathermy for tonsillectomy: A pilot randomized controlled trial.

Int J Pediatr Otorhinolaryngol. 2020 Nov 19;:110505

Authors: Fakhrealizadeh F, Hoveidaei AH, Eslami J, Hashemi SB, Hoveidaei A, Kazemi T, Nazarpour Z

Abstract
OBJECTIVE: Tonsillectomy is one of the most common surgeries performed in the pediatric population. Although different forms of instruments and various methods are used to perform tonsillectomy, none of them is still recognized as the best global technique. This study aimed to compare the outcomes of the new Bipolar Loop tonsillectomy versus bipolar diathermy technique.
METHODS: This study is a pilot randomized clinical trial and was conducted on 40 pediatric patients who were the candidate of tonsillectomy. Patients were divided into two groups of Bipolar Loop or bipolar diathermy. Operation time, intraoperative bleeding, tonsillar fossa wound, postoperative complications, and duration of return to normal diet were evaluated in the current study.
RESULTS: In both of the Bipolar Loop and bipolar diathermy groups, no significant difference was found in terms of sex, age, and weight. The average amount of the operative time, intra-operative blood loss, and postoperative pain loss were significantly less in the Bipolar Loop group (P < .001). In addition, the tonsillar fossa wound healing scores were significantly better (on the 14th day, P = 0.009). However, there was no significant difference between the two groups in terms of postoperative bleeding, duration of return to a normal diet, and postoperative symptoms of fever, otalgia, or voice change between the groups.
CONCLUSION: The study showed that blood loss and postoperative pain through day 7 were significantly less in the Bipolar Loop group. Tonsillectomy with Bipolar Loop can be recommended as one of the methods for tonsillectomy, especially in pediatric patients.

PMID: 33239197 [PubMed - as supplied by publisher]

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Self-concept of children and adolescents with cochlear implants.

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Self-concept of children and adolescents with cochlear implants.

Int J Pediatr Otorhinolaryngol. 2020 Nov 17;:110506

Authors: Boerrigter M, Vermeulen A, Marres H, Mylanus E, Langereis M

Abstract
OBJECTIVES: Communicative disorders can complicate social interactions and may be detrimental for one's self-concept. This study aims to assess the self-concept of children with Cochlear Implants (CI). Results of educational peer groups (special needs or typical) were compared. Correlations amongst speech perception, language comprehension, self-concept and other study variables are determined.
METHODS: This retrospectively patient file study contained 53 CI participants with a mean age of 14.2 (SD = 2.8). Self-concept was measured with the Dutch language version of the Self-Perception Profile for Children and Adolescents. Proportions of low, normal and high competence scores were compared to a normative sample. Outcomes were analyzed for the total CI group and for the two educational peer groups.
RESULTS: In the Scholastic Competence, Athletic Competence, Physical Appearance and Behavioral Conduct domains larger proportions of high perceived competence levels were found in the CI Total group compared to the hearing normative sample. Children with CIs in the Mainstream educational subgroup were found to have larger proportions of high levels on these domains. Remarkably, children with CI in the Special hearing impaired educational subgroup reported comparable self-concept scores as their hearing peers. Speech perception and language comprehension were positively correlated to Scholastic Competence.
CONCLUSION: This study has shown that self-concept levels of profoundly hearing impaired children with CI are comparable to those of hearing peers. They are generally satisfied with their functioning in various domains. Better speech perception and language comprehension levels are related to higher outcomes in the Scholastic Competence domain.

PMID: 33243619 [PubMed - as supplied by publisher]

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Airway management protocol for conjoined twins delivery.

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Airway management protocol for conjoined twins delivery.

Int J Pediatr Otorhinolaryngol. 2020 Nov 04;:110477

Authors: Snoap A, Varadarajan VV, Mowitz ME, Islam S, Collins WO

Abstract
OBJECTIVE: Conjoined twin deliveries require collaborative preparation by multiple specialties for successful airway management. Literature regarding neonatal airway management after conjoined twin delivery is limited to case reports. We present a case series of conjoined twins and introduce an airway management protocol for conjoined twin delivery.
METHODS: The medical records of conjoined twins and their mothers at a tertiary care center were reviewed from April 2016 to December 2018. The NCBI database was queried for literature regarding preparation for neonatal airway management after conjoined twins delivery.
RESULTS: Five sets of conjoined twins were delivered. Of 10 neonates, all required bag valve mask ventilation. Other airway interventions included continuous positive airway pressure (7), endotracheal intubation (6), and direct laryngoscopy with telescopic video evaluation (1). No patients required ex-utero intrapartum treatment or emergent tracheostomy. A protocol for airway management is described and special considerations are discussed, including anatomic variations, equipment list, operating room staffing and layout, multidisciplinary prenatal conference, and airway imaging review.
CONCLUSION: Conjoined twin deliveries have significant implications for the otolaryngologist and require multidisciplinary collaboration. An airway management protocol allows for a standardized process to secure the neonatal airway and optimize patient outcomes.

PMID: 33243620 [PubMed - as supplied by publisher]

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Feasibility of a hearing screening programme using DPOAEs in 3-year-old children in South Auckland.

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Feasibility of a hearing screening programme using DPOAEs in 3-year-old children in South Auckland.

Int J Pediatr Otorhinolaryngol. 2020 Nov 18;:110510

Authors: Su E, Leung JH, Morton RP, Dickinson LJ, Vandal AC, Balisa NB, Purdy SC

Abstract
OBJECTIVES: In New Zealand (NZ), permanent hearing loss is associated with higher levels of socioeconomic deprivation, and is more prevalent amongst Māori and Pacific than NZ European children. Many of these hearing losses are detected through newborn hearing screening, however there is a need to screen children again later, to look for childhood hearing losses that are either late-onset, progressive, or acquired. This study evaluated the feasibility of implementing an objective screening protocol that includes otoscopy, distortion product otoacoustic emission screening (DPOAEs), and tympanometry. It also evaluated the feasibility of using Early Learning Centres (ELCs) to contact families, recruit, and test 3-year-old children from an area of high socioeconomic deprivation in Auckland, New Zealand.
METHODS: Sixty-one 3-year-old children were recruited from ELCs within the Counties Manukau District Health Board (CMDHB) region which services the geographical area of South Auckland. The first part of the screening protocol consisted of otoscopy, DPOAEs, and tympanometry. Children identified with hearing loss and/or middle ear problems were either referred directly to Otolaryngology/Audiology at the local hospital or invited back for a re-screen 4-8 weeks later. Children who were referred from the screening were followed up to track and document their subsequent clinical pathway through the public health system.
RESULTS: Mean overall time for the screening protocol was 4.1 minutes. The combination of otoscopy, DPOAEs, and tympanometry was well accepted by the 3-year-old children. DPOAE amplitude and signal-to-noise ratio results significantly differentiated between different tympanometry results, providing support for this combination of measures to accurately screen for hearing loss and/or middle ear disease. Thirty-eight of the 61 children (62%) passed the screening protocol. Of the remaining 23 children, five were referred to the hospital after not passing the screening, but following more in-depth audiological testing, were discharged with normal hearing. Six children referred to the hospital were diagnosed with varying degrees of conductive hearing loss, and two of the six received grommet insertion surgery. The remaining 12 children who were referred to the hospital were lost to follow-up, highlighting challenges for the families to successfully navigate the current public health system.
CONCLUSION: This study demonstrates that identifying hearing loss and ear disease in 3-year-old children in the pre-school setting is feasible. A number of barriers were identified in the current health system that contribute to a large proportion of children referred with suspected hearing loss and ear disease being unsuccessful in accessing Otolaryngology/Audiology clinical care through the local hospital.

PMID: 33248714 [PubMed - as supplied by publisher]

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Nasal dorsum reconstruction after pediatric nasal dermoid excision.

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Nasal dorsum reconstruction after pediatric nasal dermoid excision.

Int J Pediatr Otorhinolaryngol. 2020 Nov 16;:110502

Authors: Carroll WW, Farhood Z, White DR, Patel KG

Abstract
BACKGROUND: To review our experience with pediatric nasal dermoids, and discuss reconstructive options for the nasal dorsum after pediatric nasal dermoid removal.
METHODS: Retrospective review of pediatric nasal dermoid cases from January 1 2005 through October 1 2016.
RESULTS: Twenty-five cases (12 males, 13 females) were identified. Median age at time of surgery was 24 months (7-144). Ten nasal dermoids were superficial; eleven, intraosseous; one, intracranial extradural; three, intracranial intradural. Seven were located on the glabella; fifteen, dorsum; three, nasal tip. Twelve underwent vertical midline incision; ten underwent external rhinoplasty; and three combined approach with craniotomy. There was one recurrence four years postoperatively; which was secondarily resected completely via external rhinoplasty approach. Seven cases utilized endoscopic assistance. Conchal cartilage grafting was utilized in nine cases for dorsal reconstruction. A temporoparietal fascial graft was utilized to reconstruct the soft tissue defect in three patients. Median follow-up was 1.17 years (1 month-10 years).
CONCLUSIONS: Nasal dermoid is a rare congenital pathology. Recurrence rate is generally low provided that complete surgical excision is achieved. Achieving complete surgical excision means sometimes compromising the upper lateral cartilages and nasal bones. Conchal cartilage grafting is useful in reconstruction for lesions that significantly disrupt the nasal cartilages and/or nasal bones, wherein the defect is significant and osteotomies may not be sufficient. Temporoparietal fascia is a favorable adjunct for reconstructing soft tissue deficits when the skin is thin. Further studies and longer follow up are needed to adequately assess functional and cosmetic outcomes.

PMID: 33248715 [PubMed - as supplied by publisher]

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Tumor spread through air spaces (STAS): prognostic significance of grading in non-small cell lung cancer

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Abstract
Tumor spread through air spaces (STAS) is an invasive pattern of lung cancer that was recently described. In this study, we investigated the association between the extent of STAS and clinicopathological characteristics and patient outcomes in resected non-small cell lung cancers (NSCLCs). STAS has been prospectively described from 2008 and graded its extent with a two-tiered system (STAS I: <2500 μm [one field of ×10 objective lens] from the edge of tumor and STAS II: ≥2500 μm from the edge of tumor) from 2011 in Seoul National University Bundang Hospital. We retrospectively analyzed the correlations between the extent of STAS and clinicopathologic characteristics and prognostic significance in 1869 resected NSCLCs. STAS was observed in 765 cases (40.9%) with 456 STAS I (24.4%) and 309 STAS II (16.5%). STAS was more frequently found in patients with adenocarcinoma (ADC) (than squamous cell carcinoma), pleural invasion, lymphovascular invasion, and/or higher pathologic stage . In ADC, there were significant differences in recurrence free survival (RFS), overall survival (OS), and lung cancer specific survival (LCSS) according to the extent of STAS. In stage IA non-mucinous ADC, multivariate analysis revealed that STAS II was significantly associated with shorter RFS and LCSS (p < 0.001 and p = 0.006, respectively). In addition, STAS II was an independent poor prognostic factor for recurrence in both limited and radical resection groups (p = 0.001 and p = 0.023, respectively). In conclusion, presence of STAS II was an independent poor prognostic factor in stage IA non-mucinous ADC regardless of the extent of resection.

Modern Pathology, Published online: 16 November 2020; doi:10.1038/s41379-020-00709-2

Tumor spread through air spaces (STAS): prognostic significance of grading in non-small cell lung cancer
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Recognition of filigree pattern expands the concept of micropapillary subtype in patients with surgically resected lung adenocarcinoma

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Our study aimed to validate the clinicopathological characteristics and prognosis of lung adenocarcinoma (ADC) with a filigree pattern and to further investigate the relationship between the filigree pattern and the classical micropapillary (MP) pattern. We retrospectively reviewed the clinical and pathologic characteristics of 461 Chinese patients with completely resected ADC (stage I, 310; stage II, 44; stage III, 107). The filigree pattern was more likely to be observed in ADC with a higher stage (p = 0.003) and the classical MP pattern (p < 0.001). Patients with filigree-predominant ADC showed poor survival, similar to those with classical MP-predominant ADC. Multivariate analysis confirmed that the presence of the filigree pattern was an independent prognostic factor for recurrence-free survival (hazard ratio (HR), 2.01; 95% confidence interval (CI), 1.50–2.68; p < 0.001) and overall survival (OS; HR, 1.83; 95% CI, 1.34–2.50; p < 0.001). Patients with both cl assical MP-positive and filigree-positive ADC had the worst survival compared with those with the filigree pattern or classical MP pattern alone. In stage I, ADC with both the filigree and classical MP patterns had a higher incidence of micrometastasis than ADC with the filigree pattern or classical MP pattern alone. Lymph node micrometastasis indicated poor survival in patients with ADC with the filigree pattern or classical MP pattern. Similar clinicopathologic features between patients with the filigree pattern and the classical MP pattern support the inclusion of the filigree pattern in the MP category. Recognition of the filigree pattern could provide helpful prognostic information, especially for stage I ADC.

Modern Pathology, Published online: 16 November 2020; doi:10.1038/s41379-020-00711-8

Recognition of filigree pattern expands the concept of micropapillary subtype in patients with surgically resected lung adenocarcinoma
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TP53 mutations and CDKN2A mutations/deletions are highly recurrent molecular alterations in the malignant progression of sinonasal papillomas

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Sinonasal papillomas are benign epithelial tumors of the sinonasal tract that are associated with a synchronous or metachronous sinonasal carcinoma in a subset of cases. Our group recently identified mutually exclusive EGFR mutations and human papillomavirus (HPV) infection in inverted sinonasal papillomas and frequent KRAS mutations in oncocytic sinonasal papillomas. We also demonstrated concordant mutational and HPV infection status in sinonasal papilloma-associated sinonasal carcinomas, confirming a clonal relationship between these tumors. Despite our emerging understanding of the oncogenic mechanisms driving formation of sinonasal papillomas, little is currently known about the molecular mechanisms of malignant progression to sinonasal carcinoma. In the present study, we utilized targeted next-generation DNA sequencing to characterize the molecular landscape of a large cohort of sinonasal papilloma-associated sinonasal carcinomas. As expected, EGFR or KRAS mutations were present in the vast majority of tumors. In addition, highly recurrent TP53 mutations, CDKN2A mutations, and/or CDKN2A copy-number losses were detected; overall, nearly all tumors (n = 28/29; 96.6%) harbored at least one TP53 or CDKN2A alteration. TERT copy-number gains also occurred frequently (27.6%); however, no TERT promoter mutations were identified. Other recurrent molecular alterations included NFE2L2 and PIK3CA mutations and SOX2, CCND1, MYC, FGFR1, and EGFR copy-number gains. Importantly, TP53 mutations and CDKN2A alterations were not detected in matched sinonasal papillomas, suggesting that these molecular events are associated with malignant transformation. Compared to aerodigestive tract squamous cell carcinomas from The Cancer Genome Atlas (TCGA) project, sinonasal papilloma-associated sinonasal carcinomas have a distinct molecular phenotype, including more frequent EGFR, KRAS, and CDKN2A mutations, TERT copy-number gains, and low-risk human papillomavirus (HPV) infection. These findings shed light on the molecular mechanisms of malignant progression of sinonasal papillomas and may have important diagnostic and therapeutic implications for patients with advanced sinonasal cancer.

Modern Pathology, Published online: 17 November 2020; doi:10.1038/s41379-020-00716-3

TP53 mutations and CDKN2A mutations/deletions are highly recurrent molecular alterations in the malignant progression of sinonasal papillomas
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TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1

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Renal cell carcinomas with t(6;11) chromosome translocation has been classically characterized by the rearrangement of the TFEB gene, located on chromosome 6, and MALAT1 gene, located on chromosome 11. Recently, a few other genes have been described as fusion partners in TFEB rearranged renal cell carcinomas. Although most of TFEB rearranged renal cell carcinomas have an indolent behavior, in the rare cases of advanced metastatic disease targeted therapy and predictive markers remain lacking. In the present study, we collected 13 TFEB rearranged renal cell carcinomas, confirmed by FISH, analyzing their morphology and exploring the novel gene partners. Looking for predictive markers, we have also performed PDL1 immunohistochemical analysis by using four different assays (E1L3N, 22C3, SP142, and SP263). MALAT1 gene rearrangement has been found in ten tumors, five cases showing classical biphasic morphology with "rosettes", five cases without "rosettes" mimicking other renal cel l carcinomas or epithelioid angiomyolipoma/pure epithelioid PEComa. We identified two different partner genes, ACTB and NEAT1, the latter previously unreported and occurring in a tumor with an unusual solid and cystic appearance. In both cases, the "rosettes" were absent. In one case no gene partner was identified. Overall, in 12 of 13 TFEB-rearranged renal cell carcinomas staining for PDL1 SP263 was observed, whereas the other antibodies were less reliable or more difficult to interpret. In conclusion, we described the third case of ACTB-TFEB rearranged renal cell carcinoma and a novel NEAT1-TFEB rearranged renal cell carcinoma, both without the distinctive biphasic morphology typical of t(6;11) renal cell carcinoma. Finally, PDL1 SP263 was constantly expressed in TFEB rearranged renal cell carcinoma with possible clinical benefit which requires further investigations.

Modern Pathology, Published online: 18 November 2020; doi:10.1038/s41379-020-00713-6

TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1
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Clinico-pathologic predictors of patterns of residual disease following neoadjuvant chemotherapy for breast cancer

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Among breast cancer patients treated with neoadjuvant chemotherapy (NAC) who do not experience a pathologic complete response (pCR), the pattern of residual disease in the breast varies. Pre-treatment clinico-pathologic features that predict the pattern of residual tumor are not well established. To investigate this issue, we performed a detailed review of histologic sections of the post-treatment surgical specimens for 665 patients with stage I-III breast cancer treated with NAC followed by surgery from 2004 to 2014 and for whom slides of the post-NAC surgical specimen were available for review. This included 242 (36.4%) patients with hormone receptor (HR)+/HER2− cancers, 216 (32.5%) with HER2+ tumors, and 207 (31.1%) with triple negative breast cancer (TNBC). Slide review was blinded to pre-treatment clinico-pathologic features. pCR was achieved in 7.9%, 37.0%, and 37.7%, of HR+/HER2− cancers, HER2+ cancers, and TNBC respectively (p < 0.001). Among 389 patients with residua l invasive cancer in whom the pattern of residual disease could be assessed, 287 (73.8%) had a scattered pattern and 102 (26.2%) had a circumscribed pattern. In both univariate and multivariate analyses, there was a significant association between tumor subtype and pattern of response. Among patients with HR+/HER2− tumors, 89.4% had a scattered pattern and only 10.6% had a circumscribed pattern. In contrast, among those with TNBC 52.8% had a circumscribed pattern and 47.2% had a scattered pattern (p < 0.001). In addition to subtype, histologic grade and tumor size at presentation were also significantly related to the pattern of residual disease in multivariate analysis, with lower grade and larger size each associated with a scattered response pattern (p = 0.002 and p = 0.01, respectively). A better understanding of the relationship between pre-treatment clinico-pathologic features of the tumor and pattern of residual disease may be of value for helping to guide post- chemotherapy surgical management.

Modern Pathology, Published online: 20 November 2020; doi:10.1038/s41379-020-00714-5

Clinico-pathologic predictors of patterns of residual disease following neoadjuvant chemotherapy for breast cancer
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