Frontal sinus hypoplasia in unoperated older patients with craniosynostosis: a pilot study.

PURPOSE: The purpose of this study is to determine whether patients with unoperated craniosynostosis have different frontal sinus pneumatization than unaffected controls. METHODS: Retrospective review was performed between 2009 and 2020 of previously unoperated patients with craniosynostosis older than 5 years old at first presentation to our institution. Total frontal sinus volume (FSV) was calculated using 3D volume rendering tool in Sectra IDS7 PACS system. Age-matched normative FSV data was collected from 100 normal CT scans for the control group. The two groups were statistically compared using Fisher's exact test and T-test. RESULTS: Study group included nine patients, 5-39 years old, median age 7 years. Frontal sinus pneumatization was absent in 12% of the normal 7-year-old controls, while frontal sinus pneumatization was absent in 89% of the studied craniosynostosis patients (p < .001). Mean FSV of the study group (113 ± 340 mm3) was significantly different from that of age matched control mean FSV (2016 ± 2529 mm3) (p = .027). CONCLUSIONS: Frontal sinus pneumatization is suppressed in unreleased craniosynostosis and may be an intracranial space conservation phenomenon. This absent frontal sinus can have implications in future frontal region trauma and frontal osteotomies.

Does measurement of the jugular foramen diameter on MRI help to differentiate transverse sinus thrombosis from unilateral transverse sinus hypoplasia?

BACKGROUND AND PURPOSE: The transverse sinus (TS) is a frequent location of cerebral venous thrombosis. However, unilateral TS hypoplasia is a frequent variation and radiological imaging pitfall in the diagnosis because it may mimic unilateral TS thrombosis. The purpose of this study is to find a cutoff value for bilateral jugular foramen (JF) diameter ratios on magnetic resonance imaging (MRI) for differentiating TS thrombosis from TS hypoplasia. MATERIALS AND METHODS: We retrospectively reviewed magnetic resonance venography results for 174 patients with reduced unilateral TS caliber resulting from either unilateral thrombosis (80 patients) or unilateral hypoplasia (94 patients). We calculated the ratio by proportioning the diameter of the JF ipsilateral to the TS with caliber reduction to the diameter of the contralateral JF. The Mann-Whitney U test was used to compare the ratios between thrombosis and hypoplasia groups, and the cutoff value was calculated by receiver-operating characteristic curve analysis. RESULTS: The ratio of bilateral JF diameters was lower in patients with hypoplasia than those with thrombosis (P < .01). The cutoff value to determine the diagnosis of TS hypoplasia with maximum accuracy was 0.638, with a sensitivity of 91.3% and specificity of 64.9%. CONCLUSION: In equivocal cases, calculating the cutoff value by proportioning the diameter of JF ipsilateral to the TS with caliber reduction to the contralateral JF seems to be an efficient, quick, and straightforward method and valuable aid to differentiate TS thrombosis from TS hypoplasia.

Association between Transverse Sinus Hypoplasia and Cerebral Venous Thrombosis: A Case-Control Study.

BACKGROUND: Hypoplasia of the transverse sinus (TS) is a common anatomical variation. However, the relationship between TS hypoplasia and venous thrombosis has not been studied. We analyzed the hypothesis that TS hypoplasia is a predisposing factor for ipsilateral thrombosis. MATERIALS AND METHODS: We retrospectively evaluated 20 confirmed cases with isolated TS thrombosis and 43 age- and sex-matched controls. TS thrombosis and hypoplasia were diagnosed using both computed tomography and magnetic resonance venography. Hypoplasia was defined as a TS diameter less than 50% of the cross-sectional diameter of the lumen of the distal superior sagittal sinus and by a bony groove ratio less than 1.02. Univariate analysis was performed to evaluate the association between TS hypoplasia and thrombosis. RESULTS: There were a total of 45 hypoplastic TS: 31 (49%) left hypoplastic TS (12 (60%) cases vs 19 (44%) controls (P = .24), and 14 (22%) right hypoplastic TS (9 (45%) cases vs 5 (12%) controls (P = .003). TS hypoplasia was more frequently found in cases (n = 18, 90.0%) than in controls (n = 22, 51.2%; relative risk 1.7, confidence interval [CI] 95% 1.3-2.4, P = .003). Hypoplastic TS and ipsilateral TS thrombosis showed a significant association (P = .002 for right and P = .008 for left TS hypoplasia) with relative risk of 3.8 (95% CI 1.3-10) for right and 7.5 (95% CI 1.1-48) for left hypoplasia. No significant association was found between hypoplastic TS and functional outcome at 30- or 90-day follow-up. CONCLUSION: TS hypoplasia might be a predisposing factor for ipsilateral TS thrombosis, but not for functional outcome.

Nasal endoscopy and paranasal sinus computerised tomography (CT) findings in an Irish cystic fibrosis adult patient group.

Cystic fibrosis (CF) is a common inherited disorder in Caucasians in Ireland having the highest reported incidence. CF has well-recognised clinical sequelae in several physiological systems. Its' impact on the sinonasal system is less well established. We evaluated symptoms, endoscopic and computerised tomographic (CT) findings in an Irish adult CF group with the aim of characterising the relationship between these clinical features in an Irish CF group. Adult CF patients attending a specialist clinic underwent prospective evaluation of sinonasal symptoms using a specifically designed questionnaire. They subsequently underwent nasoendoscopy and CT scanning of their paranasal sinuses. Abnormalities identified were quantified using established radiological (Lund-Mackay) and endoscopic (Lund-Kennedy) scoring systems. The relationship between symptoms of chronic rhinosinusitis (CRS), endoscopic findings and CT abnormalities were then compared. Sixty-three CF patients (n = 63) were studied. 29 patients had a CT scan. Thirty-three CF patients (52%) had no symptoms of CRS. Fifty CF patients (80% of CF group) had evidence of CRS on nasoendoscopy including thirteen patients (20%) with nasal polyposis. 98% of patients scanned have positive findings on CT scan. There was no significant difference between symptomatic and asymptomatic CF groups with respect to their Lund-Kennedy endoscopic score or their Lund-Mackay CT score. 86% demonstrated one or more hypoplastic sinus. There was no increased incidence of hypoplastic sinuses amongst Δf508 homozygotes than other mutation groups.

Wildervanck syndrome with hypoplastic frontal sinus: A rare case presentation.

We report a case of Wildervanck syndrome exhibiting Klippel-Feil anomaly, Duane's retraction syndrome and congenital deafness. Since the first case was reported in 1952, there have been more reports describing this triad either complete or incomplete. Our case has a complete triad of the syndrome along with frontal sinus hypoplasia. Our case is unique as the triad was associated with frontal sinus hypoplasia, which is very rare association.

Post-traumatic Sinus Syndrome, Proposal for a New Clinical Entity (CDR Syndrome) as Variant of the Silent Sinus Syndrome: Systematic Review and Case Series.

The diagnostic criteria for silent sinus syndrome (SSS) are still controversial, especially for the post-traumatic/surgery cases that are, nowadays, excluded from the diagnosis of SSS because lacking of spontaneously. We present a systematic review of the last 10 years and our case series of SSS associated to previous trauma/surgery, proposing a new interpretation of SSS. In this work, following the PRISMA guide lines for systematic reviews, we collected 86 articles published on PubMed, Cochrane Library and Medline Plus since 2013 to 2023 about SSS. We divided them in six groups forming the structure of the review: (1) epidemiology, (2) clinical presentation, (3) imaging, (4) etiopathogenesis, (5) sss and craniofacial trauma and (6) treatment. We reported two explicative clinical cases: two men of 34 and 37 years old, involved in motorcycle accident in 2020 and 2014, respectively, and underwent surgery. They came back in 2023 referring diplopia documented by Hess-Lancaster test. CT-scan reported two clear cases of SSS. Basing on what is reported in literature, and basing on our experience, the post-traumatic/surgery SSS are more frequent than the idiopathic ones. Our proposal is to considered them as two individual entities. We propose to adopt the name of Post-traumatic sinus syndrome, or CDR syndrome (Catalfamo-De Rinaldis), for all cases that respect four specific diagnostic criteria reported into the text.

Sinus pericranii in the setting of a posterior fossa pilocytic astrocytoma: illustrative case.

BACKGROUND: Sinus pericranii (SP) is a rare vascular anomaly, with an uncertain etiology. Often discovered as superficial lesions, they can be primary or secondary in nature. Herein, we report a rare case of SP in the setting of a large posterior fossa pilocytic astrocytoma associated with a significant venous network. OBSERVATIONS: A 12-year-old male presented with acute clinical deterioration in extremis with a 2-month history of lethargy and headaches. Outside plain computed tomography imaging revealed a large posterior fossa cystic lesion, probably a tumor, with severe hydrocephalus. There was also a midline small skull defect at the opisthocranion, without visible vascular anomalies. An external ventricular drain was placed with rapid recovery. Contrast imaging revealed a large midline SP emanating from occipital bone with a large intraosseous, and subcutaneous venous plexus in the midline draining inferiorly into venous plexus around the craniocervical junction. A posterior fossa craniotomy without contrast imaging could have resulted in catastrophic hemorrhage. A small modified off-center craniotomy provided access to the tumor with a gross total excision. LESSONS: SP is a rare but significant phenomenon. Its presence does not necessarily preclude resection of underlying tumors, provided that a careful preoperative assessment of the venous anomaly is undertaken.

Prevalence of Maxillary Sinus Hypoplasia and Silent Sinus Syndrome: A Radiological Cross-Sectional Retrospective Cohort Study.

BACKGROUND: Maxillary sinus hypoplasia (MSH), associated with enophthalmos and hypoglobus in the silent sinus syndrome (SSS), is a poorly studied condition. The real incidence of MSH and SSS in the adult population is not known. Our study aims at estimating the radiological prevalence of MSH and identifying undiagnosed cases of SSS in a retrospective cohort. METHODS: A cross-sectional retrospective cohort study was performed in adults, without a history of maxillofacial surgery or trauma, undergoing head CT scans. A radiological database of 1012 consecutive scans was reviewed independently by two authors to identify patients with signs of MSH and SSS and associated findings (septal deviation, uncinate deviation, concha bullosa, sinus opacification, bony rarefaction, and pterygopalatine fossa enlargement). The findings of MSH and SSS were compared with radiological reports. RESULTS: 891 eligible CT scans were analyzed. MSH and SSS prevalences were 6.17% (n = 55) and 0,56% (n = 5), respectively. The maxillary sinus was normally or partially ventilated in 96.36% of MSH patients. Lateralization of the uncinate process was detected in about 50% of MSH patients, while a septal deviation towards the affected sinus was detected in 21.82%. In 20% of MSH scans, a concha bullosa was identified. Radiological reports identified a single MSH case. CONCLUSIONS: Our study confirmed the literature data on MSH prevalence, while it determined a precedently unknown prevalence for SSS, underestimated in the radiology reports. This prevalence needs further confirmation but suggests a routine accurate comparison of both maxillary sinuses in CT scans.

[Thrombosis and anatomic variants of intracranial dural venous sinuses]. / Tromboz i varianty stroeniya sinusov tverdoi mozgovoi obolochki.

A rather large number of different anatomic variants of intracranial dural venous sinuses are known, and they are found so often that interpreting them only as anomalies is unlikely, except for variations such as sinus aplasia or doubling, which can be attributed to anomalies of the venous system. Diameter reduction (hypoplasia) of the transverse and sigmoid sinuses is observed relatively often. In cases of dural sinuses anomalies, sinuses on the contralateral side are necessarily evaluated as the main source of compensation. When diagnosing dural sinus thrombosis, it is necessary to have a clear idea of the structure of the cerebral venous system, so as not to mistakenly accept hypoplasia or sinus aplasia for thrombosis. Our own experience is based on the results of a neuroimaging study of cerebral veins and venous sinuses in 103 patients (average age 35±10 years) with a tension-type headache. Hypoplasia of the transverse and sigmoid sinuses was detected in 21 (22.4%) cases. In 10 cases (6 men, 4 women), a connection was established between hypoplasia of the dural sinuses and thrombosis. Four patients, presented with hypoplasia of the right transverse and sigmoid sinuses and 6 patients with hypoplasia of the left transverse and sigmoid sinuses. Thrombosis developed on the side of sinus hypoplasia (9 patients) or on the contralateral side (1 patient). Early diagnosis of cerebral venous thrombosis is crucial because the use of anticoagulants reduces the risk of a poor prognosis, severe disability without an additional increase in the risk of brain hematomas progression.

Clinical significance of maxillary sinus hypoplasia in dentistry: A CBCT study.

BACKGROUND: The anatomy of the maxillary sinus is especially important for dentists due to the close proximity of the sinus to the maxillary posterior teeth. OBJECTIVES: The aim of the present study was to investigate the frequency of maxillary sinus pathology, anatomical variations, and the relationship between the tooth roots and the maxillary sinus by comparing a group with maxillary sinus hypoplasia (MSH) and a control group using cone-beam computed tomography (CBCT). MATERIAL AND METHODS: In the study, 69 CBCT images of 50 patients with MSH and 84 CBCT images of 49 patients without MSH were evaluated for pathology, and the presence of an accessory ostium, a septum and Haller cells in each maxillary sinus. RESULTS: The coincidence of pathology with MSH was 29%, and with non-hypoplastic maxillary sinuses it was 44% (p = 0.055). An accessory ostium was found in 14.5% of scans with MSH and in 39.3% of those without MSH (p = 0.001). Haller cells were found in 2.9% of the MSH cases, whereas their incidence in the control group was 23.8% (p = 0.000). The occurrence of a sinus septum was at the level of 4.3% in the group with MSH and 23.8% in the group without MSH (p = 0.001). CONCLUSIONS: The incidence of the relationship between the sinus wall and the posterior root apices was found smaller in the dentulous MSH patients. Also, the distance between the root apices and the sinus wall was longer in the dentulous MSH patients, and the vertical and horizontal alveolar bone was larger in the posteriorly edentulous MSH patients.

Sinus hypoplasia in the cystic fibrosis rat resolves in the absence of chronic infection.

BACKGROUND: Sinus hypoplasia is a hallmark characteristic in cystic fibrosis (CF). Chronic rhinosinusitis (CRS) is nearly universal from a young age, impaired sinus development could be secondary to loss of the cystic fibrosis transmembrane conductance regulator (CFTR) or consequences of chronic infection during maturation. The objective of this study was to assess sinus development relative to overall growth in a novel CF animal model. METHODS: Sinus development was evaluated in CFTR-/- and CFTR+/+ rats at 3 stages of development: newborn; 3 weeks; and 16 weeks. Microcomputed tomography (microCT) scanning, cultures, and histology were performed. Three-dimensional sinus and skull volumes were quantified. RESULTS: At birth, sinus volumes were decreased in CFTR-/- rats compared with wild-type rats (mean ± SEM: 11.3 ± 0.85 mm3 vs 14.5 ± 0.73 mm3 ; p < 0.05), despite similar weights (8.4 ± 0.46 gm vs 8.3 ± 0.51 gm; p = 0.86). CF rat weights declined by 16 weeks (378.4 ± 10.6 gm vs 447.4 ± 15.9 gm; p < 0.05), sinus volume increased similar to wild-type rats (201.1 ± 3.77 gm vs 203.4 ± 7.13 gm; p = 0.8). The ratio of sinus volume to body weight indicates hypoplasia present at birth (1.37 ± 0.12 vs 1.78 ± 0.11; p < 0.05) and showed an increase compared with CFTR+/+ animals by 16 weeks (0.53 ± 0.02 vs 0.46 ± 0.02; p < 0.05). Rats did not develop histologic evidence of chronic infection. CONCLUSION: CF rat sinuses are smaller at birth, but develop volumes similar to wild-type rats with maturation. This suggests that loss of CFTR may confer sinus hypoplasia at birth, but normal development ensues without chronic sinus infection.

Malignant Cerebral Swelling After Cranioplasty due to Ipsilateral Intracranial Vasculopathy: Case Report and Literature Review.

BACKGROUND: Cranioplasty is a well-established surgical operation that is used worldwide for patients with skull defects following decompressive craniectomy (DC). However, in some cases, potentially fatal complications may occur, such as malignant cerebral swelling after uneventful cranioplasty. CASE DESCRIPTION: We present a rare case of massive malignant ipsilateral cerebral swelling following uneventful titanium mesh cranioplasty due to rare ipsilateral intracranial vasculopathy confirmed by magnetic resonance angiography (MRA) and magnetic resonance venography (MRV). Fortunately, we performed titanium mesh explantation and extended DC in time, and the patient survived. Malignant cerebral swelling after uneventful cranioplasty is an unpredictable but fatal complication. Most reported cases have had an unfavorable prognosis. To the best of our knowledge, the mechanism was first confirmed by MRA and MRV, which demonstrated that the cerebral swelling was due to unilateral intracranial vasculopathy, including a rare ipsilateral intracranial internal carotid artery occlusion, as well as extremely thin lateral and sigmoid sinuses. CONCLUSIONS: Our case demonstrates for the first time that ipsilateral intracranial vasculopathy is a risk factor for malignant cerebral swelling after cranioplasty. Patients with traumatic brain injury with suspected intracranial vasculopathy should undergo a comprehensive vascular evaluation before cranioplasty to help prevent malignant cerebral swelling.

Single surgical step for endoscopic surgery and orbital reconstruction of a silent sinus syndrome.

Silent sinus syndrome (SSS) is an uncommon disease characterised by enophthalmos, caused by ipsilateral maxillary sinus atelectasis. The diagnosis is clinical with radiological confirmation. The treatment has two objectives: to regulate the aeration of the maxillary sinus through achieving normal nasal cavity drainage and to restore the orbital architecture. A case of SSS treated in our hospital in a single surgical intervention is reported.

Diagnosis and Treatment of Unilateral Maxillary Sinus Hypoplasia

Paranasal Sinus CT Scans Analysis of the Primary Maxillary Sinus Hypoplasia / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Identification of primary maxillary sinus hypoplasia (PMSH) is important diagnostically and therapeutically. Recently, the clinical significance of associated abnormalities in PMSH has been suggested. The aim of this study is to investigate the incidence of PMSH, their associated abnormalities and relationship of anatomical variations and paranasal sinusitis. MATERIALS AND METHODS: We evaluated radiologic diagnostic criteria of PMSH and analyzed the relationship of the anatomical variations of nasal cavity and paranasal sinuses in paranasal sinus CT scans, retrospectively. We measured the volume estimated ratio (VER) in PMSH cases. RESULTS: The incidence of unilateral and bilateral PMSH were 11 cases (5.9%) and 10 cases (5.3%). respectively. According to the Bolger's classification, there were 13 sites (41.9%) of type I with the mean VER of 0.71, 14 sites (45.2%) of type II with the mean VER 0.50, and 4 sites (12.9%) of type III with the mean VER of 0.27. The most common anatomical anomalies in both of the unilateral and bilateral PMSH were zygomatic (90.3%) and alveolar pneumatization (90.3%); the second most common abnormal finding was high maxillary sinus floor (77.4%). Of the anatomical variations, the frequency of uncinate process abnormalities (41.9%), paradoxical middle turbinate (32.3%) and Haller's cell (19.4%) were statistically significant. CONCLUSION: These results suggest that zygomatic and alveolar pneumatization, and high maxillary sinus floor are additionally important anatomical abnormalities associated with PMSH. Careful preoperative assessment of anatomical variations in the paranasal sinus CT scans may be essential to avoid incidental iatrogenic complications during functional endoscopic sinus surgery (FESS) or Caldwell-Luc operation in patients with PMSH.