Langerhan’s cell Histiocytosis of Sphenoid Sinus causing Vision Loss: A Case Report

Mani, Suresh; Thomas, Regi; Mathew, Regi; Michael, Rajiv

doi:10.31729/jnma.3959

Cited by 3 publications

(2 citation statements)

References 9 publications

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“…Aufgrund der raumfordernden Wirkung der Manifestation der LZH sind Affektionen benachbarter Strukturen wie z. B. dem Nervus opticus möglich [398].…”

Section: Langerhans-zell-histiozytoseunclassified

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Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

Sommer

2021

Laryngorhinootologie

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ZusammenfassungSeltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis stellen aufgrund der niedrigen Inzidenz und den daraus resultierend eingeschränkten diagnostischen Kriterien sowie therapeutischen Optionen eine Herausforderung dar. Der Schwellenwert, ab dem eine Erkrankung als selten zu werten ist, liegt bei höchstens 5 Betroffenen pro 10 000 Personen. Innerhalb dieser Erkrankungen gibt es allerdings extreme Schwankungen. So gehören einige seltene Erkrankungen, wie z.B. das invertierte Papillom, zu den regelmäßig diagnostizierten und therapierten Krankheitsbildern einer größeren Klinik für Hals-Nasen-Ohrenheilkunde. Andererseits liegen bei anderen seltenen Erkrankungen und Fehlbildungen weltweit deutlich unter 100 Fallberichte vor, was die Notwendigkeit einer Bündelung der verfügbaren Erfahrung zu Diagnostik und Therapie unterstreicht.Die vorliegende Arbeit gibt eine Übersicht zu seltenen Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis aus den übergeordneten Bereichen Erkrankungen / Syndrome des olfaktorischen Systems, Fehlbildungen der Nase und Nasennebenhöhlen, Belüftungs- und Funktionsstörungen sowie benigne und maligne Tumore. Die Einordnung sowie Angaben zu Diagnostik und Therapiemöglichkeiten wurden unter Berücksichtigung der aktuellen Literatur erstellt.

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“…Aufgrund der raumfordernden Wirkung der Manifestation der LZH sind Affektionen benachbarter Strukturen wie z. B. dem Nervus opticus möglich [398].…”

Section: Langerhans-zell-histiozytoseunclassified

“…In cases of affected frontal or maxillary sinus, swellings in the face may occur 397 . Due to the space-requiring effect of the manifestation of LCH, affections of neighboring structures such as the optic nerve are possible 398 .…”

Section: Rare Diseases Of the Nose The Paranasal Sinuses And The Snterior Skull Basementioning

confidence: 99%

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

Sommer

2021

Laryngorhinootologie

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Langerhans cell histiocytosis of the sella in a pediatric patient: case report with review of the literature

Orr,

Lesha,

Dugan

et al. 2024

Childs Nerv Syst

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Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Reisi

Raeissi

Khalilabad

et al. 2021

Orphanet J Rare Dis

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Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

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Langerhan’s cell Histiocytosis of Sphenoid Sinus causing Vision Loss: A Case Report

Cited by 3 publications

References 9 publications

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

Langerhans cell histiocytosis of the sella in a pediatric patient: case report with review of the literature

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

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