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Microcornea-glaucoma-absent frontal sinuses syndrome

MedGen UID:
331860
•Concept ID:
C1834935
•
Disease or Syndrome
Synonyms: Hereditary microcornea, glaucoma, and absent frontal sinuses; Microcornea, glaucoma, and absent frontal sinuses
 
Monarch Initiative: MONDO:0007992
OMIM®: 156700
Orphanet: ORPHA2536

Definition

A rare developmental defect during embryogenesis syndrome characterized by the association of microcornea, glaucoma and frontal sinus hypoplasia. Thick palmar skin and torus palatinus have also been reported. There have been no further descriptions in the literature since 1995. [from ORDO]

Clinical features

From HPO
Absent frontal sinuses
MedGen UID:
343405
•Concept ID:
C1855669
•
Finding
Aplasia of frontal sinus.
See: Feature record | Search on this feature
Glaucoma
MedGen UID:
42224
•Concept ID:
C0017601
•
Disease or Syndrome
Glaucoma refers loss of retinal ganglion cells in a characteristic pattern of optic neuropathy usually associated with increased intraocular pressure.
See: Feature record | Search on this feature
Microcornea
MedGen UID:
78610
•Concept ID:
C0266544
•
Congenital Abnormality
A congenital abnormality of the cornea in which the cornea and the anterior segment of the eye are smaller than normal. The horizontal diameter of the cornea does not reach 10 mm even in adulthood.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicrocornea-glaucoma-absent frontal sinuses syndrome
Follow this link to review classifications for Microcornea-glaucoma-absent frontal sinuses syndrome in Orphanet.

Professional guidelines

PubMed

Orbital congestion complicating treatment of cerebral vascular anomalies.
Levin MH, Moss HE, Pineles SL, Bagley LJ, Heuer GG, Zager EL, Balcer LJ, Galetta SL, Vagefi MR
World Neurosurg 2014 Jul-Aug;82(1-2):239.e13-7. Epub 2013 Jan 28 doi: 10.1016/j.wneu.2013.01.093. PMID: 23369940Free PMC Article
Nasal and sinus endoscopy for medical management of resistant rhinosinusitis, including postsurgical patients.
Tichenor WS, Adinoff A, Smart B, Hamilos DL
J Allergy Clin Immunol 2008 Apr;121(4):917-927.e2. Epub 2007 Nov 5 doi: 10.1016/j.jaci.2007.08.065. PMID: 17981318
Nasal and sinus pain: current diagnosis and treatment.
Kennedy DW, Loury MC
Semin Neurol 1988 Dec;8(4):303-14. doi: 10.1055/s-2008-1041394. PMID: 3074426

Recent clinical studies

Etiology

Have There Been any Changes in the Epidemiology and Etiology of Maxillofacial Trauma During the COVID-19 Pandemic? An Italian Multicenter Study.
Salzano G, Dell'Aversana Orabona G, Audino G, Vaira LA, Trevisiol L, D'Agostino A, Pucci R, Battisti A, Cucurullo M, Ciardiello C, Barca I, Cristofaro MG, De Riu G, Biglioli F, Valentini V, Nocini PF, Califano L
J Craniofac Surg 2021 Jun 1;32(4):1445-1447. doi: 10.1097/SCS.0000000000007253. PMID: 33229987Free PMC Article
Saethre-Chotzen syndrome: long-term outcome of a syndrome-specific management protocol.
Den Ottelander BK, Van Veelen MC, De Goederen R, Van De Beeten SD, Dremmen MH, Loudon SE, Versnel SL, Van Den Ouweland AM, Van Dooren MF, Joosten KF, Mathijssen IM
Dev Med Child Neurol 2021 Jan;63(1):104-110. Epub 2020 Sep 9 doi: 10.1111/dmcn.14670. PMID: 32909287Free PMC Article
Sino-Nasal Anatomical Variations in Rhinogenic Headache Pathogenesis.
Sollini G, Mazzola F, Iandelli A, Carobbio A, Barbieri A, Mora R, Peretti G
J Craniofac Surg 2019 Jul;30(5):1503-1505. doi: 10.1097/SCS.0000000000005239. PMID: 31299754
Fronto-ethmoidal osteoma. Open treatment.
Reale G, Ungari C, Riccardi E, Calvani F, Quarato D, Rinna C, Filiaci F
Ann Ital Chir 2014 May-Jun;85(3):214-8. PMID: 25073747
Kartagener's Syndrome.
Dhar DK, Ganguly KC, Alam S, Hossain A, Sarker UK, Das BK, Haque MJ
Mymensingh Med J 2009 Jan;18(1):75-9. PMID: 19182755

Diagnosis

Frontal osteomyelitis post-COVID-19 associated mucormycosis.
Kulkarni P, Beeraka D, Tanwar M, Kim U, Ganesan RM, Saini P
Indian J Ophthalmol 2023 Jul;71(7):2906-2910. doi: 10.4103/IJO.IJO_3117_22. PMID: 37417145Free PMC Article
Ethmoidal silent sinus syndrome after nasal swab test.
Ribeiro SPP, Loureiro RM, Gil JM, Dutra DL, Gomes RLE, Daniel MM
Neuroradiology 2022 Jan;64(1):205-207. Epub 2021 Nov 13 doi: 10.1007/s00234-021-02856-y. PMID: 34773478Free PMC Article
Clinical pearls.
Alexander L
Optom Vis Sci 2015 Jun;92(6):736-7. doi: 10.1097/OPX.0000000000000614. PMID: 26001895
The olfactory system.
Leboucq N, Menjot de Champfleur N, Menjot de Champfleur S, Bonafé A
Diagn Interv Imaging 2013 Oct;94(10):985-91. Epub 2013 Aug 7 doi: 10.1016/j.diii.2013.06.006. PMID: 23932763
Kartagener's Syndrome.
Dhar DK, Ganguly KC, Alam S, Hossain A, Sarker UK, Das BK, Haque MJ
Mymensingh Med J 2009 Jan;18(1):75-9. PMID: 19182755

Therapy

Sino-Nasal Anatomical Variations in Rhinogenic Headache Pathogenesis.
Sollini G, Mazzola F, Iandelli A, Carobbio A, Barbieri A, Mora R, Peretti G
J Craniofac Surg 2019 Jul;30(5):1503-1505. doi: 10.1097/SCS.0000000000005239. PMID: 31299754
Headache Attributed to Airplane Travel: A Review of Literature.
Nierenburg H, Jackfert K
Curr Pain Headache Rep 2018 Jun 14;22(7):48. doi: 10.1007/s11916-018-0701-9. PMID: 29904804
Facial Fractures: Pearls and Perspectives.
Chaudhry O, Isakson M, Franklin A, Maqusi S, El Amm C
Plast Reconstr Surg 2018 May;141(5):742e-758e. doi: 10.1097/PRS.0000000000004340. PMID: 29697631
Kartagener's Syndrome.
Dhar DK, Ganguly KC, Alam S, Hossain A, Sarker UK, Das BK, Haque MJ
Mymensingh Med J 2009 Jan;18(1):75-9. PMID: 19182755
Nasal and sinus endoscopy for medical management of resistant rhinosinusitis, including postsurgical patients.
Tichenor WS, Adinoff A, Smart B, Hamilos DL
J Allergy Clin Immunol 2008 Apr;121(4):917-927.e2. Epub 2007 Nov 5 doi: 10.1016/j.jaci.2007.08.065. PMID: 17981318

Prognosis

Cerebral Venous Sinus Thrombosis in a Patient With Alcohol Withdrawal Symptoms: A Case Report and Literature Review.
Vashi AP, Boi K, Bhatia A, Johnson R, Brown CE Jr
Neurologist 2023 Jul 1;28(4):266-269. doi: 10.1097/NRL.0000000000000477. PMID: 36449713
Middle turbinate resection is unlikely to cause empty nose syndrome in first year postoperatively.
Law RH, Ahmed AM, Van Harn M, Craig JR
Am J Otolaryngol 2021 Jul-Aug;42(4):102931. Epub 2021 Jan 26 doi: 10.1016/j.amjoto.2021.102931. PMID: 33550027
Supplementary motor area syndrome after surgery for parasagittal meningiomas.
Berg-Johnsen J, Høgestøl EA
Acta Neurochir (Wien) 2018 Mar;160(3):583-587. Epub 2018 Jan 23 doi: 10.1007/s00701-018-3474-3. PMID: 29362933
Fronto-ethmoidal osteoma. Open treatment.
Reale G, Ungari C, Riccardi E, Calvani F, Quarato D, Rinna C, Filiaci F
Ann Ital Chir 2014 May-Jun;85(3):214-8. PMID: 25073747
Nasal reconstruction for maxillonasal dysplasia.
Holmes AD, Lee SJ, Greensmith A, Heggie A, Meara JG
J Craniofac Surg 2010 Mar;21(2):543-51. doi: 10.1097/SCS.0b013e3181d024b0. PMID: 20216441

Clinical prediction guides

Sinus computed tomography findings in patients with COVID-19.
Sumi DV, Loureiro RM, Collin SM, Deps PD, Bezerra LL, Gomes RLE, Daniel MM
Einstein (Sao Paulo) 2021;19:eAO6255. Epub 2021 Mar 15 doi: 10.31744/einstein_journal/2021AO6255. PMID: 33729288Free PMC Article
Sino-Nasal Anatomical Variations in Rhinogenic Headache Pathogenesis.
Sollini G, Mazzola F, Iandelli A, Carobbio A, Barbieri A, Mora R, Peretti G
J Craniofac Surg 2019 Jul;30(5):1503-1505. doi: 10.1097/SCS.0000000000005239. PMID: 31299754
Supplementary motor area syndrome after surgery for parasagittal meningiomas.
Berg-Johnsen J, Høgestøl EA
Acta Neurochir (Wien) 2018 Mar;160(3):583-587. Epub 2018 Jan 23 doi: 10.1007/s00701-018-3474-3. PMID: 29362933
The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults.
Ayas ZÖ, Asil K, Öcal R
Neurol Sci 2017 Oct;38(10):1823-1828. Epub 2017 Jul 21 doi: 10.1007/s10072-017-3074-7. PMID: 28733757
Radiological imaging findings of Dyke-Davidoff-Masson syndrome.
Gökçe E, Beyhan M, Sade R
Acta Neurol Belg 2017 Dec;117(4):885-893. Epub 2017 Apr 3 doi: 10.1007/s13760-017-0778-7. PMID: 28374239

Recent systematic reviews

Global status of neglected human Linguatula infection: a systematic review of published case reports.
Tabaripour R, Keighobadi M, Sharifpour A, Azadeh H, Shokri A, Banimostafavi ES, Fakhar M, Abedi S
Parasitol Res 2021 Sep;120(9):3045-3050. Epub 2021 Aug 3 doi: 10.1007/s00436-021-07272-y. PMID: 34342683
Prevalence and associated risk factors of Linguatula serrata infection in definitive and intermediate hosts in Iran and other countries: A systematic review.
Hajipour N, Tavassoli M
Vet Parasitol Reg Stud Reports 2019 Apr;16:100288. Epub 2019 Apr 10 doi: 10.1016/j.vprsr.2019.100288. PMID: 31027598
Computerized tomographic evaluation of supraorbital notches and foramen in patients with frontal migraine headaches and correlation with clinical symptoms.
Pourtaheri N, Guyuron B
J Plast Reconstr Aesthet Surg 2018 Jun;71(6):840-846. Epub 2018 Feb 14 doi: 10.1016/j.bjps.2018.01.040. PMID: 29526447
Considerations in the management of giant frontal mucoceles with significant intracranial extension: A systematic review.
Stokken J, Wali E, Woodard T, Recinos PF, Sindwani R
Am J Rhinol Allergy 2016 Jul;30(4):301-5. doi: 10.2500/ajra.2016.30.4323. PMID: 27456600

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