Top

BMC Ear, Nose and Throat Disorders

Published in:

Open Access 01-12-2017 | Case report

Lipoma of Columella with septal extension in Pai syndrome: report of a rare case

Authors: Surendra B. Patil, Shree Harsh

Published in: BMC Ear, Nose and Throat Disorders | Issue 1/2017

Abstract

Background

Lipoma in nasal region in a case of Pai syndrome is very rare. Very few cases of Pai syndrome with columellar lipoma with extension to septum and tip have been reported till date.

Case presentation

We report a very rare form of nasal lipoma in a case of Pai syndrome with extension to septum and tip in a 4 year old female child.

Conclusions

Pai syndrome with columellar lipoma with septal and tip extension should always be kept in mind when evaluating a swelling in the region of nasal tip, collumella and septum with notch in upper lip and corpus callosal lipoma.

Som PM, Scherl MP, Rao VM, Biller HF. Rare presentation of ordinary lipomas of the head and neck:a review. AJNR. 1986;7:657–64. 0195-6108/86/0704-0657.PubMed

Jung SN, Shin JW, Kwon H, Yim YM, et al. Fibrolipoma of the tip of nose. J Craniofac Surg. 2009;20(2):555–6.CrossRefPubMed

Deeplakshami T, Devan PP, Kavitha Ashok K, Rukma B. A rare case of spindle cell lipoma of the nose. J Surg Tech Case Rep. 2012;4(2):110–1.CrossRef

Abdulezz T, Allam K. Nasal Subcutaneous lipoma: a case report. Rhinology. 2008;46(2):151–2.

Funai MNS, Risola CF, Gomes LM, Pinto JA. Fibrolipoma of the nasal septum: an unusual pediatric case. Int J Pediatr Otorhinolaryngol. 2015;10(3). doi:10.1016/jpedex.2015.03.001.

Jacob A, Leah B, Donald L. Pai syndrome:median cleft lip, corpus callosum lipoma, fibroepithelial skin Tag. Eplasty. 2014;14:ic7. PMCID: PMC3961995.

Pai GS, Levkoff AH, Lethiser Jr RE. Median cleft of upperlip associated with lipomas of the central nervous system and cutaneous polyps. Am J Med Genet. 1987;26(4):921–4.CrossRefPubMed

Szeto C, Tewfik TL, Jewer D, Rideout A. Pai syndrome (median cleft palate, cutaneous nasal polyp, and midline lipoma of the corpus calosum): a case report and review of literature. Int J Pediatr Otorhinolaryngol. 2005;69(9):1247–52.CrossRefPubMed

Al-Mazrou KA, Al-Rekabi A, Alorainy IA, AL Kharifi T, Al-Serhani AM. Pai syndrome: a report of a case and review of literature. Int J Pediatr Otorhinolaryngol. 2001;61(2):149–53.CrossRefPubMed

10.

Blouet M, Belloy F, Jeanne PC, Leporrier N, Benoist G. Pai syndrome: challenging prenatal diagnosis and management. Pediatr Radiol. 2014;44(9):1184–7.CrossRefPubMed

11.

Castori M, Rinaldi R, Bianchi A, Caponetti A, Assumma M, Grammatico P. Pai syndrome : first patient with agenesis of the corpus callosum and literature review. Birth Defects Res A Clin Mol Teratol. 2007;79(10):673–9.CrossRefPubMed

12.

Abdelmaaboud M, Nimeri N. Pai syndrome: first reported case in Qutar and review of literature of previously published cases. BMJ Case Rep. 2012;2012:bcr0220125940. doi:10.1136/bcr-02-2012-5940.PubMedPubMedCentral

13.

Rundik SS, Zeres KA. A further patient with Pai syndrome with autosomal dominant inheritance? J Med Genet. 1994;31(6):497–8.CrossRef

14.

Mashima K, Mori Y, Minami K, Sakuda M, Sugahara T. A case of Pai syndrome. Plast Reconstr Surg. 1999;103(1):166–70.CrossRef

15.

Coban YK, Boran C, Omeroglu SA, Okur E. Pai syndrome: an adult patient with bifid nose and frontal hairline marker. Cleft Palate Craniofac J. 2003;40(3):325–8.CrossRefPubMed

16.

Guion-Almeda ML, Mellado C, Beltran C, Richieri C, Richieri CA. Pai syndrome: report of seven south American patients. Am J Med Genet. 2007;143A(24):3273–9.CrossRef

17.

Chousta A, Ville D, James I, Foray P, Bisch C, Depardon P, et al. Pericallosal lipoma associated with Pai syndrome: prenatal imaging findings. Ultrasound Obtet Gynecol. 2008;32(5):708–10. doi:10.1002/uog.6150.CrossRef

Title: Lipoma of Columella with septal extension in Pai syndrome: report of a rare case
Authors: Surendra B. Patil
Shree Harsh
Publication date: 01-12-2017
Publisher: BioMed Central
Published in: BMC Ear, Nose and Throat Disorders / Issue 1/2017
Electronic ISSN: 1472-6815
DOI: https://doi.org/10.1186/s12901-017-0035-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Lipoma of Columella with septal extension in Pai syndrome: report of a rare case

Abstract

Background

Case presentation

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2017

Reliability and validity study of Sino-nasal outcome test 22 (Thai version) in chronic rhinosinusitis

Is OperaVOX a clinically useful tool for the assessment of voice in a general ENT clinic?

Fluorescein sodium-guided surgery of parotid gland tumors as a technical advance

Glomangiomyoma of the neck in a child in Nepal: a rare case report and literature review

Evaluation of the safety and efficacy of a novel product for the removal of impacted human cerumen

Hibernoma: a rare case of adipocytic tumor in head and neck.