Lipoma of Columella with septal extension in Pai syndrome: report of a rare case
© The Author(s). 2017
Received: 30 October 2016
Accepted: 31 January 2017
Published: 6 February 2017
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.
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.
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.
KeywordsColumella Lipoma Pai syndrome Case report
Lipoma is the most common mesenchymal origin tumor. They are commonly found in trunk, neck, upper and lower extremities. Head and neck region makeupto 13% of lipomas . In the head and neck region, they occur most commonly in the posterior region. Lipoma in the nasal region is very rare. In the nose, lipoma has been reported in the tip , nasoalar crease , and in the vestibule . We are presenting a case of nasal lipoma involving columella, septum and tip in a case of Pai syndrome.
Parents of a 4 year old female child presented her with deformity of the nose and difficulty in breathing from right side of the nose for 1 year. She developed the deformity as a small swelling at the tip of the nose of about 0.5 cm which increased progressively to the size of about 1 cm in 1 year. There was no history of trauma, fever, sudden increase in size or pain associated with the swelling. There was no association of similar swelling in any family member. Child was immunised and had achieved milestones appropriate for her age. Parents of the child wanted removal of the swelling as it was a cause of social embarrassment for the child and she had difficulty in breathing from right nostril. The child had an identical twin who was operated for intususseption at 6 months of age.
Nose was opened with stair step incision in the collumella for open rhinoplasty. This incision was chosen over sublabial or septal incision in view of better operative view in the case of former incision. Fatty tissue was found over the left lower lateral cartilage. Right lower lateral cartilage was found to be deficient. Lipoma extended till the middle of collumella on the right of middle crus and right part of the caudal septum. It was excised in toto and specimen was sent for histopathological examination.
Lipoma are found mostly in 5th to 6th decade of life . It is the most common soft tissue tumor in adults. They are uncommon in paediatric population. Cases of nasal lipoma have been reported in the past in adults. There may be many causes of nasal swelling in paediatric age group. Differentials include abscess, polyps, posttraumatic deformity, hematoma and congenital anomalies like teratoma and hemangioma. Septal lipoma has been reported in paediatric population in a case of Pai syndrome and in neonate with fibrolipoma .
Many liopomas of the nasal and paranasal sinuses have been reported. Lipoma have been reported in oral cavity, hypopharynx, parotid, Retropharyngeal area and larynx . Diagnosis is clinical in subcutaneous lipoma supported by fine needle aspiration cytology. It may be supplemented with computed tomography which shows homogenous low attenuation mass between −65 and −125 hounsfield units. Capsule of lipoma is not distinct.
Midline cleft of lip along with nasal polyps and corpus callosal lipoma has been described in Pai syndrome . The syndrome was reported for the first time in 1987. Since then very few cases have been reported. It is a rare developmental anomaly of face. Median cleft of lip is rare and found in 0.2% of patients with orofacial clefts . Classicaly Pai syndrome is associated with complete midline cleft of the upper lip, cutaneous polyps and central nervous system lipomas . Hernia of Inguinal region, cryptorchidism along with clinodactyly have also been reported. Double frenulum of upper lip with median alveolar cleft  has been reported in Pai syndrome. It has been once reported in a twin  of Arabian descent.
Prenatal diagnosis can be done with the help of sonography and MRI . Agenesis of corpus callosum with hemartomatous mass in nostril in Pai syndrome has been reported in a 1 month male child . Hypopigmented fundi with pigmented rings in the optic disc and hypopigmented macule with severe midline cleft of lip and palate in a child of Arabian descent  has been reported from Qutar. Heriditary association of Pai syndrome and presentation of coloboma of right iris , with conjunctival lipoma of right eye , with bifid nose and frontal alopecia  and de novo reciprocal translocation have been reported . Prenatal counselling following prenatal detection of pericallosal lipoma should be done .
Nasal lipoma in pediatric age group in association with Pai syndrome in a twin is very rare. With proper knowledge of differentials and appropriate examination supported by cytology and imaging studies, diagnosis can be made. Surgical approach depends upon the position of the lipoma. Open rhinoplasty was the answer in our case.
Professor S.M. Kale. We acknowledge him for his valuable guidance and advice on the subject.
In preparation of manuscript, management of patient pre, intra and post operative period both the authors SBP and SH have contributed. The article was made by both the authors. The final version of the manuscript was also read by both the authors and approved by them.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent for use of patient’s data and images was taken from the parents of the patient for publication of this case report. A copy of the written consent is available for review by the editor of this journal.
Ethics approval and consent to participate
Ethics approval was taken by institutional ethics committee at Government Medical College, Nagpur, India and written informed consent was taken by the parents of the patient.
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- 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.PubMedGoogle Scholar
- Jung SN, Shin JW, Kwon H, Yim YM, et al. Fibrolipoma of the tip of nose. J Craniofac Surg. 2009;20(2):555–6.View ArticlePubMedGoogle Scholar
- 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.View ArticleGoogle Scholar
- Abdulezz T, Allam K. Nasal Subcutaneous lipoma: a case report. Rhinology. 2008;46(2):151–2.Google Scholar
- 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.Google Scholar
- 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.View ArticlePubMedGoogle Scholar
- 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.View ArticlePubMedGoogle Scholar
- 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.View ArticlePubMedGoogle Scholar
- Blouet M, Belloy F, Jeanne PC, Leporrier N, Benoist G. Pai syndrome: challenging prenatal diagnosis and management. Pediatr Radiol. 2014;44(9):1184–7.View ArticlePubMedGoogle Scholar
- 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.View ArticlePubMedGoogle Scholar
- 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.PubMedPubMed CentralGoogle Scholar
- Rundik SS, Zeres KA. A further patient with Pai syndrome with autosomal dominant inheritance? J Med Genet. 1994;31(6):497–8.View ArticleGoogle Scholar
- Mashima K, Mori Y, Minami K, Sakuda M, Sugahara T. A case of Pai syndrome. Plast Reconstr Surg. 1999;103(1):166–70.View ArticleGoogle Scholar
- 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.View ArticlePubMedGoogle Scholar
- 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.View ArticleGoogle Scholar
- 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.View ArticleGoogle Scholar