Blog - INTERNET MÉDICA

Síndrome de Gorlin-Goltz Online ( Gorlin-Goltz Syndrome)

Por Paulo Leite 25/12/2018

¨A síndrome de Gorlin-Goltz (SGG) ou síndrome do nevo basocelular, é uma desordem rara autossômica dominante. Apesar de hereditária, há casos de mutação espontânea. Caracteriza-se pela predisposição cancerígena e várias manifestações clínicas. A SGG associa-se a achados clínicos classificados em critérios maiores: CBCs mínimo dois ou um em menores de 20 anos, tumor odontogênico ceratocístico, pits palmoplantares, calcificação ectópica intracraniana, história familiar de SGG; e menores: anomalisas craniofaciais, macrocefalia, fenda labial ou palatina, bossa frontal, hipertelorismo, anomalias esqueléticas, fibroma ovariano, meduloblastoma. Define SGG com a presença de dois critérios maiores ou um maior e dois menores. O tratamento é multidisciplinar dependendo das manifestações clínicas do pacientes e requer constante vigilância a novos achados clínicos.¨

■ Síndrome Gorlin-Goltz: relato de caso

M C C Gozzano, M B C Gozzano, M L C Gozzano, J O A Gozzano

Revista da Facultade de Ciências Médicas de Sorocaba 2018, vol 20 (1)

http://revistas.pucsp.br/index.php/RFCMS/article/view/31384/pdf

■ Vismodegib-resistant basal cell carcinomas in basal cell nevus syndrome: clinical approach and genetic analysis

Sinx KAE, Roemen GMJM, van Zutven V, et al

JAAD Case Rep. 2018 Apr 30;4(5):408-411

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031482/

■ Medulloblastoma in a toddler with Gorlin syndrome

Mohamad G. Al-Rahawan, Sorleen Trevino, Roy Jacob et al

Proc (Bayl Univ Med Cent) 2018 Apr; 31(2): 216–218

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5914436/

■ Unilateral focal dermal hypoplasia (Goltz syndrome): case report and literature review

Sahar Alsharif, Sohad Hindi, Fay Khoja

Case Rep Dermatol. 2018 May-Aug; 10(2): 101–109

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5981586/

■ Multiple, multioculated, and recurrent keratocysts of the mandible and maxilla in association with Gorlin=Goltz (Nevoid basal-cell carcinoma) syndrome: a pediatric case report and follow-up over 5 years

P Santander, E M C Schwaibold, F Bremmer et al

Case Reports in Dentistry 2018, Article ID 7594840, 8 pages

https://www.hindawi.com/journals/crid/2018/7594840/cta/

■ Gorlin-Goltz syndrome

Betül Şereflican, Bengü Tuman, Murat Şereflican et al

Turk Pediatri Ars. 2017 Sep; 52(3): 173–177

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5644586/

■ Gorlin-Goltz syndrome: diagnosis and treatment options

Mendes-Abreu J, Pinto-Gouveia M, Tavares-Ferreira C, Brinca A, Vieira R.

Acta Med Port. 2017 May 31;30(5):418-421

https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/7521/5050

■ A multidisciplinary approach to the successful management of Gorlin syndrome

R N Mello, Z Khan, U Choudry

Journal of Surgical Case Reports 2017, vol 2017 (6): rjw224

https://academic.oup.com/jscr/article/2017/6/rjw224/3861673

■ Dental, dermatological and radiographic findings in a case of Gorlin-Goltz syndrome: report and review

Kumar Nilesh, Shivsagar Tewary, Sameer Zope, Jinesh Patel, Aaditee Vande

Pan Afr Med J. 2017; 27: 96

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554634/

■ Basal cell nevus syndrome (Gorlin-Goltz syndrome): genetic predisposition, clinical picture and treatment

Henryk Witmanowski, Paweł Szychta, Katarzyna Błochowiak et al

Postepy Dermatol Alergol. 2017 Aug; 34(4): 381–38

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5560190/

■ Keratocystic odontogenic tumors related to Gorlin-Goltz syndrome: a clinicopathological study

Mohammed Israr Ul Khaliq, Ajaz A. Shah, Irshad Ahmad et al

J Oral Biol Craniofac Res. 2016 May-Aug; 6(2): 93–100

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4862113/

■ Syndrome in question: Gorlin-Goltz syndrome

Ribeiro, Pauline Lyrio et al.

An. Bras. Dermatol., Aug 2016, vol.91, no.4, p.541-543

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016000400541&lng=en&nrm=iso
■ Gorlin-Goltz syndrome: an often missed diagnosis

Ninan Thomas, Sankar V. Vinod, Arun George, Aabu Varghese

Ann Maxillofac Surg. 2016 Jan-Jun; 6(1): 120–124

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979327/

■ Maxillary odontogenic keratocyst: a clinical case report.

Freitas, Daniel Antunes et al.

RGO, Rev. Gaúch. Odontol., Dec 2015, vol.63, no.4, p.484-48

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1981-86372015010500484&lng=en&nrm=iso&tlng=en

■ Gorlin-Goltz syndrome: case report and literature review

M Ramesh, R Krishnan, P Chalakkal, G Paul

J Oral Maxillofac Patho 2015, vol 19 (2): 267

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4611943/

■ Gorlin-Goltz syndrome in twin brothers: an unusual occurrence with review of the literature

Sonal Anchlia, Siddharth Vyas, Sumit Bahl, Vipul Nagavadiya

BMJ Case Rep. 2015; 2015: bcr201521160

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4551002/

■ A case of Gorlin-Goltz syndrome presented with psychiatric features

Amir Mufaddel, Mouza AlSabousi, Badr Salih, Ghanem AlHassani, Ossama T. Osman

Behav Neurol. 2014; 2014: 830874

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4006609/

■ Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study

Bomfin, Luana Eschholz et al.

Braz. J. Oral Sci., Mar 2013, vol.12, no.1, p.23-2

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1677-32252013000100006&lng=en&nrm=iso

■ Radiological features of familial Gorlin-Goltz syndrome

Shruthi Hegde, Shishir Ram Shetty

Imaging Sci Dent. 2012 Mar; 42(1): 55–60

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3314839/

■ Gorlin-Goltz syndrome – a medical condition requiring a multidisciplinary approach

Małgorzata Kiwilsza, Katarzyna Sporniak-Tutak

Med Sci Monit. 2012; 18(9): RA145–RA153

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3560657/

■ Basal cell carcinomas in Gorlin syndrome: a review of 202 patients

E A Jones, M I Sajid, A Shendon, D Gareth Evans

Journal of Skin Cancer 2011, Article ID 217378, 6 pages

https://www.hindawi.com/journals/jsc/2011/217378/

■ Early diagnosis of Gorlin-Goltz syndrome

Ana R Casaroto, Daniela CN Rocha Loures, Eduardo Moreschi et al

Head Face Med. 2011; 7: 2

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3035593/

■ Basal cell nevus syndrome: guidelines for early detection

G J Bitar, C K Herman, M I Dahman et al

Am Fam Physician 2002, vol 65 (12): 2501-2505

https://www.aafp.org/afp/2002/0615/p2501.html

by Dr Paulo Fernando Leite

■ Cardiologia/Prevenção Cardiovascular

■ Estratificação de Risco Cardiovascular

Av Contorno 8351 – Conj 01

Belo Horizonte/MG/Brasil

Tel: 31 32919216 2917003 3357229

(- consulta particular –)

CRMMG: 7026

Email: pfleite1873@gmail.com

Síndrome de Dunbar Online (Dunbar Syndrome)

Por Paulo Leite 23/12/2018

¨A síndrome de Dunbar ou síndrome da compressão do tronco celíaco ou síndrome do ligamento arqueado mediano é uma condição clínica infrequente, com poucos critérios para diagnóstico e com fisiopatologia obscura. Está usualmente associada à compressão extrínseca do tronco celíaco por banda fibrosas do diafragma e fibras neurais simpáticas, próximo a sua emergência da aorta. Alguns pacientes referem manifestações clínicas, tais como dor abdominal pós-prandial, perda de peso e vômito. O tratamento primário é a divisão cirúrgica das fibras do ligamento arqueado mediano. O acesso cirúrgico tradicional tem sido através de laparotomia abdominal superior. O acesso laparoscópico pode ser empregado com sucesso para o tratamento da síndrome de Dunbar. ¨

■ Dunbar syndrome: a rare presentation of abdominal angina treated by revascularization of the celiac artery by endovascular stenting

R Shetty, K Subramanyam, C S Jacoob

International Journal of Research in Medical Sciences 2018, vol 6 (6): 2169-2171

https://www.msjonline.org/index.php/ijrms/article/viewFile/4875/3987

■ Dunbar syndrome: a rare case of angina abdominal

Y Akcali, R Atabev

Am J Cardiol 2018, vol 121 (8): e152-e153

https://www.ajconline.org/article/S0002-9149(18)30699-4/fulltext

■ Median arcuate ligament syndromme: It is not always gastritis

A Kuruvilla, G Murtaza, A Cheema

Journal of Investigative Medicine High Impact – Case Reports set 2017

https://journals.sagepub.com/doi/full/10.1177/2324709617728750

■ Open vascular treatment of median arcuate ligament syndrome

M Duran, F Simon, N Ertas et AL

BMC Surgery 2017, vol 17: 95

https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-017-0289-8

■ Hybrid (laparoscopy + stent) treatment of celiac trunk compression syndrome (Dunbar syndrome, median arcuate ligament syndrome (MALS))

Maciej Michalik, Natalia Dowgiałło-Wnukiewicz, Paweł Lech et al

Wideochir Inne Tech Maloinwazyjne. 2016 Dec; 11(4): 236–239

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5299080/

■ Surgical treatment of Dunbar syndrome

França, Luís Henrique Gil and Mottin, Carla

vasc. bras., Mar 2013, vol.12, no.1, p.57-61

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1677-54492013000100012&lng=pt&nrm=iso&tlng=en

■ Laparoscopic treatment of Dunbar syndrome: a case report

Orlando Jorge M. Torres, Ozimo Pereira Gama-Filho, Camila Cristina Salazar Torres et al

Int J Surg Case Rep. 2017; 37: 230–232

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5524307/

■ Open vascular treatment of median arcuate ligament syndrome

Mansur Duran, Florian Simon, Neslihan Ertas, Hubert Schelzig, Nikolaos Floros

BMC Surg. 2017; 17: 95

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5575896/

■ Tratamento laparoscópico da síndrome de compressão do tronco celíaco: relato de caso

J C U Coelho, J C da Silva, M F Domingos et al

ABCD Arq Bras Cir Dig 2015, vol 28 (4): 295

http://www.scielo.br/pdf/abcd/v28n4/pt_0102-6720-abcd-28-04-00295.pdf

■ Laparoscopic decompression as treatment for median arcuate ligament syndrome

M Rubinkiewicz, PK Ramakrishnan, BM Henry, J Roy, A Budzyski

Ann R Coll Surg Engl. 2015 Sep 1; 97(6): e96–e99

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5126249/

■ Celiac artery compression syndrome: an experience in a single institution in Taiwan

J Chou, C Lin, C Feng et al

Gastroenterol Res Prac 2012: 2012: 935721

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3439958/

■ Median arcuate ligament syndrome in a patient with Crohn´s disease

Alessandro Sturiale, Giovanni Alemanno, Francesco Giudici et al

Int J Surg Case Rep. 2013; 4(4): 399–402

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3604703/

■ Median arcuate ligament syndrome in the pediatric population

Grace Z. Mak, Christopher Speaker, Kristen Anderson et al

J Pediatr Surg. 2013 Nov; 48(11): 2261–2270

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3896126/

■ The median arcuate ligament syndrome: a mimicker of mesenteric vasculitis

J C Kay, R A Arroyo

Military Medicine 2013, vol 8: e967-e969

https://academic.oup.com/milmed/article/178/8/e967/4259713