2021, Number 02
<< Back Next >>
Ginecol Obstet Mex 2021; 89 (02)
Proteus syndrome and pregnancy. A case report
Peña-Vega CJ, Ortiz-Jarillo J, Zavala-Barrios B
Language: Spanish
References: 31
Page: 151--159
PDF size: 237.50 Kb.
ABSTRACT
Background: Proteus syndrome is a rare, congenital, hamartomatous disease that
can affect all the structures of the body. This disorder is not inherited. There are no case
reports in the literature that show its sexual and reproductive implications.
Clinical case: 21-year-old woman, primiparous, diagnosed with Proteus syndrome
at 6 years of age, due to early menarche. He received treatment with growth hormone
inhibitors, followed by a multidisciplinary team. In adolescence, the diagnosis of myopia
and peripheral venous insufficiency was established. In consensus, the interruption of
pregnancy by abdominal route, use of medium compression media and acetylsalicylic
acid was suggested. At week 35 of the pregnancy, she had preeclampsia, with severity
criteria (175-123 mmHg), for which she began treatment with crystalloid solutions,
antihypertensives and Zuspan, with a reaction and hemodynamic stabilization. The
fetus had anhydramnios and redistribution of hemodynamic flows. Seven days after the
obstetric event, the antihypertensive drugs were withdrawn and continued monitoring
with the multidisciplinary team. The mother and daughter were released from the
hospital without apparent complications.
Conclusions: Vascular malformations and alterations of adipose tissue suggest a
risk factor that alters regulation in angiogenesis and venous insufficiency is a promoter
of thrombosis; in turn, this can be considered a risk of preeclampsia, so early multidisciplinary
treatment is suggested to avoid complications. So, more cases must be
reported and collected to accept this hypothesis.
REFERENCES
Guerra-Tapia A, Rodríguez-Vázquez M. Síndrome Proteus. Piel 2001; 16: 248-52. https://doi.org/10.1016/S0213- 9251(01)72456-2
Graetz I. Über einen Fall von sogenannter “totaler halbseitiger Körperhypertrophie”. Z. Kinder-Heilk 1928; 45: 381-403. https://doi.org/10.1007/BF02248685
Lal N, Bandyopadhyay D, Sarkar AK. Unilateral hypertrophic skin lesions, hemimegalencephaly, and meningioma: The many faces of Proteus syndrome. Indian Dermatol Online. J 2015;6 (5): 348-51. https://doi.org/10.4103/2229- 5178.164477.
Carbajal LR, Navarrete MJ. Editorial: Enfermedades raras. Acta Pediatr Mex 2015; 36: 369-73. https://ojs.actapediatrica. org.mx/index.php/APM/article/view/1057
Child FJ, Werring DJ, Vivier EW. Proteus syndrome: diagnosis in adulthood. Br J Dermatol 1998; 139: 132-136. https:// doi.org/10.1046/j.1365-2133.1998.02330.x
Rocha R, Estrella M, Amaral D, Barbosa A, et al. Proteus syndrome. An Bras Dermatol. 2017; 92 (5): 717-20. https:// doi.org/10.1590/abd1806-4841.20174496
Redondo P. Malformaciones vasculares (I). Concepto, clasificación, fisiopatogenia y manifestaciones clínicas. Actas Dermo-Sifiliogr. 2007; 98 (3): 141-58. https://doi. org/10.1016/S0001-7310(07)70038-8
Sánchez López M, Martínez Fernández R, Santamaría Carro A. Manifestaciones oculares en el síndrome de Proteus. Arch Soc Esp Oftalmol. 2007; 82 (3): 175-8. http://scielo.isciii.es/pdf/aseo/v82n3/comunicacion3. pdf?origin=publication_detail
Capurro NJ, Carignano AM, Ottino A. Proliferación fibrosa cerebriforme, correlación con el síndrome de Proteus. Patología. 2008; 46 (4): 351-4.
Bennàsar A, Ferrando J. Síndromes de neoplasias múltiples familiares con manifestaciones cutáneas. Med Cutan Iber Lat Am. 2009; 37 (2): 71-8. https://es.scribd.com/ document/394007641/tumores-familiares-con-manifestaciones- cutaneas-pdf
Say B, Carpenter NJ. Report of a case resembling the Proteus syndrome with a chromosome abnormality. Am J Med Genet. 1988; 31: 987-89. https://doi.org/10.1002/ ajmg.1320310434
Vaughn RY, Selinger AD, Howell CG, Parrish RA, et al. Proteus syndrome: diagnosis and surgical management. J Pediatr Surg. 1993; 28: 5-10. https://doi.org/10.1016/ s0022-3468(05)80344-6
Hamm H. Cutaneous mosaicism of lethal mutations. Am J Med Genet. 1999; 85: 342-345. https://doi. org/10.1002/(SICI)1096-8628(19990806)85:4<342::AID AJMG6>3.0.CO;2-8
Happle R. Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. J Am Acad Dermatol. 1987; 16: 899- 906 https://doi. org/10.1016/s0190-9622(87)80249-9
Bouzas EA, Krasnewich D, Koutroumanidis M, Papadimitriou A, et al. Ophthalmologic examination in the diagnosis of Proteus syndrome. Ophthalmol. 1993; 100: 334-38. https://doi.org/10.1016/s0161-6420(93)31645-3
Newman B, Urbach AH, Orenstein D, Dickman PS. Proteus syndrome: emphasis on the pulmonary manifestations. Pediatr Radiol. 1994; 24: 189-93. https://doi.org/10.1007/ BF02012188
Eberhard DA. Two years old boy with Proteus syndrome and fatal pulmonary thromboembolism. Pediatr Pathol. 1994; 14: 771-79. https://doi.org/10.3109/15513819409037674
Bale PM, Watson G, Collins F. Pathology of osseous and genitourinary lesions of Proteus syndrome. Pediatr Pathol. 1993; 13: 797-809. https://doi.org/10.3109/15513819309048266
Del Rosario Barona-Mazuera M, Hidalgo-Galván LR, De la Luz Orozco-Cobarrubias M, Duran-McKinster C, et al. Proteus syndrome: mew findings in seven patients. Pediatr Dermatol. 1997; 14: 1-5. https://doi. org/10.1111/j.1525-1470.1997.tb00417.x
Dietrich RB, Glidden DE, Roth GM, Martin RA, e al. The Proteus syndrome: CNS manifestations. Am J Neuroradiol. 1998; 19: 987-90.
Wiedemann HR, Burgio GR, Aldenhoff P, Kunze J, et al. The Proteus syndrome. Eur J Pediatr. 1983; 140: 5-12. https:// doi.org/10.1007/BF00661895.
Clark RD, Donnai D, Rogers J, Cooper J, et al. Proteus syndrome: an expended phenotype. Am J Med Genet. 1987; 27: 99-117. https://doi.org/10.1002/ajmg.1320270111
Costa T, Fitch N, Azouz EM. Proteus syndrome: report of two cases with pelvic lipomatosis. Pediatrics 1985; 76: 984-89.
Samlaska CP, Levin SW, James WD, Benson PM, et al. Proteus syndrome. Arch Dermatol. 1989; 125: 1109-1114. https://doi.org/10.1001/archderm.1989.01670200085015
Hotamisligil GS. Proteus syndrome and hamartoses with overgrowth. Dysmorphol Clin Genet. 1990; 4: 87-102.
DeLone DR, Brown WD, Gentry LR. Proteus syndrome: craniofacial and cerebral MRI. Neuroradiol. 1999; 41: 840-43. https://doi.org/10.1007/s002340050853
Biesecker LG, Happle R, Mulliken JB, Weksberg R, et al. Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet. 1999; 84 (5): 389-95. https://doi.org/10.1002/(SICI)1096 8628(19990611)84:5<389::aid-ajmg1>3.0.co;2-o
Schnake Ch, Vejar L, Solar M, Ibanez R, et al. Síndrome Proteus: Contribución a su delineación clínica. Rev Chil Pediatr. 1986; 57 (6): 585-94. http://dx.doi.org/10.4067/ S0370-41061986000600026
Sapp JC, Hu L, Zhao J, Gruber A, Schwartz B, et al. Quantifying survival in patients with Proteus syndrome. Genetics in medicine. Genet Med. 2017; 19 (12): 1376-79. https:// doi.org/10.1038/gim.2017.65
Rodenbeck D, Greyling L, Anderson J, Davis L. Early Recognition of Proteus Syndrome. Pediatr Dermatol. 2016; 33 (5): e306-10. https://doi.org/10.1111/pde.12900
Lindhurst M, Yourick M, Yu Y, Savage R, et al. Repression of AKT signaling by ARQ 092 in cells and tissues from patients with Proteus syndrome. Sci Rep. 2015; 11 (5): 17162. https://doi.org/10.1038/srep17162