Principles for the surgical management of patients with proteus syndrome and patients with overgrowth not meeting proteus criteria

Matthew Lublin, Douglas J. Schwartzentruber, Jeff Lukish, Caroline Chester, Leslie G. Biesecker, Kurt D. Newman

Research output: Contribution to journalArticle

Abstract

Background: Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Patients with Proteus syndrome present with diverse and variable phenotypes because of the syndrome's mosaic pattern of distribution. Methods: Eighty patients with Proteus syndrome, satisfying published diagnostic criteria, and 51 patients with overgrowth not meeting Proteus criteria were identified from the literature. Three additional patients, one patient with Proteus syndrome and 2 patients with overgrowth, were treated at the author's institutions and are discussed in detail. All nonorthopedic and noncutaneous surgical interventions were reviewed. Results: Fourteen genitourinary, 9 gastrointestinal, and 5 otolaryngologic operations were performed on patients with Proteus syndrome. Six genitourinary, 5 gastrointestinal, and 2 otolaryngologic operations were performed on patients with overgrowth not meeting Proteus criteria. Eight patients with Proteus syndrome and 4 patients with overgrowth experienced thoracic manifestations, generally diffuse cystic pulmonary lesions, but only 1 of 12 underwent surgical treatment. Conclusions: Patients with visceral manifestations of either Proteus syndrome or overgrowth not meeting Proteus criteria should be treated in a similar manner. Lesions involving the ovaries and testes, because of the high incidence of neoplasm, should be managed aggressively. Gastrointestinal and renal lesions may be managed conservatively with frequent follow-up to minimize abdominal explorations. All patients undergoing surgery should have a thorough preoperative assessment of their airway and pulmonary reserve because of the relatively high frequency of tonsillar hypertrophy and pulmonary cystic involvement.

Original languageEnglish (US)
Pages (from-to)1013-1020
Number of pages8
JournalJournal of Pediatric Surgery
Volume37
Issue number7
DOIs
StatePublished - 2002
Externally publishedYes

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Proteus Syndrome
Proteus
Lung
Vascular Malformations

Keywords

  • Mosaicism
  • Overgrowth
  • Proteus

ASJC Scopus subject areas

  • Surgery

Cite this

Principles for the surgical management of patients with proteus syndrome and patients with overgrowth not meeting proteus criteria. / Lublin, Matthew; Schwartzentruber, Douglas J.; Lukish, Jeff; Chester, Caroline; Biesecker, Leslie G.; Newman, Kurt D.

In: Journal of Pediatric Surgery, Vol. 37, No. 7, 2002, p. 1013-1020.

Research output: Contribution to journalArticle

Lublin, Matthew ; Schwartzentruber, Douglas J. ; Lukish, Jeff ; Chester, Caroline ; Biesecker, Leslie G. ; Newman, Kurt D. / Principles for the surgical management of patients with proteus syndrome and patients with overgrowth not meeting proteus criteria. In: Journal of Pediatric Surgery. 2002 ; Vol. 37, No. 7. pp. 1013-1020.
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