Anesthetic management for a pediatric patient of Klippel-Trenaunay syndrome with giant head by hydrocephalus

Hiroshi Hoshijima, Risa Takeuchi, Masanori Tsukamoto, Saori Ogawa, Yoshinori Iwase, Nobuyuki Matsumoto

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


Klippel-Trenaunay syndrome (KTS) is a rare disorder associated with the triad of 1) capillary vascular malformation, 2) varicose veins and/or venous malformation, 3) and soft tissue and/or bony hypertrophy. A six-month old, 6.0-kg-weight male pediatric patient was scheduled for ventriculo-peritoneal shunt operation for hydrocephalus caused by obstructive aqueductus cerebri. At the age of three months, he was diagnosed as KTS by extensive capillary vascular malformation and soft tissue hypertrophy of the right leg. Physical examination showed prominent vascular malformation over his anterior thoracic and abdominal wall, and soft tissue hypertrophy was only on his right leg. Simultaneously, he was complicated with congenital hydrocephalus because of obstructive aqueductus cerebri. His head and skull were enlarged and his head measurement reached 55 cm (chest measurement 32 cm). Anesthetic management of KTS patients should be prepared with blood transfusion against massive hemorrhage and hypovolemic shock. Furthermore, KTS patients should be always considered to have airway difficulty due to the soft tissue hypertrophy, upper and airway hemangiomas. Therefore, we planned safer tracheal intubation following practice guidelines for management of the difficult airway.

Original languageEnglish
Pages (from-to)1356-1358
Number of pages3
JournalJapanese Journal of Anesthesiology
Issue number12
Publication statusPublished - Dec 2012

All Science Journal Classification (ASJC) codes

  • Anesthesiology and Pain Medicine


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