Proteus Sendromu’nda Anestezi Yönetimi / Anesthetic Management in Proteus Syndrome
Received: 08.01.2015 Accepted: 26.01.2015 Publihed Online: 26.01.2015
Ankara, Turkey. GSM: +905053573180 F.: +90 3123340352 E-Mail: yeliz_kilic3 @hotmail.com
Proteus sendromu vücudun herhangi bir dokusunda asimetrik, orantısız aşırı doku
büyümesi ile birlikte olan son derece nadir bir konjenital hastalıktır. Proteus send-
romu olan hastaların çoğu genellikle aşırı doku büyümeleri ve ilgili deformiteler
için ameliyata gereksinim duyar. Bununla birlikte, kistik akciğer gibi pulmoner bul-
gular ve kifoskolyozu içeren vertebral anormallikler yüksek anestezi riskine yol
açabilir. Proteus sendromu olan hastalar preoperatif ayrıntılı bir şekilde değer-
lendirilmeli, anestezi sırasında ve sonrasında dikkatlice yönetilmelidir. Bugüne ka-
dar Proteus sendromlu 200 olgu bildirilmiş olmasına rağmen, bu hastalarda anes-
tezi yönetimi ile ilgili az sayıda yayın vardır. Bu yazıda, herhangi bir anestezi iliş-
kili komplikasyon olmaksızın, ayak rekonstrüksiyonu için başarılı bir şekilde ameli-
yat edilen Proteus sendromlu 12 yaşındaki bir erkek çocuk olgusu sunulmaktadır.
Havayolu Yönetimi; Anestezi; Proteus Sendromu
Proteus syndrome is an exceptionally rare congenital disorder with asymmetrical
disproportionate overgrowth that includes any tissue of the body. Most of the
patients with Proteus syndrome are generally needed to operate for overgrowths
and related deformities. However, pulmonary manifestations such as cystic lung
and vertebral abnormalities including kyphoscoliosis can lead to high anesthesia
risk. The patients with Proteus syndrome should be evaluated in detail preopera-
tively, and managed carefully during and after anesthesia. Although up to 200
cases with Proteus syndrome have been reported to date, there are few reports
on anesthetic management in such cases. In this paper, we present a Proteus
syndrome in a case of 12-year old boy who was succesfully operated for foot
reconstruction without any anesthesia-related complication.
Airway Management; Anesthesia; Proteus Syndrome
Yeliz Kılıç, Tuğba Aşkın, Suheyla Ünver, Özge Çolakoğlu
Department of Anesthesiology and Reanimation, Dr. Abdurrahman Yurtaslan Oncology Training and Research Hospital, Ankara, Turkey
Proteus syndrome (PS), first described by Cohen , is an ex-
tremely rare hamartomatous disorder with an estimated
prevalence of approximately 1:1.000.000 . It affects more
males than females , and is characterized with overgrowth
of multiple tissues. Clinical features typically start in the first
18 months of life . Its diagnosis is based on clinical features
and radiological findings as per the criteria recommended by
Biesecker . Surgery is usually indicated due to overgrowth
lesions which lead to complications such as organ deformities,
function loss or cosmetic problems. However, anesthetic man-
agement of the patients with PS is very difficult due to the pres-
ence of kyphoscoliosis, otolaryngologic conditions and cystic
lung malformation that are predisposed to severe respiratory
complications during and after anesthesia.
A 12-year-old boy with PS, weighing 40 kg, presented for left
foot reconstruction. On physical examination, there was a dis-
proportionate asymmetric overgrowth and toes macrodactyly
on his left foot (Figure 1). He also had hemihypertrophy through
the left limb. Magnetic resonance imaging (MRI) of the left foot
revealed hyperostosis of the calcaneus, talus, cuneiform bone
and 3.-4. metatarsal bones with subluxation in talotibial joint.
This overgrowth was started at 3 years old, and increased in
time. However, the patient was diagnosed with PS at 9 years
old. There was no abnormality in vertebral MRI, however cal-
varial asymmetry was detected in brain MRI with a normal elec-
troencephalogram. He had café-au-lait spots on various parts
of his body, including neck, arms and chest (Figure 2). Ultraso-
nography showed bilateral grade 2 ureterohydronephrosis and
multipl millimetric renal cysts. Cardiac and pulmoner examina-
tion were not revealed any abnormalities, with normal electro-
cardiography and chest X-ray.
All routine laboratory tests were in normal ranges. His blood
pressure, heart rate, EtCO2, and oxygen saturation were 115/65
mmHg, 105/min, 41 mmHg, and 99% respectively. Airway as-
sessment was consistent with Mallampatti class 3. Informed
consent form was taken from his parents.
Monitoring included electrocardiography, noninvasive blood
pressure and pulse oximetry. Anesthesia was induced by fen-
tanyl (2,5 µg/kg), lidocaine (1 mg/kg) and propofol (3 mg/kg)
with 100% oxygen. After rocuronium (0,8 mg/kg) endotracheal
intubation was performed with using a 6,5 no spiral endotra-
cheal tube uneventfully. Anesthesia was maintained with sevo-
flurane 2% MAC with nitrous oxide (50%) in oxygen (50%) at 3
l/min total gas flow. In addition, rocuronium was used two times
in maintenance of anesthesia. The operation was performed in
right-side position and lasted 3 hours without any complication,
atropine 0,5 mg and neostigmine 1,5 mg was used for recura-
rization, and finally the patient was extubated successfully. The
patient was given tramadol (1 mg/kg) for postoperative pain
control. The postoperative course was also uneventfully, and he
was discharged on tenth day.
PS is a complex congenital hamartomatous disorder with over-
growth of multiple tissues in a mosaic manner. For correct diag-
nosis, the following three criteria are necessary; mosaic distri-
bution, progressive nature, and sporadic occurence . Skeletal
system, connective tissue and fat are the most commonly af-
fected areas . However, this syndrome can involve any part of
the body. To date, many dermatological manifestations such as
cerebriform connective tissue nevi, Café-au-lait spots, cutane-
ous, venous and lymphatic malformations and patchy dermal
hyperplasia were reported . Our patient also had Café-au-lait
spots on the arms, neck and chest. In addition, hemihypertro-
phy was also noted in his left limb which was led to walking
PS is associated with a number of tumors. Ozkınay et al. re-
ported a case of PS with various tumoral lesions including cav-
ernous hemangiomas, pigmented nevi, lipomas and lymphan-
giomas . Of these, lipomas are the most common tumor type
that usually located in the abdominal or chest wall. Our patient
had several subcutaneous fat tissue hipertrophies. Especially
Figure 1. Macrodactyly and asymmetric overgrowth on the left foot
Figure 2. Multiple café-au-lait spots can be seen on the neck.
two types of tumor, monomorphic adenomas of the parotid
agnostic criteria because of their specificity .
Central nervous system manifestations such as brain malforma-
tions, mental retardation and seizures can be presented in up
to 40% of patients . In our case, calvarial asymmetry due to
hiperostosis was detected in brain MR. In addition, he had mild
mental retardation which was led to poor school performance.
The typical facial phenotype including coarse facies, prominent
forehead, widely spaced eyes, minor ptosis, low nasal bridge,
relative lengthening of the face, and persistently open mouth
may be present in some patients with PS. In a case series re-
ported by Angurana , 3 of 6 PS cases had facial dysmorphism.
Our patient had coarse facial lines and open mouth.
Patients with PS may also have overgrowths through the airway,
and this condition can lead to a difficult intubation. Pennant et
al.  reported such a case who has to be performed fiberoptic-
aided intubation under inhalational anesthesia. Airway assess-
ment of our case was consistent with Mallampatti class 3, but
no problem occurred during the intubation. Additionally, various
vertebral deformities such as kyphoscoliosis and dysplastic ver-
tebra also be found in these patients, and may affect the pul-
monary ventilation. In our patient, no vertebral abnormality was
detected radiologically. A portion of patients with PS may have
some pulmonary manifestations that may cause difficulties dur-
ing anesthesia. Of these, cystic changes, emphysema, atelec-
tasis, and fibrosis are the most frequently reported pulmonary
complications. In addition, asymmetric growth in the ribs may
cause restrictive lung disease. In our case, no abnormality was
detected in the lung parenchyma and ribs. Patients with PS are
also at increased risk of pulmonary embolism because of vascu-
lar malformations. Deep venous thrombosis and pulmonary em-
bolism are also one of the most common causes of death, es-
pecially in young children . For this reason, patients who will
be operated for any reason should be undertaken perioperative
anticoagulation prophylaxis . Additionally, spinal anesthesia
can be a reliable choice of anesthesia in suitable cases accord-
ing to its low risk of developing deep vein thrombosis .
Urological abnormalities such as nephromegaly and renal cysts
are found to be at a ratio of 9% . Our patient had also bi-
lateral grade 2 ureterohydronephrosis, but renal function tests
In conclusion, PS is a rare clinical entity which can lead to de-
velop difficulties in anesthetic management due to presence of
overgrowth through the airway, pulmonary manifestations and
vertebral abnormalities. Therefore, detailed preoperative anes-
thetic evaluation should be necessary for these patients.
The authors declare that they have no competing interests.
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