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Revista Brasileira de Anestesiologia

Print version ISSN 0034-7094On-line version ISSN 1806-907X

Rev. Bras. Anestesiol. vol.57 no.6 Campinas Nov./Dec. 2007 



General anesthesia after failed spinal block for emergency surgery in a patient with mucopolysaccharidosis. Case report*


Anestesia general después de la falla de la anestesia raquidea para procedimiento de urgencia en paciente con mucopolisacaridosis. Relato de caso



Fabiano Timbó Barbosa, TSAI; Eduardo Luiz de Araújo BorgesII; Roberta Ribeiro Marques BrandãoII

IAnestesiologista da Unidade de Emergência Armando Lages e do Hospital Escola Doutor José Carneiro; Médico Intensivista da Clínica Santa Juliana; Tutor da Liga de Anestesia da Universidade de Ciências da Saúde do Estado de Alagoas
IIAcadêmico de Medicina da Universidade Federal de Alagoas

Correspondence to




BACKGROUND AND OBJECTIVES: Mucopolysaccharidosis are a group of heterogenous disorders secondary to the deficiency of a lysosomal enzyme, with accumulation of acid mucopolysaccharides in the tissues. It carries a 20% perioperative mortality rate due mainly to difficulties on handling the airways. The objective of this report was to present the anesthetic approach of a patient with mucopolysaccharidosis who presented in the operating room for emergency surgery.
CASE REPORT: A 15 year-old male patient with a diagnosis of mucopolysaccharidosis was admitted to the operating room for treatment of an incarcerated umbilical hernia. After monitoring, a subarachnoid block with hyperbaric bupivacaine was performed with the patient in the sitting position. After identifying failure of the spinal block it was changed to general anesthesia. Anesthetic induction consisted of intravenous clonidine, fentanyl and propofol, followed by endotracheal intubation without intercurrences. After a 45-minute surgery, the patient was extubated and transferred to the postanesthetic care unit where he remained under observation for 12 hours, being discharged to the regular ward without intercurrences.
CONCLUSIONS: In the case presented here we did not have any difficulties handling the airways. However, this is the main problem in this group of patients and it is up to the anesthesiologist to choose the better technique to guarantee adequate handling of the airways.

Key Words: ANESTHESIA, General; COMPLICATIONS: difficult airways; DISEASES: mucopolysaccharidosis.


JUSTIFICATIVA Y OBJETIVOS: Las mucopolisacaridosis son un grupo heterogéneo de enfermedades hereditarias que ocurren debido a la deficiencia enzimática lisosomal con acumulación de muco polisacáridos ácidos en los tejidos. La mortalidad perioperatoria es de un 20% y se debe principalmente a la dificultad en el manejo de las vías aéreas. El objetivo de este relato fue el de presentar la conducta anestésica adoptada con paciente portador de muco polisacaridosis que se presentó en el centro quirúrgico para la realización de intervención quirúrgica de urgencia.
RELATO DEL CASO: Paciente de 15 años, sexo masculino, con diagnóstico de muco polisacaridosis, llegó al centro quirúrgico para tratamiento de hernia umbilical encarcelada. Después de la monitorización, se sometió a la anestesia subaracnoidea con bupivacaina hiperbárica en la posición sentado. Después de identificar la falla en la anestesia raquidea, el paciente fue sometido a la anestesia general. La inducción anestésica fue realizada con clonidina, fentanil y propofol por vía venosa, seguida de intubación sin intercurrencias. Después de 45 minutos de procedimiento quirúrgico, el paciente fue extubado y llevado a recuperación anestésica. Allá estuvo por 12 horas bajo observación, recibiendo alta para enfermería sin haber tenido intercurrencias durante ese período.
CONCLUSIONES: En el caso en cuestión, no hubo dificultad en el manejo de las vías aéreas. Sin embargo, ese es el principal problema encontrado en ese grupo de pacientes, cabiéndole al anestesiólogo escoger la mejor técnica para garantizar un adecuado manoseo de las vías aéreas.




Mucopolysaccharidosis (MPS) are a heterogenous group of inherited disorders caused by the accumulation of mucopolysaccharides, currently known as glycosaminoglycans 1-3, in the tissues. They are autosomal recessive disorders, with the exception of mucopolysaccharidosis type II, Hunter syndrome, an X-linked condition 1,2,4.

It has an incidence of approximately 1:30,000 live births 2,4,5, a prevalence of 4:100,000 people, and it caries a perioperative mortality rate of 20% 2,3,5.

Patients with mucopolysaccharidosis have a higher admission rate to hospitals for surgeries than the general population. And for this reason, although it is a rare syndrome, it is important to have a basic knowledge of this disorder 6.

The objective of this report was to present the anesthetic approach of a patient with mucopolysaccharidosis who was admitted to the operating room for emergency surgery.



A 15 years old male patient, weight 60 kg, with a diagnosis of mucopolysaccharidosis, was admitted to the operating room for the treatment of an incarcerated umbilical hernia. On physical exam, the patient had a short neck, macroglossia, mental retardation, liver edge more than 10 cm from the border of the ribcage, and edge of the spleen 20 cm from the margin of the ribcage; the remaining of the physical exam was normal.

Preoperative tests demonstrated discrete leukocytosis without a left shift, coagulation profile and platelet count without abnormalities and absence of radiographic evidence of cardiac involvement or pulmonary disorders.

The patient was monitored with pulse oximetry, cardioscope and non-invasive automatic sphygmomanometer. A venipuncture with an 18G catheter was done for infusion of Ringer's lactate at 10

With the patient on a sitting position and after preparing the thoracolumbar area with an iodine solution, a lumbar puncture was performed with a 25G Quincke needle between the third and fourth lumbar vertebrae using the median approach. After return of cerebrospinal fluid, 15 mg of 0.5% hyperbaric bupivacaine were administered. The level of the sensitive blockade was tested with a needle. After waiting for 30 minutes, failure of the spinal block was noticed. It was, then, decided to submit the patient to general anesthesia. Anesthetic induction was accomplished with the intravenous administration of 0.075 mg of clonidine, 0.1 mg of fentanyl, and 150 mg of propofol. Laryngoscopy with manual fixation of the head was performed, and it was verified that the patient satisfied the criteria for class 1 of Cormack and Lehane. Orotracheal intubation was done without intercurrences. Anesthesia was maintained with intravenous atracurium (30 mg) and fentanyl (0.1 mg) and sevoflurane with 100% oxygen.

The surgery lasted 45 minutes. Before the end of the procedure, intravenous dypirone (1 g), tramadol (50 mg), dexamethasone (10 mg), atropine (0.5 mg), and neostigmine (1.0 mg) were administered.

The patient was extubated and transferred to the postanesthetic care unit, where he remained under observation for 12 hours without intercurrences and then transferred to the regular ward.



Lysosomes are subcellular organelles that contain specific hydrolases that degrade proteins, nucleic acids, carbohydrates and lipids 7. There are more than thirty lysosomal deposit disorders that should be considered in the differential diagnosis of patients with neurologic or muscular degeneration, unexplained hepatomegaly or splenomegaly, or skeletal dysplasia and deformities 7.

Mucopolysaccharidosis (MPSs) are genetic disorders secondary to a deficiency or absence of lysosomal enzymes, with accumulation and abnormal secretion of acid mucopolysaccharides or glycosaminoglycans (GAGs) 8,9 in the tissues. The areas affected most often include bones, brain, liver, cartilages, cardiac valves, cornea and airways 10.

Glycosaminoglycans are long-chain complex carbohydrates associated with the proteins of the connective tissue 2,7. Its catabolism occurs inside the lysosomes and specific enzymes are necessary for each stage of the process 2. When the activity of the enzymes is decreased or absent, GAGs accumulate in tissues and its excess is eliminated in the urine 1,210-12. Progressive accumulation in tissues leads to functional and structural dysfunction 1,2. Patients with more severe disease have a mutation that causes complete absence of the enzymes while milder forms have detectable enzymes, but with an accentuated reduction in activity 1.

Most patients look normal when they are born, but by the age of two they have developed characteristic histopathologic changes 1. The clinical characteristics that help characterize patients with MPSs include: mental retardation 9,10, hepatosplenomegaly 3,9,10, cardiopathy due to valvar and coronary artery disease 4,9, bone changes 2,7,9,10 and changes in the airways 2,3,9,10. Mental retardation is associated with the most severe forms of the disease 10.

Bone changes characteristics of the disease include increased bone thickness of the skull 1, J-shaped sella 1, kyphoscoliosis 1,2, oar-shaped ribs with short stature 1, pectus excavatum 2, pectus carinatum 2, reduction of the intercostal spaces 2, odontoid hipoplasia 10 and atlanto-occipital subluxation 10. The thoracic anatomic changes mentioned, as well as the compromise of laryngeal and tracheal cartilages and the presence of abundant secretions in the airways, might lead to the development of chronic pulmonary disease 13.

The patient presented here did not have thoracic changes on physical exam and the chest X-ray did not demonstrate pulmonary changes or an increase in the size of the heart. This led to the supposition that the increased size of liver and spleen was secondary to the infiltration of mucopolysaccharides and not to congestive heart failure. Usually, MPSs do not cause liver failure 3,4 or changes in coagulation factors 3.


Table I


In 1972, McKusick created a classification that helps understand the types of MPSs and their clinical characteristics 2.10,14.

The diagnosis of MPS is clinical and it is confirmed by complementary exams 1,7,10, such as checking the urine for the presence of GAGs 7,10, identification of GAGs in the urine by electrophoresis 1 and determination of enzymatic deficiency in leukocytes or culture of fibroblasts 1,10.

Treatment is symptomatic 1. Enzymatic replacement is not feasible and transfusion of plasma did not show significant phenotypic improvement 1. Bone marrow transplantation offers some hope for cure 15.

Blockade of the neuro axis is advocated by some authors as an effective anesthetic technique for patients with MPSs 24. However, this did not happen in the case of this patient. Vas and Naregal 15 reported a case of failure of epidural block attributed to the deposit of GAG in the epidural space or in the sheath of the nerve fiber, preventing direct access of the local anesthetic to the nerves. A search in the Medline data base for the period from 1987 to 2005 did not reveal any reports on failure of spinal block. It is possible that the failure in this patient was secondary to the deposit of GAG in the nerve tissue, blocking the action of the local anesthetic.

The greatest difficulty of general anesthesia in those patients is airways's management 2-4,6,10-12,16, since 26% of those patients have problems in this area 3,6. Perioperative mortality is around 20% and it is attributed to respiratory failure 2,3,5.

The difficulty in handling the airways in those patients are due to a short neck 4,13,18, rigidity of the temporomandibular joint 2,4,8,10,13, macroglossia 2,4,8,13,18, anteriorization of the larynx 10,13,18, abundant and thick secretions in the airways 2,8,17, cervical instability 8,16,18, and long and relatively immobile epiglottis 18. Hyperextension of the neck can cause complete transection of the spinal cord 4,5.

When facing difficulties on tracheal intubation, one can consider other alternatives, such as tracheostomy 3,13, intubation with fibroscopy 4,12,19, or laryngeal mask (LM) 4,12,19. Laryngeal mask can be routinely used in short procedures in patients with MPS 19 and it also allows the use of the fibroscope 19,20.

Tracheostomy should be considered with caution 13 because, despite being technically more difficult 3,13 usually there is deposit of mucopolysaccharides around the cannula, leading more easily to tracheal stenosis 13.

After direct laryngoscopy, one can use the classification of Cormack and Lehane to predict the degree of difficulty in the intubation 20. In class I, the epiglottis and the vocal cords can be visualized; in class II, the epiglottis and the posterior commissure are seen; in class III, only the epiglottis is seen; and in class IV, the epiglottis cannot be visualized 20. The patient presented here was deemed class I and was intubated without the administration of neuromuscular blockers.

Mucopolysaccharidosis are not myopathies and, therefore, do not predispose to malignant hyperthermia 3. Succinylcholine is not counter-indicated in MPS 2, and the use of neuromuscular blockers after intubation is recommended 2,4,8.

The main cause of mortality is acute respiratory failure, followed by cardiovascular complications 2. These complications are consequence of valvar regurgitation, myocardiopathy and stenosis of the coronary artery. 21 Involvement of the coronary artery can be extensive; however, coronary thrombosis and myocardial infarction are extremely rare 3,4.

Some steps must be taken before elective surgeries, such as:

  1. Prophylactic antibiotics and respiratory therapy to improve pulmonary function 2,3;
  2. Investigate pulmonary function with chest X-rays and arterial blood gases 2;
  3. X-Rays of the cervical spine to evaluate cervical instability 2;
  4. Evaluate cardiac function with electrocardiogram, echocardiogram and coronary angiography 2;
  5. Use of an anti-cholinergic agent as preoperative medication to decrease secretions 2,6,9. Scopolamine and glycopyrrolate are less inotropic positive being the drugs of choice 2,3,6;
  6. Restrict the use of benzodiazepines and opioids as pre-anesthetic medication 16, since there are reports of death after preoperative sedation 9.

Recovery after anesthesia is frequently slow and with periods of apnea, bronchospasm, and cyanosis 12,6, and cases of postoperative respiratory arrest have been reported 2. Closer postoperative observation is extremely important, and patients undergoing major surgeries should be transferred to the intensive care unit for closer follow-up 6.

In the case presented here, we had no difficulties handling the airways of the patient. However, this is the main problem in this group of patients, and it is up to the anesthesiologist to choose the best technique to maintain the airways opened.



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Correspondence to:
Dr. Fabiano Timbó Barbosa
Rua Comendador Palmeira, 113/202
Edifício Erich Fromm –
57051-150 Maceió, AL

Submitted em 06 de julho de 2006
Accepted para publicação em 08 de agosto de 2007



* Received from Unidade de Emergência Armando Lages

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