Total intravenous anesthesia in Osteogenesis imperfecta patient: case report

Abstracts

JUSTIFICATIVA E OBJETIVOS: A Osteogênesis Imperfecta é uma doença genética rara do tecido conjuntivo, com prevalência de 1/10000, que primariamente envolve a ossificação endocondral, resultando em ossos frágeis, múltiplas fraturas e deformidades esqueléticas. O objetivo desse artigo foi relatar um caso de paciente portador de Osteogenesis Imperfecta, submetido à anestesia venosa total para tratamento cirúrgico de fratura de fêmur. RELATO DO CASO: Paciente do sexo masculino, 15 anos, 41 kg, 140 cm, com história de Osteogênesis Imperfecta e cardiopatia, programado para tratamento cirúrgico de fratura do fêmur. Na sala de operação foi monitorizado com ECG, FC, PANI e SpO2 e submetido à anestesia geral venosa total com propofol, alfentanil e cisatracúrio. Após IOT, foi acrescentada monitorização da P ET CO2 e da temperatura esofágica. No período intra-operatório e na sala de recuperação pós-anestésica não apresentou complicações. Teve alta hospitalar no 5º dia de pós-operatório. CONCLUSÕES: O presente relato mostrou boa evolução intra e pós-operatória de paciente com Osteogênesis Imperfecta submetido à anestesia geral venosa total. A complexidade da doença mostrou a necessidade de avaliação e monitorização adequada pelo anestesiologista.

ANESTESIA; ANESTESIA; DOENÇAS

CLINICAL REPORT

Total intravenous anesthesia in Osteogenesis imperfecta patient. Case report^*

Anestesia venosa total en paciente portador de Osteogénesis imperfecta. Relato de caso

José Francisco Nunes Pereira das Neves, TSA, M.D.^I; Roberto Silva Sant'Anna, M.D.^II; João Rosa de Almeida, M.D.^II; Rodrigo Machado Saldanha, TSA, M.D.^II; Marcos Gonçalves Magalhães, M.D.^III

^ICo-Responsável pelo CET/SBA da Universidade Federal de Juiz de Fora; Anestesiologista do Hospital Monte Sinai

^IIAnestesiologista do Hospital Monte Sinai

^IIIME₁ do CET/SBA da Universidade Federal de Juiz de Fora

^{Correspondence}

SUMMARY

BACKGROUND AND OBJECTIVES: Osteogenesis Imperfecta is an uncommon genetic connective tissue disease with prevalence of 1/10000, primarily involving endochondral ossification, resulting in brittle bones, multiple fractures and skeletal deformities. This article aimed at reporting a case of Osteogenesis Imperfecta patient submitted to total intravenous anesthesia for fractured femur surgical repair.

CASE REPORT: Male patient, 15 years old, 41 kg, 140 cm, with history of Osteogenesis Imperfecta and cardiopathy, scheduled for fractured femur surgical repair. In the operating room patient was monitored with ECG, HR, NIBP and SpO₂ and was submitted to total intravenous anesthesia with propofol, alfentanil and cisatracurium. Monitoring of P_ETCO₂ and esophageal temperature was introduced after TI. There have been no complications both in the intraoperative period and in the post-anesthetic care unit. Patient was discharged 5 days later.

CONCLUSIONS: This report has shown satisfactory intra and postoperative evolution of Osteogenesis Imperfecta patient submitted to total intravenous general anesthesia. Disease complexity has shown the need for anesthesiologist’s adequate evaluation and monitoring.

Key Words: ANESTHESIA, Intravenous: total; DISEASES: Osteogenesis Imperfecta

RESUMEN

JUSTIFICATIVA Y OBJETIVOS: La Osteogénesis Imperfecta es una rara enfermedad genética del tejido conjuntivo, con prevalencia de 1/10000, que primariamente envuelve la osificación endocondral, resultando en huesos frágiles, múltiplas fracturas e deformidades esqueléticas. El objetivo de ese artículo fue relatar un caso de paciente portador de Osteogénesis Imperfecta, sometido a anestesia venosa total para tratamiento quirúrgico de fractura de fémur.

RELATO DEL CASO: Paciente del sexo masculino, 15 años, 41 kg, 140 cm, con historia de Osteogénesis Imperfecta y cardiopatía, programado para tratamiento quirúrgico de fractura del fémur. En la sala de operación fue monitorizado con ECG, FC, PANI y SpO₂ y sometido a anestesia general venosa total con propofol, alfentanil y cisatracúrio. Después de IOT, fue acrecentada monitorización de la P_ETCO₂ y de la temperatura esofágica. En el período intra-operatorio y en la sala de recuperación pos-anestésica no presentó complicaciones. Tuvo alta hospitalar en el 5º día de post operatorio.

CONCLUSIONES: El presente relato mostró una buena evolución intra y post operatoria de paciente con Osteogenesis Imperfecta sometido a anestesia general venosa total. La complexidad de la enfermedad mostró la necesidad de evaluación y monitorización adecuada por el anestesiologista.

INTRODUCTION

Osteogenesis Imperfecta is an uncommon genetic connective tissue disease with prevalence of 1/10000, primarily involving endochondral ossification, resulting in brittle bones, multiple fractures and skeletal deformities ^1-4. Mutation of one or two type 1 collagen genes, COL1A1 or COL1A2 ¹, leads to abnormal synthesis of type 1 collagen, which is the predominant bone matrix protein, leading to osteopenia ^3-5.

Osteogenesis Imperfecta is classically divided in 4 types, according to clinical severity: I - affects patients without major bone deformities; II - the most severe and patients die in the perinatal period; III - moderately to severely affected patients, being most severe in children; IV - heterogeneous group, with variable severity and clinical characteristics ^2,6,7. DNA analysis techniques, however, have shown in several cases the existence of patients who, although clinically diagnosed as Osteogenesis Imperfecta, have no mutations in genes coding collagen production ⁶. So, there are patients suffering from the so-called Osteogenesis Imperfecta with hypertrophic callus, with pseudoglioma and rhizomelia ⁶.

This article aimed at reporting a case of Osteogenesis Imperfecta patient submitted to total intravenous anesthesia for fractured femur surgical repair.

CASE REPORT

Male patient, 15 years old, 41 kg, 140 cm, scheduled for fractured femur surgical repair. At preanesthetic evaluation patient presented history of Osteogenesis Imperfecta, cardiopathy and use of vitamin D (125 UL/day), calcium (500 mg/day) and sodium alendronate (10 mg/day). Patient was relaxed, eupneic, hydrated, normally colored mucosa, anicteric, blood pressure 110 x 60 mmHg, heart rate = 96 bpm. Subsidiary exams revealed hemoglobin = 14.8 g.dL^-1, hematocrit = 45.3%, leucometry = 16600/mm³, glycemia = 100 mg.dL^-1, platelets = 266000/mm³ and echocardiogram with mild mitral and tricuspid failure, unchanged pericardium and normal left ventricle systolic function.

Monitoring in the operating room consisted of EEG (MC5), heart rate, peripheral oxyhemoglobin saturation (SpO₂) and noninvasive blood pressure (NIBP) measured at 5-minute intervals, with data within normality range. Venous puncture was achieved with 18G catheter, hydration with lactated Ringer’s solution and anesthesia was induced with intravenous propofol (100 mg), alfentanil (2000 µg) and cisatracurium (6 mg). Tracheal intubation was performed with 7.5 mm internal diameter tube.

Then, monitoring was complemented with end tidal CO₂ (P_ETCO₂) and esophageal temperature. Anesthesia was maintained with target-controlled propofol infusion (3.5 µg.mL^-1), with satisfactory intraoperative cardiovascular stability. At surgery completion, patient was medicated with intravenous atropine (500 µg), neostigmine (1 mg), metoclopramide (10 mg) and nalbuphine (10 mg). After extubation patient was referred to the post-anesthetic care unit with maintenance of the same intraoperative monitoring care (ECG, HR, NIBP, SpO₂ and axillary temperature) and nasal oxygen therapy (3 L.min^-1), where he has remained for 120 minutes without intercurrences. Patient was discharged in the 5^th postoperative day.

DISCUSSION

There are three possible areas for orthopedic Osteogenesis Imperfecta therapeutic approach: measures to prevent the number of fractures, treatment of fractures, and surgical repair of deformities ⁶; anesthesia is needed for the two latter.

Anatomic and physiologic abnormalities may determine intraoperative problems ⁵. Although being clinically characterized by a triad made up of brittle bones, bluish sclera and hearing loss or impairment, there are several aggravating possibilities for Osteogenesis Imperfecta, depending on associated diseases. Congenital cardiopathies, valve injuries, renal lithiasis, neurological disorders, metabolic disorders, platelet dysfunction, cor pulmonale, joint, dermatologic and dental diseases may be part of clinical presentation ^1-4. So, preoperative evaluation should be very judicious.

Anesthetic difficulties start from the choice of the technique because Osteogenesis Imperfecta patients have a trend to develop hyperthermia, which may be malignant ^1,5, and anatomic abnormalities may complicate regional anesthetic induction. Vogel et al. ² have reported anesthesia in two severe Osteogenesis Imperfecta pregnant patients, one of them submitted to continuous epidural block with 2% lidocaine, fentanyl (50 µg) and 1:200,000 epinephrine in fractional doses aiming at reaching sensory block in T₄, and cesarean section went on without complications. The presence of deformities, such as kyphoscoliosis, predisposes to a higher chance of accidental dural puncture, low stature makes difficult the estimate of sensory block level, and coagulopathies and platelet changes should be considered for the indication of regional anesthesia ².

In general anesthesia there may be questions about agents and difficult tracheal intubation. In choosing agents, intravenous technique has been proposed because inhalational agents and succinylcholine may trigger malignant hyperthermia ¹.

Typical Osteogenesis Imperfecta facies, with short neck, temporal and occipital projections, and prominent mandible, may lead to difficult tracheal intubation. Fractured cervical spine, mandible and teeth, mucosal contusions and hemorrhages are possible complications after several tracheal intubation attempts ^4,5. Laryngeal mask is a good alternative for this situation because it may be inserted with patient in neutral cervical position, being a useful and easy method for airway maintenance ⁵ because it prevents laryngoscopy sympathico-cardiac stimulation, which may be desirable for patients with associated cardiopathy ⁵.

Care with manipulation and monitoring should also be considered. Patients should be carefully positioned on the operating table with protection of areas subject to compression. Tourniquet or pressure cuffs may lead to vascular and tissue fractures and/or trauma. Arterial catheterization for pressure measurement should be preferred for severe Osteogenesis Imperfecta patients ⁵.

Hemorrhage is an expected Osteogenesis Imperfecta complication ⁴ in 10% to 30% of patients ³, because collagen defect results in brittle tissues, small vessels which do no adequately contract, deficient platelet response and decreased factor VIII ⁴. Predisposition to hemorrhages is multifactorial and there are numerous factors related to disseminated intravascular coagulation ^3,4.

During preoperative evaluation, history of recurrent purpura may be indicative of coagulation disorders. Subsidiary exams, including thromboelastogram, may be useful to predict the possibility of hemorrhages ^3,4.

Desmopressin, antidiuretic hormone synthetic analog, may improve platelet function and decrease blood loss ³. Desmopressin has significant antidiuretic effect mediated by via V2 receptors and minor or none vasoconstrictor effect. Via V2 effect releases von Willebrand factor in endothelial cells, activates tissue plasminogen and factor VIII:C ³. There is hemostatic effect almost immediately after 0.3 µg.kg^-1 intravenous dose, which may be infused in 20 minutes to prevent hypotension. Side effects include: flare, headache, hypotension, tachycardia and possibility of thrombosis ³.

Antidiuretic effect impairs intravascular volume evaluation and there are reports on hyponatremia and seizures, especially in pediatric patients ³.

This report has shown satisfactory intra and postoperative evolution of Osteogenesis Imperfecta patient submitted to total intravenous general anesthesia. Disease complexity has shown the need for anesthesiologist’s adequate evaluation and monitoring.

REFERENCES

Submitted for publication December 1, 2003

Accepted for publication January 8, 2004

• 01. Porsborg P, Astrup G, Bendixen D et al - Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship? Anaesthesia, 1996;51:862-865.
• 02. Vogel T, Ratner EF, Thomas Jr RC et al - Pregnancy complicated by severe osteogenesis imperfecta: a report of two cases. Anesth Analg, 2002;94:1315-1317.
• 03. Keegan MT, Whatcott BD, Harrison BA - Osteogenesis imperfecta, perioperative bleeding, and desmopressin. Anesthesiology, 2002;97:1011-1013.
• 04. Edge G, Okafor B, Fennelly ME et al - An unusual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta. Eur J Anaesthesiol, 1997;14:215-219.
• 05. Karabiyik L, Parpucu M, Kurtipek O - Total intravenous anaesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta. Acta Anaesthesiol Scand, 2002;46:618-619.
• 06. Assis MC, Plotkin H, Glorieux FH et al - "Osteogenesis Imperfecta": novos conceitos. Rev Bras Ortop, 2002;37:323-327.
• 07. Zeitlin L, Rauch F, Plotkin H et al - Height and weight development during four years of therapy with cyclical intravenous pamidronate in children and adolescents with osteogenesis imperfecta types I, III and IV. Pediatrics, 2003;111:1030-1036.

*

Received from Hospital Monte Sinai, hospital agregado ao CET/SBA da Universidade Federal de Juiz de Fora, MG

Publication Dates

• Publication in this collection
  30 Nov 2004
• Date of issue
  Oct 2004

History

• Accepted
  08 Jan 2004
• Received
  01 Dec 2003