Management of a parturient with an acute exacerbation of idiopathic pulmonary haemosiderosis and posterior spinal instrumentation
Division of Women’s Anaesthesia, Department of Anaesthesiology, Duke University Medical Centre, Box 3094, Durham, NC 27710, USA
* Corresponding author. E-mail: ticka98{at}btinternet.com
Accepted for publication November 22, 2007.
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Abstract |
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Idiopathic pulmonary haemosiderosis (IPH) is a rare condition associated with diffuse alveolar haemorrhage and pulmonary fibrosis. We describe the anaesthetic management of a parturient with a history of posterior spinal fusion presenting with an acute exacerbation of IPH necessitating vaginal delivery at 34 weeks gestation. We used a spinal catheter for labour analgesia and bilevel positive airway pressure (BIPAP) ventilation to improve oxygenation during labour. An arterial line sited to allow frequent arterial blood gas sampling also facilitated continuous cardiac output monitoring. The use of a carefully titrated neuraxial block for analgesia, in conjunction with BIPAP, was associated with minimal haemodynamic and respiratory compromise during labour in this patient.
Keywords: anaesthesia, obstetric; analgesic techniques, subarachnoid; equipment, catheters subarachnoid; idiopathic pulmonary haemosiderosis; ventilation, bilevel positive airway pressure
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Introduction |
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Idiopathic pulmonary haemosiderosis (IPH) is a rare disorder characterized by remitting and relapsing episodes of intra-alveolar bleeding, which over time leads to progressive secondary pulmonary fibrosis, pulmonary hypertension, and cor pulmonale.1 Females with IPH rarely survive to childbearing age or are unable to become pregnant. Pregnancy may also exacerbate the symptoms of IPH necessitating early delivery.2 A previous case report described the anaesthetic management of a Caesarean delivery in a pregnant woman with IPH.3 We report the successful management of a parturient with IPH with continuous spinal analgesia and bilevel positive airway pressure (BIPAP) for vaginal delivery.
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Case report |
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A 19-yr-old primigravida at 33 weeks and 6 days gestation was admitted with a history of a sudden onset of chest pain and dyspnoea. She had a past history of IPH diagnosed on open lung biopsy at the age of 11. At the time of her initial diagnosis, pulmonary function tests (PFTs) revealed severe restrictive lung disease: forced vital capacity (FVC) 0.78 litre, forced expiratory volume at 1 s (FEV1) 0.77 litre, and FEV1/FVC 99%. She had been on long-term prednisolone, azathioprine, and oxygen 1.5 litre min–1 at night. However, she became non-compliant with her drug therapy once she became pregnant. She also had a history of idiopathic scoliosis, with a dextroscoliosis from T7 to T12 of 35° and a laevoscoliosis from L1 to L4 of 32°. This was surgically corrected 2 yr earlier with posterior spinal fusion and instrumentation from T5 to L2 with an Isola hook and screw rod system. On physical examination, she had a ventilatory frequency of 30 bpm with decreased breaths sounds bilaterally in the upper lobes. She weighed 56 kg, was 160 cm tall, and had a Mallampati class I airway with a thyromental distance of 6 cm. Oxygen saturation (SpO2) on room air was 90%. Arterial blood gases (ABG) on admission on room air were as follows: pH 7.46, PO2 8.7 kPa, PCO2 4 kPa, and HCO3– 21 mmol litre–1. Her haemoglobin was 12.6 g dl–1. An ECG showed sinus tachycardia. Chest X-ray revealed bilateral interstitial heterogeneous opacities consistent with ongoing subclinical pulmonary haemorrhage and longstanding pulmonary fibrosis (Fig. 1). She was immediately started on oxygen 10 litre min–1 via facemask, prednisolone 50 mg orally daily for 5 days, and she received repeated nebulized salbutamol 2.5 mg. Her respiratory symptoms improved and by day 4 she had been weaned to 4 litre min–1 via nasal cannula. At that time, her ABGs were: pH 7.44, PO2 10.4 kPa, PCO2 4.5 kPa, HCO3– 22 mmol litre–1, and repeat PFTs revealed severe limitation of lung function: FVC 1.25 litre (47% predicted) and FEV1 0.78 litre (31% predicted). An echocardiogram revealed mild tricuspid and pulmonary regurgitation with an estimated peak pulmonary artery systolic pressure of 27 mm Hg. A fetal ultrasound revealed a severely growth-restricted fetus with fetal growth parameters in the 3rd percentile for gestational age.
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After consultation with the paediatric respiratory physicians, and because of concerns about fetal well-being and worsening maternal pulmonary function, induction of labour was planned at 34 weeks and 4 days to expedite delivery. Pelvic examination confirmed that the cervix was already 3 cm dilated. Before augmentation of labour with oxytocin, a 20 G right radial arterial catheter was inserted to facilitate frequent ABG sampling and haemodynamic monitoring. An initial ABG done on 5 litre min–1 of oxygen via a simple facemask revealed a PO2 of 9.3 kPa and PCO2 of 4.4 kPa. Her SpO2 at that time was 93%. ABGs were repeated at 2–3 h intervals to guide oxygen therapy with the aim of maintaining a PO2
10 kPa. Because of the severity of her intrinsic lung disease, her acute presentation and uncertainty about how she would cope with the increased physiological demands of labour and delivery, we continuously monitored her arterial pressure using an arterial line and cardiac output using the lithium indicator dilution system and pulse contour analysis (LIDCO® plus cardiac sensor system, LIDCO Ltd, Cambridge, UK) (Fig. 2).
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A spinal catheter was placed using 2D ultrasonography (Sonosite Titan®, Sonosite Inc., WA, USA) to locate the midline. An 18 G Tuohy needle was used to intentionally puncture the dura in the sitting position at the L4–5 level. Five centimetres of a 20 G catheter was easily threaded into the subarachnoid space. Labour analgesia was initiated with a 2 ml bolus of a bupivacaine 0.1% and fentanyl 2 µg ml–1 solution. Ten minutes later, this bolus achieved a sensory block to cold up to and including T10 bilaterally and a Bromage score of 1 with no associated hypotension. Augmentation of labour with an oxytocin infusion was then initiated. Patient-controlled spinal analgesia with a 1 ml bolus and a 45 min lockout utilizing the same solution was subsequently used throughout labour.
In an attempt to improve her oxygenation during labour, we instituted BIPAP non-invasive ventilation (NIV) using the BIPAP® Vision® ventilatory system (Respironics, Murraysville, PA, USA). Initially, we administered an inspiratory positive airway pressure (IPAP) of 10 cm H2O and an expiratory positive airway pressure (EPAP) of 5 cm H2O via the facemask, with an immediate improvement in her SpO2 from 92% to 98% on 40% oxygen. Thirty minutes after initiation of BIPAP, an ABG revealed an improvement in PO2 to 13.3 kPa, which persisted after the discontinuation of BIPAP, at the patient’s request 1 h later (Table 1). However, the BIPAP was readily available in case there was any deterioration in oxygenation. Labour proceeded uneventfully and approximately 8 h later, the cervix became fully dilated. She had a spontaneous vaginal delivery and delivered a live male infant weighing 2075 g. The baby’s APGARs were 9 and 9 at 1 and 5 min, respectively. She received in total bupivacaine 8 mg and fentanyl 16 µg through the spinal catheter during labour. She developed no signs of cardio-respiratory decompensation during labour and delivery. After delivery, she was admitted to the surgical intensive care unit and discharged to the postnatal ward 12 h later. Her oxygen requirements were weaned to 1–3 litre min–1 via nasal cannula overnight to maintain SpO2 at 88–92%. She was restarted on oral azathioprine, montelukast, and inhaled fluticasone and salmeterol. We left the spinal catheter in situ for 24 h after delivery, and immediately before removal injected 10 ml of sterile normal saline. On the fourth postpartum day, she was discharged home on oral immunosuppressants and 1.5 litre min–1 of oxygen overnight.
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On postpartum day 8, the patient developed symptoms of a postdural puncture headache (PDPH) but declined an epidural blood patch. Her symptoms improved a day later with the use of regular non-steroidal anti-inflammatory agents, acetaminophen, and oxycodone.
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Discussion |
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IPH represents a rare cause of diffuse alveolar haemorrhage characterized by haemoptysis, dyspnoea, alveolar infiltrates, and varying degrees of anaemia.4 The intra-alveolar haemorrhage results in abnormal accumulation of haemosiderin-laden macrophages, alveolar basement membrane thickening, and ultimately interstitial fibrosis.4 5 Overall, the incidence of IPH is unknown; however, studies from Scandinavia and Asia suggest that it lies between 0.24 and 1.23 cases per million live births per year.6 7 IPH is predominantly a childhood disease, but 20% of cases are of adult onset.4 A possible genetic or environmental link has been suggested by reports highlighting familial clustering.4 PFTs generally reveal a pattern of restrictive lung disease; however, a mixed restrictive and obstructive lung disease pattern has also been described.5 Right ventricular hypertrophy in IPH may be associated with pulmonary hypertension or may be an independent finding. Cardiac dysfunction may also occur as a result of myocarditis and myocardial fibrosis resulting from chronic infiltration of the myocardium and endocardium with polymorphonuclear leucocytes, lymphocytes, and macrophages. Conduction abnormalities including left and right bundle branch blocks, intraventricular conduction delay, and complete heart block have also been described.8 Immunosuppression forms the mainstay of treatment, with glucocorticoids being particularly useful in the acute phase of the disease.4
Pregnancy appears to exacerbate IPH with the symptoms typically worsening in the third trimester. A review of seven cases found that six experienced worsening of their respiratory symptoms during the third trimester with one reported maternal death.1 3 This may reflect the continued increase in cardiac output and pulmonary blood flow observed in the third trimester unmasking subclinical pulmonary haemorrhage.1 These changes are poorly tolerated by patients with secondary pulmonary hypertension and can lead to the development of acute right heart failure and rapid cardiovascular decompensation.9 Poor compliance with immunosuppressants, a reduction in drug bioavailability caused by an increase in the volume of distribution or altered drug metabolism, and changes in the immune system associated with pregnancy may also explain this observation.1 The 25% reduction in functional residual capacity (FRC) in pregnancy, the decrease in chest wall compliance, and the increase in closing volume can exacerbate the hypoxia already associated with pulmonary fibrosis secondary to IPH.9 This reduction in maternal pulmonary function, along with an increase in maternal systemic vascular resistance and a reduction in umbilical artery blood flow, predisposes to foetal intrauterine growth retardation.10
The management of this parturient with severe chronic respiratory disease and extensive spinal instrumentation presented several challenges. First, we needed to maintain and optimize maternal oxygenation in the face of an acute exacerbation of her intrinsic lung disease during labour and delivery while trying to avoid the need for endotracheal intubation. In addition, providing safe and reliable labour analgesia in a patient with abnormal vertebral anatomy while attempting to maintain cardiorespiratory homeostasis also became a priority. We used neuraxial analgesia to provide adequate pain relief, reduce the maternal stress response to labour, and optimize oxygenation. Although i.v. opioids may be used to provide labour analgesia, they provide less effective analgesia during the later stages of labour, and are associated with maternal respiratory depression and cough suppression.11 In addition to attenuating the maternal stress response, neuraxial analgesia in labour may cause a small but significant increase in maternal lung function as evidenced by an improvement in vital capacity, FEV1, FVC, and peak expiratory flow rate (PEFR) when compared with antenatal values.12 The subtle reduction in abdominal wall muscle tension caused by neuraxial analgesia may also result in a decrease in diaphragmatic strain and ease the work of breathing during labour.12 However, neuraxial anaesthesia should be used with caution in parturients with respiratory disease as significant cephalad spread can cause a reduction in inspiratory capacity and expiratory reserve volume and may unmask or worsen ventilatory function.13
In addition to supplemental oxygen, BIPAP was instituted during labour to optimize oxygenation. Despite being used for only 1 h, we observed an improvement in oxygenation and a reduction in subsequent oxygen requirements. The non-invasive application of pressure support on inspiration (IPAP) overcomes airway resistance whereas positive pressure on expiration (EPAP) allows recruitment of alveoli and an improvement in FRC and lung compliance which translates into an improvement in oxygenation and ventilation.14
Labour analgesia in parturients with scoliosis who have had extensive posterior spinal instrumentation poses several challenges to the obstetric anaesthetist. The abnormal vertebral anatomy may result in technical difficulties during placement of central neuraxial blocks. Reviewing radiological films antenatally, and using ultrasonography may be useful aids in guiding difficult placement of central neuraxial blocks in these patients. In addition, there is an increased risk of failed or inadequate blocks, subdural and intravascular catheter placement, and dural punctures with lumbar epidural anaesthesia, resulting from adhesions of the ligamentum flavum obliterating the epidural space.15 Even if lumbar epidural provides adequate analgesia in labour, it may not be adequately extended for an operative delivery and performing spinal anaesthesia subsequently could result in a high block with respiratory compromise. Women with surgically corrected scoliosis are at two- to three-fold greater risk of a Caesarean delivery when compared with normal parturients.16 As a result, we elected to insert a spinal catheter for labour analgesia which could also be slowly and carefully extended for Caesarean delivery, and therefore avoiding the need for general anaesthesia. In a recent case report, general anaesthesia was used for an emergency Caesarean delivery in a parturient with IPH.3 The authors highlighted the use of positive end expiratory pressure (PEEP) to successfully treat hypoxia after intubation, but mechanical ventilation was needed for 6 h after operation. To reduce the risk of PDPH, we left the spinal catheter in situ for 24 h, and administered a saline bolus intrathecally before its removal.17 18
In conclusion, the management of pregnant patients with IPH should be multidisciplinary, with appropriate antenatal investigations to determine the severity of their respiratory disease and any other co-morbidities, and the formulation of an anaesthetic and obstetric plan. BIPAP may be useful for improving oxygenation. The use of a carefully titrated neuraxial block for analgesia is advocated, as it is associated with minimal haemodynamic compromise and may actually be beneficial to maternal pulmonary function during labour. We think that continuous spinal analgesia is a better choice, since it can provide adequate pain relief in labour and could be more reliably extended for an operative delivery.
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Funding |
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Departmental funds.
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Footnotes |
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This case report was presented in part at the 39th annual meeting of the Society of Obstetric Anesthesia and Perinatology in Banff, Alberta, May 16–19, 2007. 
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