Histiocytosis X is a clinically heterogeneous disorder characterized by reticuloendothelial hyperplasia and granulomatosis, secondary storage of cholesterol in the histiocytes, terminal fibrosis, and scarring. The histiocytic lesions can occur in one or multiple organs. Exophthalmos, diabetes insipidus, pleural, and pulmonary infiltrations with bullous alterations are common complications of the disease. Despite the advances in the treatment of histiocytosis X, many children with histocytosis do not survive to adulthood which explains why histiocytosis X is rarely associated with pregnancy. The literature reveals about eight cases of patients with histiocytosis X who experienced pregnancy. A 19-yr-old primigravida was transferred to our hospital due to respiratory distress at the 27th week of gestation. The chest X-ray demonstrated bilateral pneumothoraces and mediastinal emphysema. Initial arterial blood gases were pH 7.32, PO2 50 mmHg, PCO2 50 mmHg and SaO2 81%. During 1 week of hospitalization, supportive therapy with oxygen and bronchodilators proved to be ineffective as respiratory conditions deteriorated (PCO2 85 mmHg, PO2 45 mmHg). The usual dose of 2×12 mg betamethasone (Celestan®) was administered for prevention of infant respiratory distress syndrome. It was then decided to deliver the child preterm by Caesarean section in order to avoid the risk of fetal hypoxaemia and to improve pulmonary function of the mother.
Eight days after admission, epidural anaesthesia for Caesarean section was performed by inserting a catheter via an 18-G Sprotte needle and administering a total of 150 mg ropivacaine 0.75% into the epidural space. At this stage, the patient's weight was 55 kg and height 164 cm. The catheter was inserted at L3/4 with the woman in the sitting position. The sensory level was simultaneously determined by temperature perception to alcohol and sharp/dull discrimination to pinprick. The level of analgesia reached to T4.
To avoid arterial hypotension, 33 mL kg−1 body weight of crystalloid and 20 mL kg−1 body weight of hydroxyethylstarch (HAES® 7.5%) were given prior to surgery. A vasopressor (Akrinor®) was administered twice to keep the mean arterial pressure at a level above 75% of baseline. The patient's respiration was continuously monitored using an expiratory carbon dioxide monitor and a pulse oximeter. Oxygen 4 L min−1 was given via nasal cannulae. Routine electrocardiogram and non-invasive blood pressure (BP) measurements were performed during Caesarean section. Overall blood loss was estimated with 350 mL and urine production was 300 mL. Forty-eight hours after Caesarean section the catheter was removed.
The fetus was monitored using ultrasound and antepartum fetal heart rate (HR) testing because maternal hypoxia might place the infant at increased risk. A 970 g male infant was delivered who had Apgar scores of 3, 7, and 9 and had to be ventilated with positive airway pressure and 35% oxygen for 8 days on the paediatric intensive care unit (ICU). After Caesarean section the patient was monitored on the ICU. The pulmonary function stabilized and blood gases were PCO2 50 mmHg and PO2 95 mmHg while receiving 4 L min−1 oxygen via nasal cannulae. The postoperative course was complicated by recurrent pulmonary fistulae causing pneumothoraces and needing operative treatment. At admission lung function was impaired with a vital capacity of 1.66 L and forced expiratory volume in 1 s of 1.1 L. Lung transplantation was considered.
The description of histiocytosis X is as diverse as its symptoms and signs. Previously known as Hand-Schüller-Christian disease, Letterer-Siwe disease, eosinophilic granuloma, and more recently as Langerhans cell histiocytosis, this disorder encompasses a wide variety of clinical syndromes with involvement of one or more organ systems. The incidence of dyspnoea without evidence of deterioration in blood gases or pulmonary function tests in pregnant patients with histiocytosis X shows peaks late in pregnancy . The aetiology of this patient's dyspnoea is uncertain. In our case dyspnoea was atypically associated with deteriorating blood gases and this was attributed to the pneumothorax which is a common complication. Symptoms vary greatly according to the size of the pneumothorax and they were aggravated by bullous changes. Physiologic alterations of the respiratory system are an important consequence of pregnancy itself. Increases in tidal volume and minute volume are due to elevated progesterone levels and carbon dioxide production. The decreases in residual volume, expiratory reserve volume, and functional residual capacity are secondary to cephalic displacement of the diaphragm from the enlarging uterus. This might create the potential for regional ventilation-perfusion mismatching and hypoxia. Supine, and even worse, Trendelenburg positioning for Caesarean section may further distort the ventilation-perfusion relationship.
Considering the lung disease, pregnancy, and the positioning during Caesarean section we decided to perform epidural anaesthesia although our standard procedure for elective Caesarean section in otherwise healthy parturients is single shot spinal anaesthesia with bupivacaine and sufentanil. Spinal anaesthesia, however, may cause haemodynamic side-effects including bradycardia and hypotension. The spread of the sensory and motor blockade is difficult to foresee and even careful dosing may result in a more extensive anaesthetic blockade reaching dermatomes beyond T4/5. We assumed that anaesthesia via an epidural catheter should allow us to titrate the anaesthetic generating sufficient analgesia but avoiding a negative impact on haemodynamics and respiratory function. Maintaining haemodynamic stability and avoiding negative impact on oxygen exchange was our major goal for this patient.
Most patients with chronic lung disease tolerate spinal or epidural anaesthesia well with a level below T10 . Caesarean section, however, requires an anaesthesia level of T4/6. The anaesthetic level in our case reached T4 and remained stable during and after the Caesarean section. The sensory level was simultaneously determined by temperature perception to alcohol and sharp/dull discrimination to pinprick. In order to reach this level, but avoiding uncontrolled spread of the blockade as may happen during a single shot spinal, we initially administered 75 mg ropivacaine 7.5% and then stepwise 25 mg increments up to a total dose of 150 mg through the epidural catheter. We waited approximately 5 min between boluses.
Arterial oxygenation is usually well maintained with mechanical ventilation. Nevertheless, ventilation-perfusion mismatching may occur during general anaesthesia owing to decreased functional residual capacity and compression atelectasis. Positive pressure ventilation with elevated airway pressure to counteract atelectasis is associated with elevated risk of pneumothorax in these patients . Considering the lung disease of this pregnant patient we did not expect pre-oxygenation to be very successful as PaO2 values decrease an average 139 ± 13 mmHg as opposed to 58 ± 8 mmHg in non-pregnant patients during 1 min of apnea while intubating . General anaesthesia for Caesarean section itself is related to morbidity and mortality. During induction of general anaesthesia there is a sevenfold higher incidence of difficult intubation in the obstetric population compared with the non-obstetric population , apart from an elevated risk of regurgitation and aspiration .
Ropivacaine is reported to show a greater degree of separation between motor and sensory blockade relative to bupivacaine . Definitive studies demonstrating a clinical difference between the degree of separation of motor and sensory blockade at equianaesthetic concentrations are not yet available.
Restoration of lung function by adequate post-operative pain control, especially by the use of epidural analgesia is limited. Theoretically, epidural analgesia may be more helpful in patients with respiratory disease because it is associated with a lower PaCO2 and fewer episodes of oxygen desaturation than with parenteral opioids. Small improvements in pulmonary function might have been helpful in this case.
The present case demonstrates that in patients suffering from severe pulmonary complications of histiocytosis X catheter epidural anaesthesia represents a feasible and secure technique and provides excellent analgesia for Caesarean section without negative side-effects on pulmonary function.
Department of Anaesthesiology; University of Wuerzburg; Wuerzburg, Germany
Department of Gynaecology and Obstetrics; University of Wuerzburg; Wuerzburg, Germany
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