Anesth Analg 2003;96:1805-1808
© 2003 International Anesthesia Research Society
OBSTETRIC ANESTHESIA
Epidural Labor Analgesia for a Patient with Disseminated Lymphangiomatosis
Isuta Nishio, MD PhD*,
Gordon L. Mandell, MD
,
Sivam Ramanathan, MD, and
Jules H. Sumkin, DO
*Department of Anesthesiology, University of Pittsburgh, UPMC St. Margaret Pain Medicine Center, Pittsburgh, Pennsylvania; and Departments of
Anesthesiology and
Radiology, University of Pittsburgh, Magee-Womens Hospital, Pittsburgh, Pennsylvania
Address correspondence and reprint requests to Isuta Nishio, MD, UPMC St. Margaret Pain Medicine Center, 200 Delafield Ave., Ste. 2070, Pittsburgh, PA 15215. Address e-mail to nishioi{at}anes.upmc.edu
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Abstract
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IMPLICATIONS: We describe a case of a parturient with disseminated lymphangiomatosis involving the thorax, retroperitoneum, and lumbar vertebrae who received epidural labor analgesia. Clinical presentations vary depending on the organ systems involved, the extent of the disease, and the stage of pregnancy. Anesthetic implications are discussed.
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Introduction
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Lymphangiomatosis is a rare malformation of the lymphatic system. The presentation and severity of the disease vary depending on its anatomical location(s). We report the first case of a laboring parturient with disseminated lymphangiomatosis (DLAM) involving the lumbar vertebrae and thorax. The anesthetic management of patients with DLAM is discussed.
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Case Report
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The patient, a 25-yr-old nulliparous parturient with a history of DLAM, presented in active labor at 39 gestational weeks, and an anesthetic consultation was requested. At her birth, lymphangiomas were found on her neck, various bones, chest, and retroperitoneum. Despite multiple rib and clavicular fractures during childhood, her general health had been excellent. Magnetic resonance imaging (MRI) at 15 wk of gestation showed abnormal changes in the lumbar supine, ilium, and sacrum; a large right pleural effusion; and extensive lymphangiomas in the thorax and retroperitoneum (Fig. 1). The lumbar epidural space and spinal canal appeared to be unaffected. Transthoracic echocardiography showed normal ventricular size and function. The patient remained asymptomatic until 25 wk of gestation, when she presented with shortness of breath, which was managed with the drainage of chylous fluid by thoracentesis. There were otherwise no pregnancy-related complications. Her physical examination was unremarkable except for diminished breath sounds at the lung bases. Neither scoliosis nor tenderness was found in the lumbar vertebral area. No neurologic signs or symptoms, such as lower back pain or radiculopathy, were noted. Unfortunately, imaging studies were not repeated after 15 wk of gestation. At 4 cm of cervical dilation and with the consent of the patient, epidural analgesia was induced. With use of a loss of resistance to air technique, an epidural catheter was placed at the L3-4 interspace without difficulty. After the administration of an epidural test dose, analgesia was established with a bolus of 8 mL of 0.125% bupivacaine plus 100 µg of fentanyl, followed by continuous infusion of 0.125% bupivacaine with 0.0002% fentanyl at a rate of 10 mL/h. The patient had complete pain relief with a T10 sensory level and minimal motor block throughout labor. She delivered a healthy baby approximately 5 h after the induction of epidural analgesia without complications. Apgar scores were 9 at 1 and 5 min.
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Discussion
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Lymphangiomas, or cystic hygromas, are localized malformations that are thought to arise from sequestered lymphatics that fail to communicate normally with the lymphatic system. They can occur in any tissue in which lymphatics are normally found. It is one of the most common tumors in the pediatric age group but can occur in adults as well. Although this is still controversial, lymphangiomas are generally not considered neoplasms and grow very slowly. Lymphangiomatosis is characterized by the presence of multiple lymphangiomas, whereas DLAM (systemic or generalized lymphangiomatosis) is the term used for diffuse involvement of soft tissue, viscera (especially lungs and spleen), or bone by a lymphangiomatous proliferation. There appears to be a predilection for neck, thoracic, and bony involvement (1). No characteristic symptoms, signs, laboratory abnormalities, or tumor markers have been defined. Presenting symptoms depend on the organ systems involved and the extent of the disease (Table 1). To date, there is no report of DLAM in the anesthesiology literature. However, like other systemic diseases, DLAM has important anesthetic implications. Bony involvement may predispose the patient to fractures and vertebral instability if care is not taken during positioning. Cardiopulmonary function can be affected in these patients secondary to pleural and pericardial chylous effusions, and the patient may require preoperative thoracentesis or pericardiocentesis before the induction of anesthesia. Splenic involvement can cause thrombocytopenia, which affects the choice of anesthesia.
The femur, pelvis, ribs, and vertebrae are often affected (2–5). Precautions must be taken to protect bony lesions from fractures during positioning. The central nervous system is usually spared because of its lack of a lymphatic system (4), although paraplegia due to spinal instability secondary to osteolysis has been reported (5). Lymphangiomas have been found in the lumbar epidural space (6). If present, the epidural venous engorgement that normally occurs during pregnancy may be more pronounced, increasing the risk of intravascular catheterization during epidural catheter insertion. If possible, it is important to evaluate neurological function and vertebral/epidural involvement before attempted epidural analgesia. In our patient, an MRI of the spine at 15 weeks demonstrated a lymphangioma at the lateral aspect of the L3 vertebral body but no epidural/neural involvement or osteolytic lesions. In the absence of lumbar tenderness, fractures, scoliosis, and neurological signs/symptoms and because of previously negative epidural involvement according to MRI, we concluded that epidural analgesia was not contraindicated.
MRI is the study of choice for this disease, especially during pregnancy, because of a lack of radiation and better evaluation of soft tissue involvement. No additional MRI was performed before the epidural analgesia, because lymphangiomatosis is considered to progress very slowly. It may have been more prudent to exclude any possible changes or progression of the disease by obtaining a second MRI in the third trimester, because this might have helped guide our decision to perform neuraxial blockade. There is, however, no evidence to suggest that pregnancy exacerbates the progression of the disease. The only possible exception is renal lymphangiomatosis, which is reportedly exacerbated by pregnancy, probably because of marked increases in glomerular filtration rate and reabsorption of water and solutes during pregnancy that may not be adequately handled by an abnormal lymphatic system (16).
The thorax is frequently affected by this disease. Pleural and pericardial chylous effusions are common. Presenting symptoms may result from compression of vital structures by lymphangiomas, accumulation of fluid, or both. Many patients experience wheezing, which may be misdiagnosed as asthma (1). Surgical resection may be technically difficult and associated with a frequent rate of recurrence (7,23); thoracenteses (8), pericardiocenteses (9), partial pleurectomy (8), and thoracic duct ligation (8,9) have been used with varying degrees of success.
Despite a large pleural effusion, our patient had been asymptomatic until 25 weeks, when she developed shortness of breath. Although exacerbation of DLAM-related pulmonary symptoms by pregnancy has not been reported, this may be explained by a reduction in functional residual capacity secondary to pregnancy superimposed on a large chylothorax. Furthermore, the increased fluid retention, capillary permeability (24), and intravascular volume expansion that occurs during pregnancy may have created fluid overload that could not be adequately drained by the abnormal lymphatic system. Alternatively, changes in lymph flow regulation during pregnancy (25,26) may have contributed to a further accumulation of chyle. Taken together, a parturient with thoracic lymphangiomatosis and chylothorax may not tolerate a large IV fluid bolus. Care should be taken if the pa-tient requires cesarean delivery, particularly under neuraxial block, during which a large fluid preload is often administered. It should also be kept in mind that sudden lung expansion after drainage of chronic pleural effusion can cause reexpansion pulmonary edema, jeopardizing the patient and her fetus (27,28), whereas multiple pleural aspirations can result in marked protein loss, lymphocytopenia, and a critically low level of immunity (10).
Most cases of retroperitoneal lymphangiomatosis are asymptomatic (22), whereas with intraperitoneal lymphangiomatosis, the spleen (9,12,13), liver (13–15), kidneys (16–18), and intestines (19–21) can be involved. Thrombocytopenia and consumptive coagulopathy can occur in DLAM involving the spleen (18). It has also been reported after thoracic duct ligation, probably because of increased back-pressure and lymph flow to splenic lymphangiomas (9). Asymptomatic disseminated intravascular coagulation, although rare, has been described in DLAM involving the thorax and retroperitoneum, possibly secondary to local production of fibrinolysin by lymphangiomas (11). We did not obtain a platelet count because of the absence of clinical signs of coagulopathy, preeclampsia, and splenic involvement by MRI. However, we believe that this was an error and recommend that a platelet count and coagulation profile be obtained before neuraxial block, especially if splenic involvement is suspected by MRI. Hypertension secondary to renal artery involvement (18), as well as intestinal bleeding (19), protein-wasting enteropathy (20), and acute abdomen (21) in intestinal lymphangiomatosis have also been described but were not seen in this patient.
In conclusion, DLAM is rare but can affect many organ systems simultaneously, with variable degrees of symptoms. Even a patient who appears to be asymptomatic and otherwise healthy may have significant multisystem involvement. Therefore, before the administration of an anesthetic, a thorough preoperative evaluation of the patient and appropriate laboratory and imaging studies should be conducted to identify the extent of the disease and guide anesthetic management. Particular attention should be paid to possible pleuropulmonary, mediastinal, splenic, bony, and epidural involvement, and a coagulation profile should be obtained before an anesthetic plan is formulated. With this information, an individualized strategy can be developed on the basis of the severity and anatomical locations of the disease.
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Accepted for publication February 24, 2003.
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Abstract
Introduction
