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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 4  |  Issue : 1  |  Page : 38-40

Anesthetic consideration in a preeclamptic parturient with Von Recklinghausen's neurofibromatosis


Department of Anesthesiology and Critical Care, Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India

Date of Web Publication20-May-2014

Correspondence Address:
Tarandeep Singh
Department of Anaesthesia and Critical Care, PT BDS PGIMS, Medical Mor, Rohtak - 124001, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4472.132823

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  Abstract 

Neurofibromatosis is a multisystem genetic disorder that is associated with cutaneous, neurologic and orthopedic manifestations. Type 1 neurofibromatosis is characterized by dermatological lesions, such as benign neurofibromas of the skin and cafι-au-lait spot, and is more common than type 2 neurofibromatosis. Anesthesiologists must be aware of and consider each of the multisystemic complications of the disorder when evaluating and managing patients for surgical procedures. Anesthesia for the pregnant patient with neurofibromatosis is not well documented. We report a successful case of spinal anesthesia for cesarean section in a parturient with type 1 neurofibromatosis.

Keywords: Neurofibromatosis, parturient, preeclampsia


How to cite this article:
Singh T, Hooda S, Anand A, Kaur K, Bala R. Anesthetic consideration in a preeclamptic parturient with Von Recklinghausen's neurofibromatosis. J Obstet Anaesth Crit Care 2014;4:38-40

How to cite this URL:
Singh T, Hooda S, Anand A, Kaur K, Bala R. Anesthetic consideration in a preeclamptic parturient with Von Recklinghausen's neurofibromatosis. J Obstet Anaesth Crit Care [serial online] 2014 [cited 2018 Apr 25];4:38-40. Available from: http://www.joacc.com/text.asp?2014/4/1/38/132823


  Introduction Top


Neurofibromatosis is a multisystem genetic disorder that is associated with cutaneous, neurologic and orthopedic manifestations. Type 1 neurofibromatosis (NF 1), also referred to as von Recklinghausen's disease, is characterized by cafι-au-lait spots and benign cutaneous neurofibromas. Type 2 neurofibromatosis (NF 2) affects the central nervous system (CNS) via spinal cord tumors and bilateral vestibular Schwannomas. [1],[2] Anesthesiologists must be aware of and consider each of the multisystemic complications of the disorder when evaluating and managing patients for surgical procedures. We report a successful case of spinal anesthesia for cesarean section in a preeclamptic parturient with NF 1.


  Case report Top


A 26-year-old booked primigravida (height 159 cm, weight 68 kg) with 37 weeks' gestation and a known case of von Recklinghausen's neurofibromatosis with mild preeclampsia was scheduled for elective lower segment cesarean section (LSCS) in view of breach presentation. Past medical history revealed - NF 1, which was diagnosed 8 years ago with the characteristic cafι-au-lait spots and benign cutaneous neurofibromas. There was no history of previous surgeries and drug allergies. On examination, her pulse was regular with 96 beats per minute, blood pressure 150/100 mmHg and respiratory rate 22 per minute. She had bilateral pitting pedal edema. She had mouth opening of three fingers with no mass in the oral cavity. Her Mallampati grade was three with normal extension and flexion of the neck. There were no oral mass. Auscultation revealed normal heart and breath sounds. There were no neurological symptoms and no mass was observed in the tongue, pharynx and larynx. Her laboratory investigations showed hemoglobin of 11.0 gm%. Results of serum electrolytes and renal and liver functions were within normal values, except proteinuria (2+). She was advised oral ranitidine (150 mg) at bed time on the night before and on the morning of surgery following an overnight fast.

Inside the operating room, monitoring included 5-lead ECG, noninvasive blood pressure and SpO 2 . Preloading was carried out with 1 L of lactated Ringer's solution after securing an IV line with an 18 G cannula. The patient was positioned as left lateral decubitus [Figure 1]. After preparation of the back of the patient, appreciation of the tips of the spinous processes and interspinous spaces, sub-arachnoid space was located in the L3-L4 space with a midline approach. Lumber puncture was performed with a 25G Quincke's spinal needle and subarachnoid block established using 1.8 mL of 0.5% hyperbaric bupivacaine (9 mg). The patient was placed in the supine position and a wedge inserted under the right hip to minimizes aorto-caval compression. A T6 sensory block was obtained after 2 min. A male infant weighting 2.5 kg was delivered after 10 min. Inj. oxytocin 2.5 IU bolus followed by infusion at a rate of 10 IU/h was commenced after clamping of the umbilical cord. The APGAR scores were 9 and 10 at 1 and 5 min, respectively. The patient remained hemodynamically stable throughout the surgery. She received a total of 1700 mL of crystalloid and the urine output was 100 mL intraoperatively. Surgery lasted for 40 min. Postoperative analgesia was obtained with diclofenac transdermal patch 200 mg once a day for the next 48 h. The postoperative course of the patient was uneventful and she was discharged on the sixth day.
Figure 1: Benign cutaneous neurofibromas sized 1-2 cm spread throughout the entire body as well as the back

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  Discussion Top


Neurofibromatosis is an inherited autosomal dominant disease and is classified as type 1 and type 2. It is caused by a mutation of different chromosomes, type 1 in chromosome 17q11.2 and type 2 in chromosome 22q12.1. NF 1 is characterized by dermatological lesions such as benign neurofibromas of the skin and café-au-lait spots, and is more common than NF 2. [1],[2],[3],[4] Neurofibromas may also be found in the oropharynx and larynx, and can produce difficulties with laryngoscopy and tracheal intubation. [5] Pulmonary pathology includes pulmonary fibrosis and cystic lung disease. [6] The cardiovascular manifestation includes hypertension, which may be associated with pheochromocytoma or renal artery stenosis. [7],[8] Neurofibromas may also affect the gastrointestinal tract and carcinoid tumors may be found in the duodenum. [9],[10] In patients with multiple cervical neurofibromas, painless dislocation of the cervical vertebrae has been reported. It has been suggested that radiographic examination of the neck should be performed before administering anesthesia in these patients in order to avoid spinal cord damage during laryngoscopy and tracheal intubation. [11]

NF 2 characteristically has bilateral vestibular Schwannomas leading to gradual hearing loss. [12] Other clinical features such as meningioma of the brain, Schwannoma of the cranial, spinal or peripheral nerve and juvenile cortical cataract can also exist. [13]

Neurofibromatosis appears to have no intrinsic effect on fertility; a high rate of spontaneous abortion and stillbirth has been reported. Neurofibromatosis parturients are known to be associated with hypertension, HELLP syndrome, spontaneous abortion and increasing of number and size of neurofibromas. [14],[15]

Anesthesia for the pregnant patient with neurofibromatosis is not well documented. Because of the involvement of the CNS, regional anesthesia in NF 2 without careful preoperative examination can be extremely dangerous, and many anesthesiologists prefer general anesthesia. On the other hand, regional anesthesia could be useful in NF 1 because CNS involvement is rare. Additional masses in the tongue, pharynx and larynx in NF 1 may interfere with intubation during general anesthesia, although our patient had no such oral pathology. Also, there have been many reports suggesting an increased sensitivity of patients with NF 1 to nondepolarizing neuromuscular blocking drugs. [16] Thus, they should have neuromuscular transmission monitored when neuromuscular blocking drugs are used. In addition to NF 1, our patient was a preeclampic parturient, thereby increasing the risk for airway-related complications due to associated anatomic and physiologic changes. Complications of general anesthesia in hypertensive parturients include intracranial hemorrhage or pulmonary edema due to extensive increase of blood pressure during tracheal intubation and extubation. Keeping in view that regional anesthesia is relatively safe in NF 1, and considering the inherent complications of general anesthesia in preeclamptic parturients, we opted for subarachnoid block in this patient.

To conclude, manifestations of neurofibromatosis are often mild, but there may be associated pathology of direct relevance. Anesthesiologists should consider the complex and diverse associated factors to provide optimal anesthesia. Weather to proceed with general or regional anesthesia in these cases must be viewed within the appropriate clinical context, like associated system involved and type of surgery.

 
  References Top

1.Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: Clinical presentations and anaesthetic implications. Br J Anaesth 2001;86:555-64.  Back to cited text no. 1
    
2.Spiegel R, Mächler M, Stocker HP, Boltshauser E, Schmid W. Neurofibromatosis Type 1: Genetic studies with DNA markers in 38 families. Schweiz Med Wochenschr 1991;121:1445-52.  Back to cited text no. 2
    
3.Jouhilahti EM, Peltonen S, Heape AM, Peltonen J. The pathoetiology of neurofibromatosis 1.Am J Pathol2011;178:1932-9.  Back to cited text no. 3
    
4.Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: A twenty first century perspective. Lancet Neurol 2007;6:340-51.  Back to cited text no. 4
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5.Baden E, Pierce HE, Jackson WF. Multiple neurofibromatosis with oral lesions; review of the literature and report of a case. Oral Surg Oral Med Oral Pathol 1955;8:263-80.  Back to cited text no. 5
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6.Burkhalter JL, Morano JU, McCay MB. Diffuse interstitial lung disease in neurofibromatosis. South Med J 1986;79:944-6.  Back to cited text no. 6
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7.Salyer WR, Salyer DC. The vascular lesions of neurofibromatosis. Angiology 1974;25:510-9.  Back to cited text no. 7
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8.Bourke E, Gatenby PB. Renal artery dysplasia with hypertension in neurofibromatosis. Br Med J 1971;3:681-2.  Back to cited text no. 8
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9.Dayal Y, Tallberg KA, Nunnemacher G, DeLellis RA, Wolfe HJ. Duodenal carcinoids in patients with and without neurofibromatosis. A comparative study. Am J Surg Pathol 1986;10:348-57.  Back to cited text no. 9
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10.Basile U, Cavallaro G, Polistena A, Giustini S, Orlando G, Cotesta D, et al. Gastrointestinal and retroperitoneal manifestations of type 1 neurofibromatosis. J Gastrointest Surg 2010;14:186-94.  Back to cited text no. 10
    
11.Lovell AT, Alexander R, Grundy EM. Silent, unstable cervical spine injury in multiple neurofibromatosis. Anaesthesia 1994;49:453-4.  Back to cited text no. 11
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12.Kanter WR, Eldridge R, Fabricant R, Allen JC, Koerber T. Central neurofibromatosis with bilateral acoustic neuroma: Genetic, clinical and biochemical distinctions from peripheral neurofibromatosis. Neurology 1980;30:851-9.  Back to cited text no. 12
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13.Hope DG, Mulvihill JJ. Malignancy in neurofibromatosis. Adv Neurol 1981;29:33-56.  Back to cited text no. 13
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14.Segal D, Holcberg G, Sapir O, Sheiner E, Mazor M, Katz M. Neurofibromatosis in pregnancy. Maternal and perinatal outcome. Eur J Obstet Gynecol Reprod Biol 1999;84:59-61.  Back to cited text no. 14
    
15.Hadi HA. Clinical significance of neurofibromatosis in pregnancy. Am J Perinatol1995;12:459-61.  Back to cited text no. 15
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16.Magbagbeola JA. Abnormal responses to muscle relaxant in a patient with Von Recklinghausen's disease (multiple neurofibromatosis). Br J Anaesth 1970;42:710.  Back to cited text no. 16
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