Through the Anaesthetic lens: Managing challenges in a parturient with Empty Sella Syndrome and Idiopathic Intracranial Hypertension.

 (Dr Divyangna Sapra, Dr Mruthunjaya Hulgur)

 

Introduction:

• The pituitary gland located at the base of the brain within the sella turcica, regulates endocrine function
• Empty Sella Syndrome (ESS): Rare condition with absent or compressed pituitary gland.^1,2

                        Primary: Defect in the diaphragma sella,

                        Secondary: Due to space-occupying lesions, surgery, or ↑ ICP

• Idiopathic Intracranial Hypertension (IIH): Rare condition with ↑ ICP without apparent pathology.

 

Challenges:

• Rarity of ESS and IIH during pregnancy.
• Scarcity of information and the diagnostic complexities
• Multidisciplinary approach and recognizing potential complications.

 

 Case report:

•  Patient Details:
• 36-year-old primipara (BMI 32) with dual diagnosis of IIH and ESS.
• Scheduled for elective caesarean section under spinal anaesthesia at 36+6 weeks.
• Medical History: Chronic headaches, hypertension, visual disturbances
• Pregnancy exacerbated her symptoms, necessitating a multidisciplinary intervention.
• The overlap of symptoms with pregnancy-induced hypertension, pre-eclampsia, and post dural puncture headache complicated the diagnosis.
• Managed with acetazolamide (teratogenic before 20 weeks), antihypertensives and analgesics
• Preoperative Evaluation:

• Blood tests, endocrine workup: Normal
• Neurological and systemic examinations: Normal
• MRI: No signs of raised ICP
• Regular neurological and ophthalmic reviews: No papilloedema, opening pressure = 27cmH₂O & normal CSF composition on LP
• Risks and benefits of various anaesthesia options explained to patient.
• Anaesthetic Considerations:
• Choice of anaesthesia: While dural puncture is contraindicated in patients with ↑ ICP from SOLs to avoid coning, evidence supports spinal anaesthesia safety in IIH.
• CSF drainage helps prevent herniation by maintaining uniform brain swelling, making RA preferable over GA, which could elevate ICP.³
• Avoid increase in ICP during labour pains and uterine contractions:
• Adequate analgesia, shortening 2^nd stage of labour, slow epidural injections to reduce transmission of pressure to subarachanoid space.
• Postpartum pituitary necrosis following severe haemorrahge and hypotension may worsen ESS
• Monitoring BP, IV fluids and titrating phenylephrine along with availability of vasopressin in-case of hypotension due to posterior pituitary insufficiency exacerbated by surgical stress.⁴

• Adequate postoperative analgesia.
• Outcome and Follow-Up:

• Successful delivery of a healthy baby.
• Normal postoperative investigations.
• Continued acetazolamide and regular follow-ups.

 

Discussion:

• ESS, often discovered incidentally, requires attention to both anterior and posterior pituitary functions.
• The stress from surgery or anaesthesia may unmask pituitary insufficiency, emphasizing the importance of early diagnosis and  hormonal replacement.
• The patient's complex history involved secondary ESS possibly caused by persistently ↑ ICP due to IIH.
• The successful maternal and foetal outcome in this case underscores the significance of a multidisciplinary team (obstetric, anaesthetic, neurology and ophthalmology), early diagnosis, and individualized anaesthetic management.

 

Learning Points:

1. Comprehensive perioperative planning and thorough pre-op optimization and multidisciplinary team collaboration.
2. Early establishment of an anaesthesia plan, coupled with active patient involvement, contributed to a positive patient experience.
3. Perioperative Vigilance: Anticipating and mitigating perioperative pitfalls.
4. Individualized Care tailored to unique patient profiles.

 

References:

1. Fouad W. Review of empty sella syndrome and its surgical management. Alexandria Journal of Medicine 2011;47(2):139–47. Doi: 10.1016/j.ajme.2011.06.005.

2. Lamont SNJ, McBride WT, Bill KM, Varadarajan B. Profound vasodilatory hypotension in a patient with known empty sella syndrome following cardiac surgery. Anaesthesia 2007;62(8):846–9. Doi: 10.1111/j.1365-2044.2007.05105.x.

3. Bagga R, Jain V, Das CP, Gupta KR, Gopalan S, Malhotra S. Choice of therapy and mode of delivery in idiopathic intracranial hypertension during pregnancy. MedGenMed : Medscape general medicine 2005; 7: 42.

4. Lamont SNJ, McBride WT, Bill KM, Varadarajan B. Profound vasodilatory hypotension in a patient with known empty sella syndrome following cardiac surgery. Anaesthesia 2007; 62: 846–9.