|LETTERS TO EDITOR
|Year : 2020 | Volume
| Issue : 2 | Page : 168-170
Anesthesia management of a patient with advanced juvenile Paget's disease
Prasanna Vadhanan1, Nikhilesh Bokka1, Cheran Kalyanasundaram1, Saravanan Kasirajan2
1 Department of Anaesthesiology, Vinayaka Missions Medical College, Karaikal, Puducherry, India
2 Department of Orthopedics, Vinayaka Missions Medical College, Karaikal, Puducherry, India
|Date of Submission||11-Jun-2020|
|Date of Decision||16-Jun-2020|
|Date of Acceptance||23-Jun-2020|
|Date of Web Publication||19-Sep-2020|
Prof. Prasanna Vadhanan
Department of Anaesthesiology, Vinayaka Missions Medical College, Karaikal - 609 609, Puducherry
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Vadhanan P, Bokka N, Kalyanasundaram C, Kasirajan S. Anesthesia management of a patient with advanced juvenile Paget's disease. Indian Anaesth Forum 2020;21:168-70
|How to cite this URL:|
Vadhanan P, Bokka N, Kalyanasundaram C, Kasirajan S. Anesthesia management of a patient with advanced juvenile Paget's disease. Indian Anaesth Forum [serial online] 2020 [cited 2020 Oct 24];21:168-70. Available from: http://www.theiaforum.org/text.asp?2020/21/2/168/295389
Juvenile-onset Paget's disease (JPD) is an extremely rare disease (incidence <1 in 100,000) of bone metabolism characterized by rapid bone turnover. Very few case reports of anesthesia management in JPD exist. We discuss the management of such a patient twice – once for a cholecystectomy and subsequently for a cesarean section.
A 25-year-old female presented with complaints of recurrent upper abdominal pain and was planned for elective cholecystectomy. She had a history of progressive skeletal deformity from infancy affecting her lower limbs initially. The deformities gradually progressed and involved the spine, shoulder, hip and knee joints. The key clinical features and investigations are listed [Figure 1] and [Table 1]. At the age of 20, she was diagnosed as JPD due to elevated alkaline phosphatase and a bone biopsy. She was on calcitonin nasal spray, alendronate, and Vitamin D supplements.
|Figure 1: The progression of the disease over the period of 5 years. (a) Normal neck extension and flexion deformity of the hips. (b) Taken 5 years later shows fixed flexion of the spine, hip, and knee joints with genu varum. Abduction of the shoulder joint was also limited. (c) Fixed kyphotic posture, (d) Radiograph showing fused spine, hip joints curved femur with ankylosis of hip, and SI joints and enlarged skull. (e) Positioning done in beach chair, breasts taped up to optimize surgical access, ProSeal Laryngeal mask airway in situ|
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Informed written consent was obtained. A trial of patient positioning was done with adequate supports, and the surgeon opted for an open cholecystectomy. The patient was premedicated with oral diazepam 5 mg, ranitidine 150 mg, and ondansetron 4 mg. Standard monitors (electrocardiography, noninvasive blood pressure, SpO2, and EtCo2) were applied. After preoxygenation, injection midazolam 1 mg and injection fentanyl 50 μg were administered, and anesthesia was induced with injection propofol 110 mg. A size 3 ProSeal® laryngeal mask airway (LMA) was introduced, the cuff was inflated with 20 ml of air, and a gastric suction tube was placed. The EtCo2 trace was satisfactory. Bilateral ultrasound-guided subcostal transverse abdominis plane (TAP) block was given with 20 ml of 0.25% bupivacaine. Anesthesia was maintained using oxygen and nitrous oxide (50:50) and sevoflurane (2%) with spontaneous ventilation and gentle manual assistance. At the end of the procedure, ProSeal was removed. Postoperative period was uneventful.
After 13 months, she presented again for an emergency cesarean section for fetal distress. Anesthesia was administered using the same technique (preoxygenation, propofol followed by ProSeal LMA, spontaneous ventilation under sevoflurane, fentanyl 50 μg after baby delivery, and bilateral posterior TAP block) and was uneventful.
JPD, first described in 1956, is characterized by mutations of gene TNFRFS11B which encodes osteoprotegerin, resulting in rapid bone turnover. A recent exhaustive review by Polyzos et al. describes the disease in detail. Extraskeletal manifestations include deafness, visual loss, vascular calcifications, Chiari malformations, and carotid artery aneurysms. Diagnosis is usually made in childhood due to failure to thrive and elevated levels of alkaline phosphatase; the levels decrease with advancing age. Our patient had all the radiographic features of JPD.
In JPD, unanticipated difficult airway has been reported, and central neuraxial blockade is equally problematic due to the calcified spine. Successful spinal anesthesia has been reported; our patient had uneventful spinal anesthetics before 8 years, but her disease has progressed since rendering them untenable. A ProSeal LMA was chosen instead of awake fiber-optic intubation mainly due to the ease of insertion and to avoid muscle paralysis. Spontaneous ventilation might offer unique benefits, and it essentially necessitates a good analgesia, provided by TAP block. Second-generation supraglottic airway devices have been shown as an acceptable choice in obstetric surgeries. During both the surgeries, the surgeons were reminded to be gentle and avoid excessive retractions and bowel manipulations.
In conclusion, understanding the myriad effects of JPD, a balanced anesthesia technique, good communication between the anesthesiologist, the patient, and the surgeon will help in avoiding complications and improving outcomes.
The Authors wish to acknowledge Prof. Ambujam, MS, Dean and Prof. Sambandamurthy MS, Head, Department of surgery, Vinayaka Missions Medical College for their contributions.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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