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Congenital Nephrogenic Diabetes Insipidus and the Trauma Injured Brain: A Case Report
Ryan Davids*, Nadiya Ahmed, Reinhard Overmeyer, Abdurragmaan Domingo
Department of Anaesthesiology and Critical Care, Stellenbosch University, South Africa
Department of Surgical Sciences, Stellenbosch University, South Africa
Received Date: 27/09/2021; Published Date: 02/11/2021
*Corresponding author: Ryan Davids, Anaesthesiology and Critical Care, Stellenbosch University, South Africa
Abstract
Diabetes insipidus is commonly attributed as post-surgical complication of neurosurgical procedures. The case presented here describes the antithesis, where a young man with nephrogenic diabetes insipidus requires emergency neurosurgical intervention. Complex treatment goals and fluid strategies are discussed.
Keywords: Case Report, Nephrogenic Diabetes Insipidus, Fluid management, Traumatic Brain Injury
Introduction
Diabetes Insipidus (DI) is not a common pathology, with an overall prevalence of 1 in 25,0000. The congenital forms are even more infrequent accounting for less than 10% of these cases. In both central and nephrogenic DI patients produce large quantities of dilute urine resulting in electrolyte abnormalities. Hypernatremia and hypokalaemia being the most prominent abnormalities observed [1].
More than 20% of patients present with central DI after neurosurgical intervention [2]. The case below speaks to the inverse, where a young man with congenital nephrogenic DI presents for neurosurgery after trauma. This rare presentation juxtaposes treatment goals for the dual pathology as the fluid management of any traumatic brain injured patient is contentiously debated, compounded here by the pre-existing water and electrolyte derangement in the form of nephrogenic diabetes insipidus. The discussion to follow identifies the specific treatment goals for the perioperative management of this complex patient, furthermore, proposes a pragmatic approach to fluid and electrolyte management in this acute setting.
Case
A 13-year-old male was involved in a high velocity motor vehicle accident. Upon arrival in the emergency department, he was found to have a decreasing level of consciousness as well as features in keeping with shock, presumed at the time to be hypovolemic in nature. Immediate resuscitatory efforts were launched. Which included crystalloid and colloid resuscitation as well as the initiation of inotropy. His initial vital signs indicated Blood pressure 91/54, HR 162 bpm, Oxygen Saturation of 91% on 40% face mask oxygen at respiratory rate of 32 bpm. The young man’s Glasgow Coma Scale (GCS) continued to fall prompting intubation and ventilation. Basic bedside imagery, primary and subsequent surveys could not account for the ongoing decreasing GCS as well as the hemodynamic instability. Urgent radiological investigation was requested revealing that our patient had a large subarachnoid hemorrhage and was in need of urgent decompressive craniectomy. At the time of obtaining consent for this emergent procedure it was uncovered that our patient has congenital nephrogenic diabetes insipidus, managed on outpatient basis utilizing diuretics and tap water self-correction of sodium. Prior to surgery our patient had a serum sodium of 192 mmol/l and in need of ongoing fluid resuscitation.
The operative management was uncomplicated. The anaesthetist remarked a difficult resuscitation as fluid choices, electrolyte management and excessive fluid losses in the form of urine and blood proved onerous. During the 60-minute procedure the patient received 1800 ml of Sodium Chloride 0,45% solution, as well as 2 units of packed red cells intravenously as well as 500ml tap water enterally. Electrolytes were checked every fifteen minutes. He did not need inotropy and mean arterial pressures were maintained at sufficient levels throughout. His urine output was 5ml/kg/hr and serum sodium at the end of the procedure was 189 mmol/l. The patient was transferred to a neurosurgical intensive care unit, where he was extubated and deemed neurologically intact with GCS 15/15. He was successfully discharged from the ICU 3 days later.
Discussion
Whole body water and electrolyte balance is regulated by the antidiuretic hormone (ADH) [1]. The physiological response to ADH is to concentrate urine via the V2 receptors on the renal tubules. Nephrogenic diabetes insipidus is characterized by the body’s inability to adequately respond to ADH with resultant high outputs of dilute urine. In most instances of congenital nephrogenic DI, there is abnormal gene coding for the V2 receptors, or the aquaporin channels themselves and as such treatment with desmopressin (DDAVP) has negligible effects [4]. The urine osmolality in these patients is typically less than < 200 mOsm/kg with serum hypernatremia [4].
The majority of patients with nephrogenic DI are able to manage their condition themselves with the use of diuretics and oral replacement of their free water deficit [1]. However, the trauma situation often prevents this management strategy, compounded hereto significant blood and fluid losses. During the resuscitation phase the timing, dose and type of fluid has to be individualised in order to achieve haemostasis, organ perfusion and restore circulatory volume [5]. Minimizing large fluctuations in serum sodium levels is an added treatment target. Resuscitatory fluids, as well as raised intracranial pressure treatment aides such as mannitol and Hypertonic Saline (HTS) contain large sodium loads [2,3]. Despite this there is insufficient evidence to make recommendations on the use of osmotic agents in TBI. Furthermore, the side effects of these drugs may have deleterious effects - especially in patients with already high urine outputs and sodium abnormalities like nephrogenic diabetes insipidus [8-12]. With desmopressin, mannitol and hypertonic saline demonstrating more harm than benefit in this condition, the treatment strategy hinges upon careful fluid management.
The complexity in the resus of a patient with inherited nephrogenic diabetes insipidus is underpinned by the paucity of evidence with which to provide recommendations of fluid management in such cases. Some authors recommend that 5% dextrose water be administered in volumes to match urine output. 0.9% normal saline, in the dose of 10ml/kg in children, is then reserved for resuscitation in the shocked patient. In theory this appears to a viable solution. It would require frequent serum sodium monitoring to avoid the risk of rapid shifts in serum sodium should the administration of 5% dextrose water far exceed the urinary loss. For similar reasons loading with 0.9% saline may also result in rapid changed in sodium level[6].
A proposed recommendation, particularly in the resource limited setting, where resuscitation of such patients is needed is as follows:
- Admission to a unit where strict input and output can be monitored.
- Replace urine losses equally with 5% dextrose water. If patient can take free water enterally, then use the enteral route in combination with 5% dextrose water to match urine losses.
- Twice a day serum sodium level.
- Resuscitation should be achieved using 0.45% saline and only in the shocked patient.
- Multi-disciplinary team approach – paediatrician/intensive care specialist/nephrologist to assist with the fluid management.
Conclusion
Treatment options are few in the scenario provided. Careful fluid choices, strict fluid balance and meticulous electrolyte checks are proposed as the mainstay of therapy in patients with congenital nephrogenic diabetes presenting for operative management.
Declarations
The authors declare no competing interest as well as no financial disclosures.
Acknowledgements
The authors thank the mother of our patient who consented to the publication of this case report.
Authors’ contributions
RAD case description, literature search, scientific write-up; NPA case description, literature search, scientific validity; RO literature review, scientific validity, AD literature review, scientific validity.
References
- Stanhewicz AE, Kenney WL. Determinants of water and sodium intake and output. Nutrition Reviews. 2015 Aug 18; 73: 73–82.
- Zheng K, Xie Y, Li H. Congenital nephrogenic diabetes insipidus presented with bilateral hydronephrosis and urinary infection. Medicine (United States). 2016; 95(22).
- Hui C, Khan M, Radbel JM. Diabetes Insipidus. Medicine (United Kingdom). 2021; 49(8): 495–497.
- Ramesh GH, Uma JC, Farhath S. Fluid resuscitation in trauma: what are the best strategies and fluids? International Journal of Emergency Medicine, 2019; 12(1): 1–6.
- Guarino S, DIplomatico M, Marotta R, Pecoraro A, Furlan D, Cerrone L, et al. Nephrogenic Diabetes Insipidus in Childhood: Assessment of Volume Status and Appropriate Fluid Replenishment. Pediatric Emergency Care. 2020;36(7): E402–E404.
- Wakai A, McCabe A, Roberts I, Schierhout G. Mannitol for acute traumatic brain injury. Cochrane Database of Systematic Reviews 2013; Issue 8. Art No CD001049.
- Shi J, Tan L, Ye J, Hu L. Hypertonic saline and mannitol in patients with traumatic brain injury: a systematic and met-analysis. Medicine 2020; 99: 35.
- Gu J, Huang H, Huang Y, Sun H, Xu H. Hypertonic saline or mannitol for treating elevated intracranial pressure in traumatic brain injury: a meta-analysis of randomized controlled trials. Neurosurg Rev 2019; 42: 499-509.
- Burgess S, Abu-Laban RB, Slavik RS, Vu EN, Zed PJ. A systematic review of randomized controlled trials comparing hypertonic sodium solutions and mannitol for traumatic brain injury: Implications for emergency department management. Annals of Pharmacotherapy 2016; 50(4): 291-300.
- Boone MD, Oren-Grinberg A, Robinson TM, Chen CC, Kasper EM. Mannitol or hypertonic saline in the setting of traumatic brain injury: What have we learned? Surgical Neurol International 2015; 6: 177.
- Miyoshi Y, Kondo Y, Suzuki H, Fukuda T, Yasuda H, Yokobori S. Effects of hypertonic saline versus mannitol in patients with traumatic brain injury in prehospital, emergency department, and intensive care unit settings: a systematic review and meta-analysis. Journal of Intensive Care 2020; 8: 61.
- Carney N, Totten AM, O'Reilly C, Ullman JS, Hawyrluk GWJ, et al. Guidelines for the management of severe traumatic brain injury: Fourth edition. Neurosurgery 2017; 80: 6-15.
