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Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 149-150

Cleft lip and palate with diabetes insipidus: Perioperative management

Department of Plastic and Reconstructive Surgery, Government Medical College, Kozhikode, Kerala, India

Date of Submission24-Nov-2020
Date of Acceptance11-May-2021
Date of Web Publication7-Jun-2021

Correspondence Address:
Dr. Deepak Bhaskaran
Department of Plastic and Reconstructive Surgery, Government Medical College, Kozhikode, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jclpca.jclpca_42_20

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Nasal desmopressin spray is used for the management of diabetes insipidus (DI). Difficulties arise during the perioperative period of cleft repair for the management of DI with nasal desmopressin due to decreased nasal absorption and reduced oral intake. Key to successful peri-operative management is strict monitoring of serum electrolytes, serum and urine osmolality, and fluid administration titrating to urine output. Oral and intravenous desmopressin needs to be used in the perioperative period for managing the condition.

Keywords: Cleft lip, cleft palate, desmopressin, diabetes insipidus

How to cite this article:
Bhaskaran D, Rajan T M. Cleft lip and palate with diabetes insipidus: Perioperative management. J Cleft Lip Palate Craniofac Anomal 2021;8:149-50

How to cite this URL:
Bhaskaran D, Rajan T M. Cleft lip and palate with diabetes insipidus: Perioperative management. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2022 Jul 6];8:149-50. Available from: https://www.jclpca.org/text.asp?2021/8/2/149/317787

  Introduction Top

We would like to discuss a rare case of the cleft lip and palate associated with diabetes insipidus (DI) and perioperative management during cleft lip/palate repair. Only a few cases have been reported in the literature. Nasal desmopressin use in cleft palate /lip patients is difficult. We would like to describe our experience in such a case.

  Case Report Top

A 1-year-old male child with DI presented for cleft lip and palate repair. He was the second child from a non-consanguineous marriage. His mother was on treatment for hypothyroidism. The perinatal period was uneventful. The magnetic resonance imaging of the brain of the child showed benign enlargement of subarachnoid spaces. The child weighed 7.5 kg on admission. He was on treatment with nasal desmopressin spray one dose each day. During his stay, we coordinated closely with the pediatric department during the perioperative period. The reconstruction was done separately for lip and palate at an interval of 6 months.

On preoperative day (pod), we kept the child nothing by mouth for 8 h before surgery and fluid maintenance was given as 250 ml DNS with 2.5ml KCl (5mEq) every 8th h. Urine output was measured hourly using urinary catheterization and fluids were titrated according to urine output. The child was monitored both for features of dehydration and fluid overload. Intraoperatively and perioperatively, 200 ml of ringer lactate was given over 4 h by the anesthetist, and urine output was 220 ml.

During the postoperative period, fluids were given as 30 ml DNS and 0.3 ml KCL hourly. Urine output was monitored 4 hourly, and serum electrolytes and urine osmolality were measured 6 hourly. An extra dose of desmopressin spray was given in conditions such as urine low osmolality, hypernatremia, and high urine output. In the immediate postoperative period due to nasal crusting, nasal spray was less effective due to erratic absorption. We planned for IV desmopressin, but this was not readily available. Hence, oral desmopressin, 0.05 mg, was started as an afternoon dose, plus 2 nasal doses at 6 am and 6 pm. Extra dose of desmopressin was withheld when urine osmolality and serum electrolytes became normal. After adequate oral intake, intravenous fluids were stopped on postoperative day 7. Saline nasal drops were given for nasal crusts before administering spray. Urine osmolality became normal within 24 h; hence, oral dose was stopped.

Cleft palate repair was undertaken after 6 months. A single dose of oral desmopressin was used along with usual nasal spray on the preoperative day. Only oral desmopressin tablet 0.1 mg was given in the postoperative period applied in the form of a paste over the tongue from day 1 evening. Urine output was maintained around 2 ml/kg/h. He had features of mild dehydration on pod 1 due to decreased free water intake. Oral fluids and intravenous DNS were titrated according to urine output. Intravenous fluid was stopped on pod 2. Urinary catheter was removed as he had a fever spike on pod 7 and urine output was calculated using diaper weight. The patient was discharged on pod 11 with oral desmopressin 0.1 mg daily.

  Discussion Top

Hacıhamdioğlu et al.[1] have reported a case of Genoa syndrome consisting of midline cleft lip and palate with holoprosencephaly, optic nerve hypoplasia, and an absence of the septum pellucidum along with growth hormone deficiency and DI; Khourti et al.[2] also reported a similar case.

On reviewing literature, Ozaydin et al.[3] have used a similar approach. A more consistent response was obtained using the oral route than using the nasal route by Stick et al.[4]

Desmopressin is available in the doses of 100-200 μg (oral), 10–20 μg (nasal spray), and 1–2 μg (IM/IV).[5] Guidelines suggest that if a known case of DI requires surgery and needs prolonged oral fluid restriction, the usual dose of desmopressin is withheld before surgery. The child is kept on 1 L/m2/d of restricted intravenous fluids. When the effect of the previous desmopressin dose wanes off and DI sets in, intravenous vasopressin is started as per the protocol.[6],[7] Acute central DI during neurosurgery is managed with care to avoid hyponatremia.[6] As fluid intake more than 1 L/m2/d preoperatively results in dilutional hyponatremia, restriction of fluids to 2/3 normal maintenance is recommended. The onset of DI is suspected if the child becomes polyuric with serum sodium of >145mEq/l and the serum osmolality >300 mOs/kg. It is treated with an intravenous infusion of vasopressin (0.5 mU/kg/h) and titrated every 10 min to maintain a urine output <2 ml/kg/h and a stable rate of intravenous fluid, and vasopressin infusion can be maintained. It is advisable to switch to oral fluids as soon as possible, so that fluid intake is driven by thirst and serum osmolality is better maintained. Intravenous desmopressin should be used with caution because of its long half-life.[6],[7]

We conclude that with a team effort along with pediatrician and anesthesiologists, it is possible to minimize risk and successfully treat children with DI who have a cleft deformity

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We express our sincere gratitude to Dr. H.S Adewalla and Dr. P V Narayanan, Senior Cleft surgeons, Charles Pinto Centre for Cleft Lip, Palate and Craniofacial Anomalies, Jubilee Mission Medical College and Research Institute, Thrissur, Dr. Ajith Kumar, Professor and HOD, Department of Paediatrics, Calicut Medical College, Prof. Mukund Thatte, HOD Plastic Surgery, Bombay Hospital and Wadia Children's Hospital, Mumbai, for their valuable support and guidance.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Hacıhamdioğlu B, Şıklar Z, Savaş Erdeve Ş, Berberoğlu M, Deda G, Tıraş ST, et al. Genoa syndrome and central diabetes insipidus: a case report. J Clin Res Pediatr Endocrinol. 2010;2:89-91.  Back to cited text no. 1
Kourti M, Pavlou E, Rousso I, Economou I, and Athanassiadou F. Holoprosencephaly and Diabetes Insipidus in a 3-Month-Old Infant. J of Child Neurology 2008:23:118–20.  Back to cited text no. 2
Ozaydin E, Aycan Z, Yusufoglu AM, Cetinkaya E, Ergen S, Unal S, et al. Management of central diabetes insipidus with oral desmopressin in a patient with ectrodactyly and cleft lip/palate (ECP) syndrome. Turk J Pediatr. 2009;51:89-93.  Back to cited text no. 3
Stick SM, Betts PR. Oral desmopressin in neonatal diabetes insipidus. Arch Dis Child. 1987;62:1177–8.  Back to cited text no. 4
Baldeweg SE, Ball S, Brooke A, Gleeson HK, Levy MJ, Prentice M, et al. Society For Endocrinology Clinical Guidance: Inpatient management of cranial diabetes insipidus. Endocr Connect. 2018;7:G8–11.  Back to cited text no. 5
Wise-Faberowski L, Soriano SG, Ferrari L, McManus ML, Wolfsdorf JI, Majzoub J, et al. Perioperative management of diabetes insipidus in children. J Neurosurg Anesthesiol. 2004;16:220–5.  Back to cited text no. 6
Muglia LJ, Majzoub JA. Pediatric endocrinology. Disord Posterior Pituitary WB Saunders Co, Philadelphia. 1996;206.  Back to cited text no. 7


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