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Year : 2016  |  Volume : 9  |  Issue : 5  |  Page : 642-644  

Benign intracranial hypertension associated with hypothyroidism in a hemophilic child

1 Department of Ophthalmology, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Paediatrics, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication13-Oct-2016

Correspondence Address:
Chandana Chakraborti
A/1/1, Pearl Apartment, 50B Kailash Bose Street, Kolkata - 700 006, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.192162

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Benign intracranial hypertension (BIH) due to hypothyroidism in children is rare. We report a case of BIH due to hypothyroidism with bilateral sixth nerve palsy in a hemophilic child. The child presented with headache, vomiting, and inward deviation of both eyes for 10 days. On examination, her vision was 20/40 both eyes with restricted abduction in both the eyes. Anterior segments were within normal limits. Fundus showed bilateral disc edema. No marked abnormality was detected by neuroimaging. Baseline investigations were normal except a raised thyroid stimulating hormone. We started the patient on tablet acetazolamide, domperidone, and systemic steroids. Disc edema and ocular movements started improving. We have not come across any such case in literature after thorough PubMed search. The presentation, management, and future problems of this rare case are discussed.

Keywords: Childhood benign intracranial hypertension, haemophilia, hypothyroidism

How to cite this article:
Chakraborti C, Barua N, Chishti R, Mazumdar J, Kumar S. Benign intracranial hypertension associated with hypothyroidism in a hemophilic child. Med J DY Patil Univ 2016;9:642-4

How to cite this URL:
Chakraborti C, Barua N, Chishti R, Mazumdar J, Kumar S. Benign intracranial hypertension associated with hypothyroidism in a hemophilic child. Med J DY Patil Univ [serial online] 2016 [cited 2020 Nov 30];9:642-4. Available from:

  Introduction Top

Benign intracranial hypertension (BIH) is defined by modified Dandy's criteria that includes symptoms and signs suggestive of increased intracranial pressure (ICP), normal cerebrospinal fluid composition, no abnormal neurologic finding apart from occasional sixth nerve palsy, no episode of impaired consciousness, for which no secondary cause is evident on neuroimaging or other evaluations. The incidence in the overall population of pediatric age group is low, 1 out of 100,000.[1] It is common among adolescents (12-15 years) with female predominance as compared to young children (2-12 years) where males are affected more.[2],[3]

  Case Report Top

A 3-year-old child, known hemophilic A, presented to ophthalmology OPD with chief complaints of vomiting, headache along with inward deviation of both eyes for 10 days. Headache was intermittently associated with nausea and few episodes of vomiting. Inward deviation was noticed by parents mostly in the right eye [Figure 1]. There was no history of convulsion, fever, rash, joint pain, altered sensorium, or any neurologic deficit. He had received transfusion of free frozen plasma three times in last 1 year (last transfusion 2-3 months before this episode).
Figure 1: Clinical photograph of patient showing inward deviation of both eyes (right > left)

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On examination, the patient was alert, conscious, and slightly irritable. Ocular examination revealed best corrected visual acuity of 20/40 both eyes with normal pupillary reactions. Hirschberg test showed deviation of 30° and 15° in the right and left eyes, respectively, with restricted abduction in both eyes, (right more than left) suggesting bilateral sixth nerve palsy [Figure 2]. Fundus revealed bilateral papilledema [Figure 3]. Contrast enhanced magnetic resonance imaging of brain and orbit showed no abnormality [Figure 4]. Further blood investigations showed normal values of complete blood count, liver function test, and electrolytes. His recent prothrombin time was 12 s. Thyroid stimulating hormone level was 19.08 uIU/ml (normal limit 0.39-6.16 uIU/ml) with normal T3 and T4(FT3 1.32 pg/ml, FT4 1.98 ng/ml) levels. A diagnosis of BIH with bilateral sixth nerve palsy in a case of hemophilic hypothyroid child was made. After neurology and pediatric consultation, we started the patient on tablet acetazolamide (40 mg/kg/day 3 divided dose), domperidone (0.5 mg/kg/day in three divided dose), oral prednisolone (1 mg/kg/day, one daily dosing with tapering for 2 months), and tablet eltroxin 50 µg/day. After 2 days, the patient improved symptomatically. There was marked improvement of clinical features, on the 3rd week the papilledema started decreasing and by the 6th week, the optic disc was near normal. Ocular movements started improving and by the 6th week, the left eye showed full abduction and there was minimal deficit in the right eye [Figure 5].
Figure 2: Ocular movements showing abduction deficit in both eyes

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Figure 3: Fundus photograph showing bilateral early papilledema

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Figure 4: Magnetic resonance imaging brain and orbit depicting normal study

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Figure 5: Ocular movements showing resolving sixth nerve palsy

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  Discussion Top

Pediatric BIH is quite different than adults. In children, there are no specific diagnostic criteria of BIH. In adults, the disease affects more obese population, but in pediatric BIH a weak association with obesity has been reported.[3] Hereditary plays an important role in pediatric disease. Some important associations are homozygous and heterozygous female twins, siblings, mother-daughter pairs, mother-son pairs, and cousins.[4]

Secondary causes of BIH are less frequently found in adults in contrast to pediatric population where 53.2-77.7% of cases have definite cause.[5] It can be secondary to endocrinological disorders associated with thyroid replacement,[6] corticosteroid withdrawal, growth hormone supplement, Addison's disease, etc. Other causes reported are infections (acute sinusitis and viral infection), drugs (nalidixic acid, tetracyclines, Vitamin A toxicity, and nitrofurantoin), anemia (iron deficiency, acquired aplastic anemia, and sickle cell disease), malnutrition and renutrition, Miller–Fisher syndrome (ophthalmoplegia, ataxia, and areflexia), and prothrombotic states (antiphospholipid antibodies, hyperfibrinogenemia, anticardiolipin antibodies, lupus, and Behcet's disease).[7],[8]

Neuroimaging plays an important role in excluding secondary causes of increased ICP. CSF opening pressure is another important diagnostic procedure as papilledema may not be present in all cases. In adults, an opening CSF pressure of >250 mm of H2O confirms raised ICP. Normal levels in a young child can vary between 10 and 100 mm of H2O.[9] Lumbar puncture is not advocated in a hemophilic child as in our case, due to increased chance of bleeding.

Carbonic anhydrase inhibitor is the drug of choice in children with BIH. Dose of 15 mg/kg in two to three divided doses per day with maximum limit of 650 mg until headache, disc swelling, and visual field abnormalities subsides. Duration of the treatment may extend up to 3-9 months.[10],[11]

Thyroxine alters CSF dynamics by affecting sodium transport.[12] In our case, thyroid supplementation was started in low dose as few cases of pediatric BIH have been documented following thyroxine replacement therapy in juvenile hypothyroidism.

There are few key issues regarding the management of this type of cases. First, regular follow-ups are needed for early diagnosis of any recurrence. Second, whenever the disease recurs, medical management is the treatment of choice, even with intractable disease surgical intervention being extremely risky. Third is the side effects of starting and withdrawal of steroids. Withdrawal of steroids may precipitate BIH, so it has to be slow tapering. Fourth, thyroid supplementation has to be strictly dose titrated as iatrogenic hyperthyroidism may lead to BIH.

Education to care providers about early signs of disease recurrence and the need for regular follow-up is recommended. Screening of siblings is must as it may run in family. An integrated approach including ophthalmologist, radiologist, neurologist, pediatrician, and hematologist is needed to be taken every time the disease relapses.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-5.  Back to cited text no. 1
Gordon K. Pediatric pseudotumor cerebri: Descriptive epidemiology. Can J Neurol Sci 1997;24:219-21.  Back to cited text no. 2
Babikian P, Corbett J, Bell W. Idiopathic intracranial hypertension in children: The Iowa experience. J Child Neurol 1994;9:144-9.  Back to cited text no. 3
Karaman K, Gverovic-Antunica A, Zuljan I, Vukojevic N, Zoltner B, Erceg I, et al. Familial idiopathic intracranial hypertension. Croat Med J 2003;44:480-4.  Back to cited text no. 4
Scott IU, Siatkowski RM, Eneyni M, Brodsky MC, Lam BL. Idiopathic intracranial hypertension in children and adolescents. Am J Ophthalmol 1997;124:253-5.  Back to cited text no. 5
Campos SP, Olitsky S. Idiopathic intracranial hypertension after L-thyroxine therapy for acquired primary hypothyroidism. Clin Pediatr (Phila) 1995;34:334-7.  Back to cited text no. 6
Lessell S. Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992;37:155-66.  Back to cited text no. 7
Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr 2010;74:381-9.  Back to cited text no. 8
Fishman RA. Cerebrospinal Fluid in Diseases of the Nervous System. 2nd ed. Philadelphia: Saunders; 1992.  Back to cited text no. 9
Schoeman JF. Childhood pseudotumor cerebri: Clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol 1994;9:130-4.  Back to cited text no. 10
Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007;52:597-617.  Back to cited text no. 11
Raghavan S, DiMartino-Nardi J, Saenger P, Linder B. Pseudotumor cerebri in an infant after L-thyroxine therapy for transient neonatal hypothyroidism. J Pediatr 1997;130:478-80.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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