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Year : 2003  |  Volume : 13  |  Issue : 3  |  Page : 297-300
Hand schuller christian disease causing diabetes insipidus


Department Radiology, Civil Hospital, Asarwa, B.J. Medical College, Ahmedabad-380016, India

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Keywords: Hand Schuller Christian disease, Diabetes Insipidus

How to cite this article:
Shah A K, Solanki R N, Mahajan A. Hand schuller christian disease causing diabetes insipidus. Indian J Radiol Imaging 2003;13:297-300

How to cite this URL:
Shah A K, Solanki R N, Mahajan A. Hand schuller christian disease causing diabetes insipidus. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Feb 28];13:297-300. Available from: http://www.ijri.org/text.asp?2003/13/3/297/28702

   Introduction Top


We wish to report "Hand Schuller Christian Disease causing diabetes insipidus." Diagnosed by various radiological and imaging modalities and confirmed by histopathology and immunocytochemistry.


   Case report Top


Informant father of four and half year old male child described illness started two years back. There is history of increased frequency of micturation with increased water intake for two years. There is history of vomiting for 15 days 2-3 times per day. There is also history of swelling over right parietal region for two years with no history of trauma.

Patient was on nasal desmopressin plus steroids since one and a half years. Patient developed signs of steroid toxicity (stria, abdominal distension and puffy face) for which steroids were discontinued.

No history of trauma on scalp, and no history of psychiatric illness.

'X-ray skull lateral view' [Figure - 1] was done which shows lytic geographical area in right high parietal region. X-ray chest supine view was normal.

MRI brain plain and contrast was done [Figure - 2],[Figure - 3] which shows calvarial lesion in the right high parietal region and appears hypointense on T1WI and hyperintense on T2 WI and shows post contrast enhancement erosion with overlying soft tissue component and involvement of the underlying dura.

INVESTIGATIONS :

URINE OSMOLALITY----- 1.005

S.CORTISOL-------- 3.1(NORMAL 6.5---26)

AFTER TREATMENT :

S.OSMALALITY--------------------- 273mosmol/ltr

PLASMA SODIUM------------------ 138meq/ltr

PLASMA SODIUM/S.OSMOLALITY--- 0.5

HEMOGLOBIN ------------------------- 11.2gm/dl

TOTAL COUNT----------------------- 150100ccm

DIFFERENTIAL COUNT------------ 40/56/2/2/0

URINARY SPECIFIC GRAVITY----- 1.02

HISTOPATHOLOGY (20/2/2003) GCRI

SPECIMEN : INCISIONAL BIOPSY OF RIGHT PARIETAL BONY REGION

IMPRESSION : SUGGESTIVE OF LANGERHANS

CELL GRANULOMATOSIS

IMMUNOHISTOCHEMISTRY (3/3/2003) :

LANGERHANS CELL GRNULOMATOSIS


   Discussion Top


Hand Schuller Christian Disease is a variant of Langerhans Cell Histiocytosis.

Langherhans cell Histiocytosis is a disease of unknown etiology that covers a wide spectrum of pathological and clinical features course and prognosis.It is a characterized primarily by abnormal proliferation of Histiocytes. Estimated incidence of (LCH) range from 0.05 to 0.5 per 100000 population annually.

The Langherhans cell is considered today to be the main element in lesions of Histiocytosis X and presence in some known for the diagnosis of this disorder.

The term encompasses three clinical and pathological syndromes, once believed to represent distinct entities but now considered different eponyms of the same fundamental disorder.

The syndromes are : 1) Letterer -Siwe Syndrome ( Disseminated Histocytosis) Letterer-Siwe Syndrome, the acute disseminated form of Histocytosis X that occurs in infants is rapidly progressive and affects primarily soft tissues with no or minimal bone involvement. 2) Hand Schuller Christian Disease ( Multifocal Histiocytosis X syndrome) The chronic form of disseminated Histiocytosis X that usually affects young children and is characterized by visceral and variable degree of bone involvement

Clinical Features :

The triad of calvarial lesions, exophthalmos and dibetes insipidus is not constant found only in 10% of cases. Seventy five percent of these go on to disease free survival but require hormonal replacement. About half of the patients show diabetes insipidus early in the disease, which respond well to pituitrin.

With disseminated disease systemic manifestations such as weakness, weight loss, fever and loss of appetite and failure to thrive occur.Patient with monoarticular lesions are asymptomatic. Solitary or Multifocal bone lesions usually present with pain-localized tenderness, soft tissue swelling and limitation of motion.

Radiological Findings:

1) Skeletal Most common site for solitary bone lesion is skull followed by long bones extremities the pelvis, the ribs & the spine.Bone lesions are characterized in radiographs by round or oval lytic defects involving the medullary cavity with slightly denser and at times scalloped margins.

Skull lesions are round or oval and are punched out with non sclerotic margins, uneven involvement of the inner and outer cranial table gives a beveled edge appearance to many lesions resembling a "hole in a hole" Perforation of either cranial table may produce extension in a epidural space or soft tissues. Enlargement, coalescence of initial lytic lesions results in scattered irregular areas of bone destruction and a morphological appearance which is responsible for the term "Geographic Skull". A unidense focus within a lytic cranial lesion has been termed "Button Sequestrum".Lesions of the jaw are present in forty percent.

2) Extra skeletal manifestations It involves mainly hypothalamus, infundibulum and Optic chrasma. CNS involvement is generally associated with diabetes insipidus. Gadolinium enhanced MR imaging is essential for evaluation of Hypothalamus Pituitary Axis in children with LCH. In patients with LCH thickening with abnormal enlargement of infundibulum and median eminence are seen in LCH with diabetes insipidus.

Patients with associated Anterior Pituitary dysfunction such as Growth Hormone insufficiency may have small Adenohypophysis. Histiocytosis may mimic other hypothalamic region lesions of childhood most notably granulomas and Hypothalamic gliomas.

3) Eosionophilic Granuloma Which usually presents as a solitary bone lesion in an older child and may be asymptomatic or produce symptoms limited to bone[5].

 
   References Top

1.D.J. Stoker and A.Saifuddin ; Myeloproliferative and similar disorders .In R.G. Granger, D.J.Allision ,A.Adam, A.K Dixon, Diagnostic radiology (Volume -3) fourth edition Churchill Livingstone. Edinburgh, PP 1916-1917.  Back to cited text no. 1    
2.Mayer J.S, Harty MP, Mahboubi S etal 1995 Langerhans cell histiocytosis; presentation and evaluation of radiological findings with clinical correlation. Radiographics 15:1135-1146.   Back to cited text no. 2    
3.Cheemum Lum, WalterKucharczyk, Walter J Montancra and Laurence E. Becker. The Sella turcica and Paracellar region in Magnetic Resonance Imaging of Brain and Spine (Volume 2) Third edition Lippincott and Williams and Wilkins PP 1340-1342.   Back to cited text no. 3    
4.Tie R D, Newton TH, Mc Dermott MW, et al Thickened Pituitary Stalk On MR Images in patients with diabetes insipidus and Langerhans cells histiocytosis, AJNR Am of Neuroradiology 1990;11:703-708.   Back to cited text no. 4    
5.Donald Resnick, M.D Lipidoses, Histiocytosis and Hyperlipoprotenimeas in Donald Resnick, M.D Bone and Joint Imaging (2nd edition) W.B Saunders Company. PP 615-618  Back to cited text no. 5    

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Correspondence Address:
R N Solanki
39 Suryanagar Society, in front of Gandhi Ashram Post Office, Gandhi Ashram, Ahmedabad-380027
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]



 

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