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atelectasis, intrinsic or extrinsic obstruction of a major bronchus and unilateral acquired bronchiolitis (Swer-James-macleod Syndrome).4

The history of recurrent respiratory tract infections throughout his childhood and adolescence signals a chronic or possibly congenital etiology such as hypoplastic pulmonary artery, Swer-James Syndrome

The cT scan report entertained the idea of Swer- James Syndrome (SJS). This arises as a post –infectious complication followed by chronic lung disease showing bronchiolar abnormality, air trapping, and abnormal lung dynamics during inspiration and forced expiration. Patients with SJS have a small lung, compensatory overexpansion of the contralateral lung, peripheral bronchi and bronchioles “pruned” secondary to obliterative bronchiolitis, a mosaic pattern of hyperlucency on cT, and small vessels and vascular occlusions in the abnormal areas.

Our patient; despite the positive history of infection had no abnormalities of pulmonary function. his chest x-ray and cT scan show no evidence of air trapping or bronchiolar abnormality characteristic of SJS.

To further support our diagnosis as congenital unilateral pulmonary artery hypoplasia we have come across various papers describing a considerable incidence of high altitude pulmonary edema; often at moderate altitudes and sometimes recurrent in subjects with unilateral pulmonary artery hypoplasia, underlying the importance of a restricted pulmonary vascular bed in the pathogenesis of hAPE.5

References1. Fraser rS, müller NL, colman N, Pare PD. Developmental

anomalies affecting the pulmonary vessels. In: Fraser RS, Pare PD, Eds. Fraser and Pares Diagnosis of Disease of the chest. Fourth edition. Philadelphia. WB Saunders company 1999;637-675.

2. rene m, Sans J, Dominduez J, et al. Unilateral pulmonary artery agenesis presenting with hemoptysis: Treatment by embolization of systemic collaterals. Cardiovasc Interv Radiol 1995;18:251-254.

3. Sharma A, Bieuei F, Dillon E. Agenesis of the left pulmonary artery with PDA and hypoplasia of left lung. Appl Radiol Online 2004;6:33.

4. mason rJ, Broaddus Vc, murray JF, Nadel JA, editors. murray and Nadel’s textbook of respiratory medicine. 4th ed. Philadelphia (PA): Elsevier Inc 2005;1125-6.

5. Harkel A, Blom N, Ottenkamp J. Isolated Unilateral Absence of a Pulmonary Artery. Chest 2002;122:1471-1477.

were started. As the patient’s saturation did not improve he was non-invasively ventilated with BIPAP. On the 6th day of hospitalization chest x-ray showed significant clearing of the right lung opacities and repeat 2D Echo revealed improvement in pulmonary artery pressure. On the basis of a negative D dimer investigation, pulmonary embolism was ruled out and patient was presumed to have responded to antibiotics and discharged.

Past history revealed that patient had normal milestones. No history of scholastic backwardness.

Patient is actively involved in sports with no history of reduced effort tolerance, cyanosis or syncope. Non-smoker. No relevant family history.

On general examination at present admission; he was found to have normal growth and development for his age. height- 6 feet, weight- 71 kgs., asthenic built. he had visible mandibular prognathism. his vitals were stable. Systemic examination was unremarkable. On routine pre-operative investigation; haemoglobin-14g/dl, TLc-6550/cumm, platelets-2.3lac/cumm, s.creat-0.8mg/dl, liver functions-within normal limits.

his chest x-ray, however revealed: leftward shift of trachea and mediastinum. Hyperinflation of the right lung, few patchy shadows in left perihilar region (Fig. 1). Upper dorsal scoliosis with convexity to the right. The cardiac size was normal

In view of the history and present chest x-ray findings; an hrcT chest with pulmonary angiography was advised which revealed: right sided aortic arch with left pulmonary artery hypoplasia with evidence of small left hemithorax and ipsilateral shift of trachea and mediastinum suggestive of Swyer-Jame’s syndrome (Figs. 2, 3, 4).

Pulmonary function test was performed which revealed no abnormality.

Patient was taken up for bilateral sagittal split osteotomy with mandibular set back. Intra and post-operative period was uneventful. Patient was discharged on the 7th post operative day.

DiscussionOur patient as described above was asymptomatic on

presentation with normal physical findings. however, his past history coupled with an abnormal routine pre operative x-ray chest prompted us to further evaluate for various causes of unilateral hyperlucent lung. These would include congenital anomalies, intrinsic and extrinsic obstruction of one pulmonary artery, compensatory hyperinflation secondary to

*Associate Professor, **Professor, ***resident, Department of Neurology, PSG Institute of medical Sciences and research, Avinasi road, Pelamenu, coimbatore – 641 002. INDIAreceived : 27.10.2009; Accepted: 15.12.2009

Hyperostosis Frontalis Interna Mimicking Mount Fuji SignB Prakash*, MB Pranesh**, N Parimalam***, R Harish Kumar***

AbstractWe report an interesting case of ‘hyperostosis frontalis interna’ in a 73yrs old female whose mrI pictures mimics the cT appearance of ‘mount fuji sign’ in tension pneumocephalus a neurological emergency.

Seventy-three years old female, presented with vertigo of one year duration, which occurs intermittently, worsened by

standing, lasts for few days, recurring every month or so, and the last episode being one week prior to admission. Examination

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revealed thin built apparently healthy woman, having horizontal gaze evoked nystagmus to both sides and minimal imbalance while walking. No other defi cits made out. Other systems were normal. She was suspected to have vertebra basilar insuffi ciency. her urine routine examination, blood counts, sugar, renal parameters, calcium, phosphorous, alkaline phosphatase and electrolytes were within normal limits. The chest X-ray and EcG did not show any abnormalities.

mrI of the brain was done. It did not show any ischemia or infarct. No other parenchymal abnormalities were noted. On viewing the axial sections, the higher cuts appeared like having the ‘mount-Fuji sign’ (Fig. 1).

Fig 1: Higher cut axial section of MRI of the patient with ‘Hyperostosis frontalis interna’ resembling ‘Mount Fuji sign’

Fig 3: The Mount Fuji in Japan

Fig 4: Coronal and saggital sections of MRI of the patient showing ‘Hyperostosis Frontalis Interna’

‘mount-Fuji sign’ is an imaging observation in cases of tension pneumocephalus (Fig. 2). It occurs due to surgery or trauma and the air used to get trapped in bilateral frontal subdural spaces with a ball-valve mechanism.1 In anterior cranial fossa, the duramater is thin and closely applied to bone and the arachnoid is adherent to frontal lobes, hence the air is being trapped in the subdural space of the anterior cranial fossa.2 As the accumulation becomes more, it compresses the frontal lobes on both sides, making the CT of the brain resembling like the silhouett e of a volcano, such as mount Fuji.3 It leads to progressive clinical deterioration and may turn out to be a neurosurgical emergency, requiring burr-hole evacuation, sometimes closure of the dural defect.

mount Fuji is a perfectly symmetrical cone shaped volcano, which is the highest mountain (3776 meters) of Japan situated between Yamanashi and Shizuoka. It had last erupted in 1708 and is dormant afterwards, covered by snow nowadays (Fig. 3).

The coronal and saggital sections of the mrI brain (Fig. 4) revealed, no pneumocephalus and the pressure eff ects were seen only at the higher cuts. Both frontal lobes were pressed by symmetrical thickening of the frontal bones. This condition is described as ‘hyperostosis frontalis interna’, which is reported mostly in post menopausal elderly women.4 The etiology is unknown. If it is seen in combination with endocrine and neuro-psychiatric disturbances a diagnosis of morgagni or morgagni-Stewart-morel syndrome is made.5 In our patient the thickening is symmetrical and focal, but it can be diff use, involving up to parietal bone and may be sessile or nodular. It usually spares the superior saggital sinus and venous channels, and has no clinical signifi cance. The diff erential diagnoses include Paget’s disease, fi brous dysplasia and sclerotic metastasis. These were

Fig 2: A representative CT picture of classical ‘Mount Fuji Sign’ in a case of ‘Tension Pneumocephalus”

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excluded in our patient.‘mount-Fuji Sign’ is a classic clue for the diagnosis of the

neurosurgical emergency - the tension pneumocephalus. hyperostosis frontalis interna which is a benign condition of no clinical significance should be included as a differential diagnosis in mount Fuji appearance of brain imaging.

References1. Steven J. michel. The mount Fuji Sign. Radiology 2004;232:449-450.

2. Amit Agarwal, Brij raj Singh. mount Fuji sign with concavo-convex appearance of epidural haematoma in a patient with tension pneumocephalus. Radiology Case 2009;3:10-12.

3. Josef G. heckmann, Oliver Ganslandt. The mount Fuji Sign. N Engl J Med 2004;350:1881.

4. rosemary she, Juliana Szakacs. hyperostosis frontalis interna: case report and review of Literature. Annals of clinical & Laboratory Science 2004;34:206-208.

5. Smith S, hemphill rE. hyperostosis frontalis interna. J Neurol Neurosurg Psychiatry 1956;19:42-45.

1Assistant Professor, 2Professor and head, 3Junior resident, Department of medicine, PSGImS&r, Peelamedu coimbatore 641004, Tamil Nadureceived: 31.08.2009; revised: 22.12.2010; Accepted: 23.12.2010

Kikuchi-Fujimoto’s Disease : A Report of Five CasesV Mukta1, K Jayachandran2, S Hemapriya3

Abstract Kikuchi Fujimoto’s disease is a rare, self limiting disorder characterized by fever and cervical lymphadenitis. It is often an incidental finding in a patient suspected to have tuberculosis or lymphoproliferative disorder. We diagnosed this disease in a young 23 year old female who was suffering from prolonged fever, cervical lymphadenopathy, anemia, leucopenia and mild splenomegaly; based on histopathological study of the excised lymphnode. We reviewed the clinical records and histopathologic findings of four more patients of Kikuchi-Fujimoto’s disease, who were treated by others at our institute. All five patients improved with NSAIDS (non steroidal anti inflammatory drugs). histopathological finding of histiocytic necrotizing lymphadenitis or kikuchi’s disease proved invaluable in characterization of these cases.

Introduction

Kikuchi – Fujimoto’s disease is a self limited disease1, characterized by fever and cervical lymphadenitis .

Diagnosis is established by excision lymph node biopsy and histopathological study2,3. It is a benign disease of unknown etiology and symptoms resolve with NSAIDS. Nevertheless , it is a close differential diagnosis for tuberculosis and autoimmune disorders and is often wrongly treated with empirical antituberculous drugs. Follow up of these cases is a must, in order to detect any autoimmune connective tissue disorder which may develop at a later stage4,5 . We present here a report of five cases from a teaching hospital in Coimbatore, Tamilnadu.

Case ReportsCase 1

A 23 year old female from kerela presented with fever of 21/2 months duration. She complained of weight loss and poor appetite. She had been treated ouside with empirical antituberculous drugs for 1 month. On examination and investigations, she was found to have severe microcytic hypochromic anemia, leucopenia; mild splenomegaly and bilateral posterior cervical ,enlarged, nontender, nonmatted lymph nodes ; largest measuring 2x2cm in size. ANA and hIV serology was negative . Excision biopsy of the lymph node was suggestive of kikuchi’s disease. She improved with nonsteroidal anti-inflammatory drugs (NSAIDS). Blood transfusion and hematinics were given.Case 2

A 20 year old female from coimbatore presented with fever for 2 days and neck mass on the right side. Past history of pulmonary tuberculosis was present. Examination and

investigations revealed right sided cervical lymphadenopathy 4x3 cm in size, nontender , nonmatted. Mantoux was negative. ANA , dsDNA and hIV serology was negative . Excision biopsy of lymphnode was suggestive of kikuchi’s disease. She improved with NSAIDS.Case 3

A 30 year old female from Tiruppur was admitted with history of fever of 1 month duration. Examination and investigations revealed enlarged right sided deep cervical lymph node , splenomegaly and microcytic hypochromic anemia. mantoux was 10 mm in size. hIV serology was negative . Excision biopsy of lymph node was suggestive of kikuchi’s disease. She was treated with empirical antituberculous drugs and NSAIDS . Patient was lost to follow up.Case 4

A 23 year old male from Erode was admitted with high grade fever for 10 days. Examination and investigations revealed enlarged right upper deep cervical and posterior auricular lymph nodes , elevated ESr, LDh , liver enzymes; ANA serology was not done. hIV serology was negative. Excision biopsy of lymph node was suggestive of kikuchi’s disease. he was started on empirical antituberculous drugs and NSAIDS. Antituberculous drugs were stopped once biopsy report was obtained. Patient was asymptomatic on follow up.Case 5

A 20 year old male presented with fever for 10 days and left sided neck swelling of 2 weeks duration. Examination and investigations revealed 3x3 cm posterior cervical lymphadenopathy. ANA serology was not done. hIV serology was negative . Excision biopsy of lymph node was suggestive of kikuchi’s disease . he improved with NSAIDS.

Lymph node biopsy in all the five cases, showed areas of necrosis, karryorhexis, histiocytes , plasmacytoid monocytes with complete absence of neutrophils and granulomas on histopathological examination. The lymphnode specimen stained