Case: A boy of 4 yrs with Langerhans Cell Histocytosis

Mushfiq, a boy of 4 yrs presented with fever for 7 days, gradual pallor for 2 months, and multiple swelling over the scalp for 1 month.

On admission, he was febrile, toxic and pale. He was undernourished, his pulse was 110/m,BP -105/65 mmHg,respiration 25/m, temp-103 degree F, anaemia +++, jaundice-absent,no bleeding tendency, no lymphadenopathy, no bony tenderness and no hepatosplenomegaly.

The swelling over the skull are multiple,firm, non tender and fixed with the underlying structure.

on investigation, Hb-4.5 gm/dl, RBC-2.06m/cmm, MCV-85.4 fl, MCH 21.8 pg, MCHC 25.6 g/dl, WBC-7500/cmm, Neutrophil-60%,Lymphocytes -32%,Monocytes-04%,Eosinophil-04%. PBF shows anisosytosis and anisocromia with roulex formation, WBC mature with a few myelocytes, platelet suggestive of chronic disorder/infection.



X-ray of the skull shows focal scalp swelling in frontal and parietal region, pepper-potlucencies in the vault and subtle erosion on the outer tables of vault underneath the swelling and sutural diastasis suggestive of Langerhans cell histocytosis with raised intracranial pressure.

FNAC from the scalp growth shows hypercellualr material containing small round to oval cells having lobulated and folded nuclear with opened up chromatin;these cells are arranged diffusely and the cells show mild cellular atypia; the backgrond shows necrotic tissue debris suggestive of 'small round cell tumour' where Langerhans cell histocytosis may be considered.

CT scan of the brain shows extensive intracranial hyperdense massesshowing distribution along dura and frontal parenchyma. No mass effect or midline shift is present; there are multiple scalp swelling , some of them show underlying outer table erosion; there is splitting of sagital and coronal suture. The impression is of Langerhans cell histocytosis.

The patient was refered to special cancer center for further management.


Dr. Badrud Doza
Dr. Harun

Link:
Langerhan's Cell Histocytosis- Boston Children Hospital
Langerhan's Cell Histocytosis-Medscape.com

Case: Ewing's Sarcoma with metastsis to the lung in a boy of 7 yrs

Child Abu Bakhr, 7 yrs male child was admitted into our hospital on 24/10/08 with the complaints of cough for 15 days , low gradw fever for the same period and respiratory distress for last 4 days and swollen edematous right leg for 3 months.

On admission, the child was dyspnic , ill-, pulse -100/m, Respiratory rate-30/m, Temparature -99 d f . on auscultation, pt had bilataeral crepitations all over the lung.

On local examination, skin was erythematous,swollen and tender without any discharging sinus or pus point- the child was a previously diagnosed case of Ewing’s Cell Carcinoma of the tibia and histopathological report was suggestive.

The Child received one cycle of chemotherapy and Radiotherpy 3 months back. On admission, CXR was done which showed wooly ball appearance.

The patient is lebelled as Ewing's Sarcoma with Metastasis to the Lung.


badrud doza
fahim

Link:
Ewing's Sarcoma- Wikipedia

Case: Tubereculoma of the brain

The patient, a boy of 3 years presened with low grade fever for 2 months , neck bending for a week and convulsion for a few times in the last 48 hours.
There was history of contact with the open case of tuberculosis.

On examination, the patient was drowsy, malnourished, neck rigidity and kernig's sign are positive, reflexes are exxagerated.

CSF studies show mild lymphocytosis, sligtly increased protein and decreased noraml sugar.Gm stain and AFB staiin were negative.







The CT scan of the patient reveals multiple small cercular shadows of the brain , the radiologist identified them as Tuberculoma.

The patient was lebelled as a case of Tuberculoma of the brain.

He was given 4 drug antitubercular regimen and gradually improved and dischsrged after 1 month staying in the hospital.

badrud doza

Link:
Tuberculoma of the brain by Bhaskara Reddy & V. Kameswararao
An interesting account on Tuberculoma of the brain by Maurry H. Cambell MD writhhen in 1945

Case: a girl of 4 months with osteogenesis imperfecta

The girl, 4 months of age admitted in our hospital for Pneumonia. But her history reveals # of the rt upper arm at the age of 7 days for which she was taken admission in the other hospital of the city. They made multiple x-rays and found # in other sites also.




She was labelled as a case of Osteogenesis imperfecta and advised the parents to take precaution to handle the baby and provide necessary counselling.



badrud doza

Link:

Osteogenesis Inperfecta Foundation

Case: Neuroblastoma with severe anaemia

Atunu Acharjee, 1 yr 4 months from Chittagong admitted in our hosspital for severe anemia. On examination , a huge mass on the left side of the abdomen, firm, non tender with no notch likely kidney and multiple small masses all over the abdomen were found . Liver was also enlarged 6 cm from the costal margin, firm and non tender.

On evaluation of history, it was found that the patient was consulted in Kolkata 2 months back and CT scan there suggest abdominal neuroblastoma, diffuse tumour infiltration of the left kidny and encasement of large vessels like aorta by the tumor mass.

Laparotomy was done in a hospital there and excised leftsided mass including part of the left kidney.The histopathology suggests Neuroblastoma. Patient was discharged on palliative treaatment from there.

In our ward, we have given the patient blood transfusion and refered the patient to oncologist.


Dr. Badrud Doza
Dr. Abu Sayeed

Link:

Neuroblastoma in Children in Cancerbackup/Uk
Neuroblastoma in NIH

Case: a boy of 11 months with scleroderma

Minhaj, aged 11 months, Male baby was lying stiff on the bed. On first look , I first mistook it as a case of cerebral palsy –spastic quadriplegia.

But his birth history is normal and the spasticity is of 7 months duration and gradually progressing. Both the extremities are markedly affected and the facial muscles are also affected and it could not cry with his full mouth open.

His skin is thick like sclerema but in almost all the body and he has no sign of infection.
.
Clinically we suspect the case as “Scleroderma’.

We arranged skin biopsy for him. The sample was taken the thigh. The report narrates increased fibrosis and moderate numbers of chronic inflammatory cells around the periappendigeal region in the dermis. The subcutaneous fat is partly replaced by collagen. The overlying epidermis shows no significant changes and concludes that the features are compatible with scleroderma.

Dr. Badrud Doza
Dr. Fahim

Link:
Screloderma Foudation

Case: Solid mass in the left Iliac fossa with acute abdomen

The patient was seen at my chamber in practice. The girl of 11 years presented with severe abdominal pain and intermittent fever of two days duration.
Menustartion yet not started.

On examination, moderate tenderness was present in the left iliac fossa but no mass could be palpated. The patient was admitted on emergency and given conservative treatment for Acute Abdomen. Later, on evaluation a solid mass of 5cmX 4 cm was found in the left Iliac fossa on ultrasonography.
The patient was improved on conservative treatment of antibiotic, fluid, antipyretic and analgesic.
The follow up X-ray also showed a solid mass pushed the uterus to upward and to the right.
Laparotomy was done. The mass was found to be of ovarian origin and brown in color.
The change of color and solidity of the mass is likely due to haemorrhage into the overy due to torsion of the ligament of the overy.