Pineocytoma with facial palsy, hemiplegia and hydrocephalus

A boy of one and half years develops Lt sided facial palsy 6 weeks back and gradually developed Lt sided hemiplegia and hydrocephalus. He had no vomiting or convulsion. No history of meningitis or head trauma.

On examination, his facial palsy and the hemiplegia was of upper motor neurone type. There was no bladder or bowel involvement and no sign of meningitis
opthalmoscopic examination shows bilateral papilloedema

On investigation, Hb-9.5 gm/dl, ESR-25mm in 1st hour, TC, DC normal. US shows moderate hydrocephalus. CT scan shows a isodense mass in the pineal region, posterolateral to the third ventricle on the right side,also encroching the thalamsus. Moderate ventriculomegaly due to compression on superior aqueduct

The CT scan impression is likely Pineocytoma .

MRI is requested for further evaluation.

Systemic onset JRA with multiple Arthritis, severe pain and prolong fever

An 11 year boy admitted in our hospital with fever for one and half months, severe pain and swelling of multiple joints. He has no recent history of sore throat, no family history of RA, no features of Psoriasis, no h/o contact with tuberculous patient, no h/o hear disease.

On examination, subcutaneous nodules over bony prominences, multiple, discrete, non tender, lymph nodes at different sites were found. There were no hepatosplenomagaly, no murmer, no bony tenderness and no bleeding manifestations .

He has anaemia, leucocytosis with neutropenia, high ESR. Urine analysis, Platelet, PBF, X ray of the chest and ECG were normal. Xray of the right knee joint shows soft tissue swelling, blood culture negative. ASO titre was 400 IU ( may be a coincidental finding of past streptococcal infection). RA factor abd ANF were absent.

Possibility of tuberculosis, leaukaemia, bacterial endocarditis were excluded.

The patient was level as Systemic onset JRA.

The patient was given tab Ibuprofen and 2 days later as the patient was not improving oral prednisolone was added.

One week after the admission the patient is now settled.


Bdoza

Female pseudohermaprodite with hypospadiasis and ramnant of ovary

The parents of a child, apparently male of 6 weeks of age seek the advice of a physician for hypospadiasis. On examination the scrotum was found empty, no testes in the inguinal canal, the penis is small with urethral opening in the under surface.

Ultrasonography of the Abdomen reveals a cystic structure behind the urinary bladder.

Laparoscopic exploration of the abdomen was carried out by our surgical collegue. No testes was identified in the abdomen and a tissue from the cystic lesion was taken which on histological examination reveals to be uterine pole of the right ovary.

The patient is awaiting for reconstructive surgery and the external feature would be changed to female type.


Bdoza

Guillain- Barre Syndrome with Ascending paralysis and Hypertension

A boy of 4 years admitted in our ward with gradual weakness of lower limbs started 5 days back with inability to stand and walk. The weakness gradually involves the upper limbs and the patient become unable to move or hold anything by his hands.

He had a history of short viral episode 7 days back
Patient is conscious but irritable, had tachycardia (pulse 130/m) and Hypertension (BP 130/85 mmHg) but the patient had no bladder or bowel involvement.

Muscle Tone and Muscle Power of the limbs were decreased. The reflexes of the limbs were absent or diminished. Sensory minimally affected.

The patient was level as Gullaine-Barre Syndrome with Ascending Paralysis and hypertension.

CBC is normal, CSF study on the 8th day showed Lymhocytes 30/cmm, Protein 145 mg/dl.

The patient was given Inj Methyl Prednisolone for 5 days. The IVIg was not available in the local market. For hypertension no specific drug was given, it came to normal on 3rd came down to normal on 3rd hospital day.

The patient now is slowly improving, his power of the upper limbs is much regained but the lower limbs is still weak and he is not able to stand or walk.

The patient is still in our ward and under our care.


Bdoza

Links : GBS-information page of National Institute of Neurological Disorders & Stroke

GBS Foundation International

Enteric fever in a child of immigrant parents to USA

A boy of 8 years, a child of immigrant parents to USA, who recently came to visit Bangladesh presented with fever, abdominal pain and vomiting.
He had no definite tenderness in the abdomen and no other abnormal finding. Other members of the family had viral fever in the recent past.

Initially the child was thought to be a case of viral fever. But as the fever was not subsiding as that of others, investigations were done. Widal test showed strongly positive (TO 1:320), in ICT for Salmonella IgM was positive but not the IgG, MP in blood film and in ICT was negative.

The Patient was treated with Injection Ceftriaxone and supplemented by oral Azithromycin Response was slow. Initially vomiting stppped, then the abdominal pain, fever took 6 days to come to the base line after introduction of Ceftraixone. He had fever for total 11 days

We continue Ceftriaxone for 4 afebrile days and Azithromycin for 5 afebrile days.


Bdoza

Congenital leukaemia

A boy of 14 days admitted in the Neonatal ward of our hospital with fever, respiratory distress, rash and abdominal distention.

On close examinatin, the rashes were found to be purpura and ecchymosis; the lung has bilateral crepitations, the abdomen is distended for hepatospenomegaly.

On clinical imprestion it was first thought to be a case of Neonatal Sepsis. The next possibility is Torch Infection.

But the blood count reveals Hb-5.3 gm/dl, TC of WBC -1,600,000 /cmm, 90 of them are blast cells, peripheral blood film study reveals them as lymphoblasts.

So the diagnosis was lebelled as Congenital Acute Lymphobalstic Leukaemia.


Bdoza

Prader-Willi syndrome with epilepsy with acute metabolic derangements

A girl of 7 and half years presented in gasping with obesity, uncounciousness with a history of gastroenteritis. She was on Sodium Valproic Acid for the last 5 years for siezure disorder. Her CT Scan at 2 and half year showed cerebral atropy due to hypoxic ischaemic encephalopathy.

She was extremely obese and short statured, had developmental delay and mental retardation. But her facial dysmorphismis not marked and she had no polydyctaly.

Labratoty finding shows sodium-187 meq/L, potassium-2.3 meq/L, cl, HCO,glucose and Ca normal.

The patient was lebelled as Prader-Willi Syndrome with epilepsy and acute metabolic derangement.

The patient was intubated, fluid was appropriated, dopamine and dolbutamine was given, antibiotic and anticonvulsive were added

Bdoza

Congenital Teratoma

Congenital Teratoma

A boy of 3 days, home delivered presented with massive abdominal distension. Otherwise the patient had no sign of sepsis, hemorrhage or toxicity. Bowel and bladder moved normally.

CBC, CRP, urine for R/E, Liver and Renal functions were found normal. US shows solid tumor suspecting teratoma.
The patient was shifted from Neonatal ward to the Paediatric Surgery.
Laparatomy was done, a solid tumor adherent with the posterior wall of the stomach was found.
The tumor was resected, stomach repaired and anastomosis done.

The excised tissue was sent for histological and was reported as Teratoma containing elements of connective tissue and neural tissue.

Bdoza