Pierre Robin Syndrome with Cleft Palate, Aspiration Pneumonia and Partial Intestinal Obstruction

In infant of 4 months presented with cough, respiratoy distress, abdominal distension and constipation.

He was deliverd normally. Then in the first week he developed Pneumonia for which he was admitted in the hospital. He had occassional asphyxia and advised to be kept in prone position.

On examination, he was ill-looking and dysponeac and found to have micrognathia, high arched palate and posterior cleft palate. His growth was compromised. Abdomen was distended and hypertympanic. He had no neurological sign.

P/R examination was normal.

For the abdominal distenstion and constipation, initially Hirschsprug was suspected. But as the suction biopsy was found normal and abdominal distension was increasing our surgical collegue did the laparotomy and found a fibrous band extended from the meckl’e diverticulum to the mesentry.

The band was released and the distension gradually decreased.

The patient was finally lebelled as Pierre Robin Syndrome with Cleft Palate, Aspiration Pnemonia and Partial Intestinal Obstruction due to fibrous band.

The patient was recovered with antibiotic, surgery and other supportive measures and discharged.

A case of Septic Arthritis and Spontaneous Pneumothorax

A boy of 2 year 4 months presented with respiratory distress of 1 day, fever of high grade for 7 days and swelling of left shoulder and arm of 8 days

On examination, he was found in dyspnoea, left side of the chest full with restrictricted movement,vocul fremitus absent with shifting of the trachea and apex beat and breath sound and vocal resonance absent.

His left shoulder was found swelled, hot and tender which extended down to the arm.

His X-ray of the shows Pneumothorax of the Lt side with collaps of the lungs and shifting of the mediastinum. Xray of the left arm shows no bony pathology.

The case was labeled as Septic arthritis of the left shoulder and Spontaneous with Pheumothorax of both lungs.

Water seal drainage of the chest was given immediately after admission and later when the patient settled the pus was drained out from the left shoulder by giving incision. Inj Ceftriaxone and Inj flucloxacillin were given as antibiotics.

Interestingly the patient developed later pneumothorax of the rt side of the chest which was found on check x-rays.




Temporary relieve of the tension in the Rt chest was also required. Now the Patient is recovering and we hope to discharge the patient within few days.



Our surgical and orthopedic colleagues helped us in the management of the patient.

Bdoza

A case of Biliary Atresia

A boy of 3 months presented with persistent jaundice from 3 days o life, pale color stool , high color urine and occasional vomiting.

On examination he is found nutritionally compromised, moderately jaundiced, has hepatosplenomegaly but no ascites.

On investigation, Serum bilirubin- was 8.8 mg/dl( direct -3.6mg/dl, indirect- 5.2 mg/dl)
SGPT (ALT)-105 U/L, Serum Alkaline phosphatase- 970 U/L, prothrombin time -23 sec against control of 15 sec.

USG shows non visualized gall bladder and common bile duct

HIDA scan shows radiotracer concentration in the liver in early images. No radiotracer concentration is seen in the gall bladder region and in the intestine even after 2 and ½ hours .

The case is diagnosed as Biliary Atresia

Our surgical colleagues were consulted for surgical management of the patient.


Bdoza

Dengue Haemorrhagic Fever -grade II

A boy of 3 years coming from endemic area of Dengue presented with 5 days fever, vomiting for several times, gum bleeding and bleeding per rectum He has no h/o bleeding in the past.

On exam the found irritable, mildly anemic, not jaundiced or cyanosed. There is no lymphadenopathy, no hepatosplenomegaly, no bony tenderness. His has tachycardia but blood pressure is normal.

He develops no ascites or pleural effusion.

On investigation, he has thrombocytopenia( 60,000) but PCV -normal (36%)
His IgG and IgM for Dengue were positive.

The patient is lebeled as Dengue Haemeorhagic Fever -grade II.

He is kept under mosquito net, given infusion/ORS, antipyretic, normal diet and kept under close monitoring.


Badrud Doza

Hepatic Encephalopathy with Hepatic Failure and Hepatorenal Syndrome

A boy of 2 years and 3 months presented with deep jaundice, in semiconscious condition and with bleeding per rectum.

When asked mother said that he developed jaundice I month back from which he gradually recovered. Then he was given antihelmenthic 3 days back after which his condition deteriorates
On examination, he was found to be deeply jaundiced with oedema and ascites.
On admission pulse was 126/m, BP 90/60 mmHg, temperature 102.5, respiratory rate 50/m, liver was not palpable.

On investigation, his Hb was 11.5 gm/dl(on admission), TLC-15200/cmm, N-78%, L-12%, bilirubin 28.8 mg/dl, direct 12.7, indirect-16.1, SALT 342 U/L , Alkaline phosphatase 487U/L , HBsAg negative, Prothrombin time 60 sec with control 22 sec, ratio 4.28, Index 23.3%, INR 5.56 Serum Albumin 3.4gm/dl. Random blood sugar 42.8 mg/dl, Serum creatinine .6 mg/dl, serum electrolytes-normal.

Patient was progressively deteriorating, develops convusions and anuria

Patient is labeled as Hepatic Encephalopathy with Hepatic failure and Hepatorenal Syndrome

The patient is kept on parental fluid, oxygen, antibiotics, lactoluse, konakion, pulv streptomycin, Inj Mannitol and blood , domamine etc.

The patient is fighting for his life.

[Note: When the liver in hepatitis patient regresses in size, it indicates grave sign and poor prognosis
Recovery from hepatorenal syndrome is also difficult
When Hepatic Encephalopathy is grade IV, mortality is also high]


Badrud Doza

A case of Rheumatic Chorea

A boy of 8 years presented in outdoor setting with choreotic movements of the limbs of the right side and slurring of the speech for the last one and half months.
He has no h/o sore throat, no joint pain or chest pain.

On examination, he was found alert but emotionally labile and afebrile.

Muscle tone and power of the limb on the right side decreasedBut reflexes normal and gait were found normal
Milkman’s grip- positive, serpentine tounge- absent, supination pronation test normal.


On Investigation- hw was mildly anaemic, ESR slightly raised , TLC & DLC normal, ASo titre 200 iu/ml, Xray, ECG normal, CT –was also found normal .

Patient was provisionally diagnosed as Rheuamtic Chorea.

The patient was treated with Injection pecilicillin, Tab prednisolone, Tab peridol,Tab parkinil, then Tab clobam

The patient responded to treatment and his symptoms alleviated. Patient was on close follow up.

The posibilities of intracranial tumor was excluded on first examination ansl on follow up and recovery of the patient on anti Reumatic treatment and his wellbeing at one year aftyer diagnosis assures the first clinical conclusion.

Diagnosis of Reumatic Chorea is more clinical.

[Footnote: Rheumatic Chorea does not follow the Jones criteria for dianosis.
Rheumatic Chorea as a single manisfestation may indicate Rheumatic fever.
ESR may be low as the inflammatory changes in the brain is minimal.
The clinical manifestation may occur months or years after the initil sterptococcal infection]

A case of Bell's Palsy

A boy of 8 years presented with facial paralysis of 5 day duration in the outdoor setup. He had a history of febrile episode 5 days back. Initially he had mild pain, no history of trauma, no features of CNS involvment.

On examination, he was found to be alert, conscious, non fibrile.

His facilal palsy was found to be of right side and lower motor in type. Other CNS findings are normal.

He was lebelled as a case of Rt sided Bell's Palsy.

Badrud Doza