May 29, 2015

Klippel-Feil syndrome

Introduction:
Klipple-Feil Syndrome is a congenital malformation.It is first described by Maurice Klippel and Andre Feil in 1912.68% found in females. It may be because of problem in the early neural tube development.          

Features

1.short webbed neck
2,fused cervical vertebrae
3. secondary torticollis
4. facial asymmetry
5.low hairline

Frequency:

1 in 42000 live births

Types:

Type I - Single-level fusion
Type II - Multiple, noncontinuous fused segments
Type III - Multiple, contiguous fused segments

Associated anomalies:

Cleft palate
deafness
ventricular septal defect
rib defects
fetal alcohol syndrome
syringomyelia
scoliosis
cervical meningomyelocele
Sprengel anomaly

Presentation:

short neck
Decreased neck movements (ROM)
Facial asymmetry
toticollis

Investigations: 

Lateral flexion-extension radiographs of the cervical spine
CT scan of cervical spine/whole spine
Ultrasonography for renal anomalies

Management:

Hyper-mobility of upper cervical spine are at risk of developing neurologic development, While hyper-mobility of lower cervical spine are exposed to early degenerative disease and its consequences.
Syptomatic spinal fusion my require decompression








Feb 4, 2013

Hepatoblastoma

Introduction:
The liver is a large, very important organ situated in the right upper quadrant of abdomen. The normal functions of the liver in the body are: 


To help store nutrients from food
To break down and remove harmful chemicals from the body
To build chemicals that the body needs to stay healthy.


Hepatoblastoma: The most common type of liver cancer in children is Hepatoblastoma. it occurs most frequently in infants or very young children between 2 months and 2 year.

Symptoms:
  • Lump in the abdomen (most common)
  • Reduced appetite
  • lethargy
  • nausea / vomiting
  • Pain in abdomen
  • Jaundice
Investigations:The following minimal investigations are proposed to diagnose hepatoblastoma

  • Tumor markers- serum AFP
  • CT scan of abdomen with Ct guided FNAC or Biopsy
  • X-ray ches/CT Chest
  • Complete blood count
  • Biochem profile
Treatment: 

Surgery & chemotherapy are two treatment options

Surgery: Liver resection according to the location & extent of the tumor. It will be either
  • Rt hepatectomy
  • Lt hepatectomy
  • Extended Rt or Lt hepatectomy
  • Bi or trisegmentectomy
If liver resection is not possible because of multicentric disease then liver transplant is the only treatment of option.

Chemotherapy:
Chemotherapy is given before or after surgery. Surgery is done either as a sandwich surgery (in between cycles of chemotherapy) or it is done at the start & then chemotherapy is given.


The drugs used in chemotherapy are
  • Cisplatin
  • Doxorubicin
  • Vincristine
  • 5-Fluorouracil

Jan 31, 2013

Ksharsutra for fistula in ano

Ksharsutra is barbouslinen No.20 coated with latex of ‘EUPHORBIA NERIFOLYA’ ash of
‘ACHYRANTHUS ASPERA’and dried powder of ‘TURMERIC’.

It has antibacterial, antifungal, anti-inflammatory, antioxidant, anticoagulant properties.

How it is used-

Under general anesthesia fistula site is probed

Tract delineated by methylene blue and hydrogen peroxide

Internal opening is identified

Ksharsutra passed through the probe

Knot tied and kept

Re application of Ksharsutra is done after 10 days

Ksharsutra cut through the fistula over a span of 3 weeks

Wound healed well with secondary intention

Ksharsutra performs chemical cauterization

Complications are less

It can avoid incontinence, stricture and bleeding

High cure rate

Early ambulation and early discharge

Average cutting time 0.78 to 1 cms/week

Jan 28, 2013

Palatal abscesses


Hard palate is the partition between the nose and floor of the mouth. Embryologically, hard palate is formed by the palatine process of maxilla and the horizontal plate of the palatine bone. Anterior portion of the hard palate contains irregular ridges which help during feeding. Hard palate continues posteriorly in a soft portion called soft palate.

Abscesses of hard palate are known. The etiology may be trauma, ulceration, secondarily infected nasopalatine cyst or infected tooth. The line of treatment will be antibiotics and anti-inflammatory followed by incision and drainage.

Dec 20, 2012

Vesicoureteral reflux (VUR)

Introduction:

Vesicoureteral reflux (VUR) represents one of the most significant risk factors for acute pyelonephritis in children

Spontaneous resolution of VUR occurs in 78–90% of grades I–III VUR that is diagnosed during evaluation of antenatal hydronephrosis.

Grades IV and V may spontaneously resolve but usually require surgical intervention


Classification of vesicoureteral reflux (VUR)

Grade I- Reflux of urine in non dilated ureter
Grade II- Reflux of urine into a non dilated pelvis
Grade III- Reflux of urine into a dilated ureter up to the renal pelvis with possible blunting of calyceal fornices.
Grade IV- Reflux of urine into a grossly dilated ureter with moderate blunting of the calyces.
Grade V- Reflux of urine into a massively dilated and tortuous ureter with loss of the papillary impressio.
  
Management:

The objectives in the current treatment
the prevention of episodes of acute pyelonephritis with its associated morbidity and mortality.

to prevent the scarring of the kidney associated with vesicoureteral reflux (reflux nephropathy), which increases the risk of hypertension and renal failure.

Indications for surgical treatment

1.Breakthrough febrile UTIs despite adequate antibiotic prophylaxis
2. Severe reflux (grade V or bilateral grade IV) that is unlikely to spontaneously resolve,especially if renal scarring is present
3.Poor compliance with medications or surveillance programs
4.Poor renal growth or function or appearance of new scars. 



Surgical management of VUR : Ureteric Reimplantation:

Principle-Reconstruction of the ureterovesical junction (UVJ) to create a lengthened submucosal tunnel for the ureter,which functions as a one-way valve as the bladder fills

Types of Surgical repair:


Cohen procedure
Politano-Leadbetter
Lich-Gregoir,
Psoas hitch techni
que
 

Dec 18, 2012

Surgical management of Ureteropelvic junction (UPJ) obstruction:

The goals in treating patients with ureteropelvic junction (UPJ) obstruction are to improve renal drainage and to maintain or improve renal function.

Surgical Treatment:

Endopyelotomy,
Open pyeloplasty
Laparoscopic pyeloplasty
Robotic-assisted laparoscopic pyeloplasty.

The principles of surgical repair:

Formation of a funnel
Dependent drainage
Watertight anastomosis
Tension-free anastom

Types of pyeloplasty

Anderson-Hynes dismembered pyeloplasty
 Foley Y-V plasty
Culp and DeWeerd
Scardino and Prince

Endopyelotomy

1. The stricture should be short (< 1.5 cm), and no crossing vessels should be defined on imaging
2. An endopyelotomy incision is performed through the area of obstruction with a laser, electrocautery, or endoscopic scalpel.
3. This is followed by prolonged ureteral stenting, for a period of 4-8 weeks. 
4. When open pyeloplasty fails, endopyelotomy is particularly useful

Laparoscopic pyeloplasty

This procedure is replacing open pyeloplasty as the criterion standard with 95% success rates.Significant learning curve associated with laparoscopic suturing

Complications:

Complications from open surgical pyeloplasty include UTI and pyelonephritis,urinary extravasation and leakage, recurrent ureteropelvic junction (UPJ) obstruction, or stricture formation.

Oct 31, 2012

Intestinal malrotation in children

Types of Intestinal Malrotation


Nonrotation: Bowel returns to coelom.It fails to rotate.Result will be Small bowel to the right & Colon to the left

 

Reversed rotation:It is rare anomaly.There is 90 clockwise rotation around SMA, as a result of which Cecum & transverse colon comes dorsal to SMA while duodenum anterior to SMA

 

Incomplete rotation: There is premature arrest of normal rotation so that Ladd’s bands forms causing duodenal obstruction.Base of the mesentery is narrow  which can cause to clockwise rotation.


Clinical features: 

The newborn presented with bilious vomiting, haematemesis & haematochezia.


In older children, Either they wll be asymptomatic or intermittent abdominal complaints or acute obstructive symptoms &
  signs of impending abdominal catastrophe.

Diagnosis: 

A.X-ray abdomen in erect position- 1.Paucity of gas in the X-ray abdomen 2. Only gastric air-fluid level seen or there will double bubble appearance.

 

B. Upper GI studies:1.Right-sided DJ junction 2. proximal jejunal loops 3. Dilated stomach s/o incomplete obstruction of the duodenum.
4. Midgut volvulus - Corkscrew appearance.

 

C.Ultrasound doppler: There will be either vertical orientation or left-right inversion of SMA & SMV.

 

D.CT Scan of the abdomen: 1.Alteration of SMA & SMV orientation. 2. DJ & small bowel loops seen on right side of the spine. 3. large bowel is seen on left side of the abdomen. 4. there will be signs s/o duodenal obstruction.


Management: Ladd's procedure.Step 1.- lysis of ladd's bands & many other adhesivebands. 2.Mesenteric base widening 3. straightening of duodenum. 4. appendicectomy 5. Placing small bowel on right side of abdomen while large bowel on the left side of the abdomen.