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 technique  

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.

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.

Liver injury grading system

The AAST Liver injury grading system is as follows

• grade I : 
  • haematoma : sub capsular, < 10% surface area 
  • laceration : capsular tear, < 1cm depth
• grade II : 
  • haematoma: sub capsular, 10 - 50% surface area 
  • haematoma : intraparenchymal < 10cm diameter 
  • laceration: capsular tear, 1 - 3cm depth, < 10cm length
• grade III : 
  • haematoma : sub capsular, > 50% surface area, or ruptured with active bleeding
  • haematoma : intraparenchymal > 10 cm diameter
  • laceration : capsular tear, > 3 cm depth
• grade IV :  
  • haematoma : ruptured intraparenchymal with active bleeding
  • laceration : parenchymal distruption involving 25 - 75% hepatic lobes or
  • 1 - 3 segments (within one lobe)
• grade V : 
  • laceration : parenchymal distruption involving >75% helpatic lobe or
  • > 3 segments (within one lobe)
  • vascular : juxtahepatic venous injuries (IVC, major hepatic vein)
• grade VI : vascular : hepatic avulsion

Umbilical hernia in children

   Introduction: Umbilical hernias in children result from a weakness in the abdominal wall that is present at birth. 


Clinical Presentation: Generally, Parents notice a bulge at the umbilicus,which increases in size as the child cries, strains, coughs.It reduces spontaneously when the child goes to sleep or clam down.

Investigations: Its clinical diagnosis & does not require any further test if there are no other signs.


Treatment: 1. Reassurance- If the child is less than 2 years, small defect & no h/o irreducibility then just reassurance is needed (90% of umbilical hernia resolute spontaneously)

2. Indications for surgical repair-1. Age is more than 2 years 2. The defect is more than 1.5 cm.3.If there is h/o irreducibility or obstruction.

Sacrococcygeal Teratoma

Sacrococcygeal teratoma is the tumor arising in sacrococcygeal region.It is the commonest tumor found in newborns. It is also seen in infants, children & very rarely in adults. The SCT is more common in girls than boys with ratio of 3:1. The routine use of prenatal ultrasound has made the diagnosis early during fetal life.

Symptoms:
1. Sacral mass
2. Mass in the abdomen & perineum
3. Distension of abdomen
4. Displacement of anus due to sacral mass
5. Constipation
6. Sacral sinus.

Classification: Altaman's classification
Type 1- Entirely outside
Type 2- Mostly outside
Type 3-Mostly inside
Type 4- Entirely inside

Diagnosis:
1. Prenatal Ultrasound- Solid/ cystic mass occupying abdomen as well as perineum
2. CT Scan abdomino-pelvic region/ MRI abdomino-pelvic region
3. Tumor markers- AFP or Alfafetoproteins

Treatment:
1. Surgical excision in benign or mature teratoma
2. Associated with chemotherapy in malignant or immature teratoma

Chemotherapy:
Bleomycin, Etoposide & Cisplatin (BEP) protocol is the commonest first line protocol used.

Prognosis- Good if complete surgical excision is done along with removal of coccyx.

Germ Cell Tumor

Introduction:
Germ Cell Tumors are the neoplasm arising from primordial germ cells which produces specialized cells in the body like sperm and egg cells. With the recent advances in cisplatin based chemotherapy, the cure rate of germ cell tumors is increased if diagnosed in early stages. It frequently occurs in three modal peaks of life, infancy, 25-40 yrs and around 60 yrs.

Sites:
Testes
Ovaries
Paratesticular area
Abdomen (retroperitoneum)
Mediastinum
Brain

Types: 
Malignant GCTs: The tumors such as yolk sac tumors, choriocarcinoma, and immature tearatomas encompass this type. The elevated tumor markers, rapid growth signifies malignant transformation.

Benign GCTs: Teratomas are benign tumors. They have characteristic appearance where there are teeth, bone, hair is found inside the tumor.

Symptoms:
Painless scrotal mass
Abdominal mass
Abdominal pain
Breathlessness
Sacral mass
Mediastinal mass

Investigations:
Tumor markers like AFP, β-HCG, LDH
Biopsy
CT Scan/ MRI

Treatment: 
Surgery: The location of the tumor may influence the need for surgery. When possible, the first choice is usually to try and remove the entire tumor. This can be enough to cure most teratomas and immature teratomas. The coccyx needs to be removed in case of sacrococcygeal teratomas.

Chemotherapy: BEP (Bleomycin, Etoposide & Cisplatin) is the first line chemotherapy for the malignant germ cell tumors. This tumors are very much chemosensitive & the fall in tumor marker gives the idea about the response. If the complete resection is not possible initially then neoadjuvant chemotherapy is advisable.

Radiotherapy: It is indicated as a local therapy when the surgery is not possible.

Prognosis:
The 5-year survival rate is about 95% in germ cell tumors.

Phimosis

Phimosis is narrowing of prepucial opening. In infancy, prepucial skin is adherent to the glans.Over the period it gets separated. This is physiological phimosis. Pathological phimosis generally occurs in older children because of repeated infection also called as balanoposthitis.

The child will present with urinary complaints like crying during micturition, redness at glans/meatus, ballooning of prepuceal skin while micturating, repeated urinary tract infection, failure to thrive.

Circumcision is the procedure of choice where the excess prepucial skin is cut under local/general anesthesia.In some religions like muslim, it is done as a ritual custom.

It is day care procedure & within 8-10 days child recovers & wound dries up. The sutures are absorbable like catgut, vicryl rapid etc.Oral antibiotics, analgesics,local ointment & local sitz bath is required  after the procedure. Complications are immediate bleeding, recurrent adhesion (if the excess skin is not cut adequately), excess cutting of the prepucial skin, meatal stenosis etc.

Hodgkin's Disease

Introduction:

Hodgkin’s disease is a cancer of the lymphatic system. It is also called as Hodgkin’s lymphoma. Hodgkin’s disease accounts for 5% of cancers diagnosed in children. It is rare before the age of five years. The number of cases increases significantly in the second decade of life.

Symptoms:

1. enlarged lymph nodes, called lymphadenopathy
(Painless, firm, rubbery, and movable)
2. loss of appetite
3. loss of weight
4. fever
5. lethargy
6. lump in abdomen
7. pain in abdomen
8. itching
9. night sweat
10. cough or breathlessness.

Investigations:

1. X-ray Chest
2. Ultrasonography / CT scan of abdomen
3. lymph node biopsy
4. Complete Blood Count (specially ESR)
5. Biochemistry ( specific- LDH, β2 macroglobulin, serum albumin)
6. Bone marrow / Bone scan in advanced stage
7. PET scan

Treatment:

Hodgkin’s disease is treated with chemotherapy & radiotherapy. The surgery is done only for the diagnosis.

Chemotherapy:

The following drugs are used
1. Adriamycin
2. Bleomycin
3. Vinblastine
4. Dacarbazine

Radiotherapy:

Radiotherapy is used after 4 or 6 cycles of chemotherapy.

Prognosis: Excellent. 5 year survival rate is >85%.

Sacrococcygeal Teratoma

Sacrococcygeal teratoma is the tumor arising in sacrococcygeal region & it is the commonest tumor found in newborns. It is also seen in infants, children & very rarely in adults. The SCT is more common in girls than boys with ratio of 3:1. The routine use of prenatal ultrasound has made the diagnosis early during fetal life.

Symptoms:
1. Sacral mass
2. Mass in the abdomen & perineum
3. Distension of abdomen
4. Displacement of anus due to sacral mass
5. Constipation
6. Sacral sinus.

Classification: Altaman's classification
Type 1- Entirely outside
Type 2- Mostly outside
Type 3-Mostly inside
Type 4- Entirely inside

Diagnosis:
1. Prenatal Ultrasound- Solid/ cystic mass occupying abdomen as well as perineum
2. CT Scan abdomino-pelvic region/ MRI abdomino-pelvic region
3. Tumor markers- AFP or Alfafetoproteins

Treatment:
1. Surgical excision in benign or mature teratoma
2. Associated with chemotherapy in malignant or immature teratoma

Chemotherapy:
Bleomycin, Etoposide & Cisplatin (BEP) protocol is the commonest first line protocol used.

Prognosis- Good if complete surgical excision is done along with removal of coccyx.