Lymphangioma

Introduction: It is also called cystic hygroma. It arise as the result of abnormality of the development of lymphatic system.This is soft,multicystic, ill defined cystic mass that is brilliantly transilluminant

Sites: It can occur in any part of the body but the neck is the most commonest part. Other areas which gets affected are axillae,mediastinum, groin, and retroperitoneum.

Symptoms & signs: The presenting symptoms is usually soft, non tender swelling rarely evident at birth but may become evident as the child grows. The swelling may be subcutaneous deep or may be in the facial planes or in the muscular plane. There swelling is multicystic & cyst varies in size from macrocystic to microcystic in nature. The swelling is compressible & may be minimally reducible. It is brilliantly transilluminant.

Investigations:
Ultrasonography
CT scan/ MRI

Treatment:
1. Injection sclerotherapy: Aspiration of fluid & injection of sclerosants inside the cysts is the treatment advisable where the surgical excision is not possible.

2. Surgical excision: Excision of lymphangioms is the best form of treatment. This is benign lesion which can be excised without the margin of normal tissue. Wherever it is close to the vital structures, the cyst can be marsupilized, provided by all macroscopically abnormal lymphatics are removed.

Congenital Diaphragmatic Hernia

Introduction:

Congenital diaphragmatic hernia is a birth defect in which there is hole in a portion of diaphragm causing abdominal organs to go up in the thoracic cavity.This causes lung compression & respiratory distress in a newborn.

Incidence:

1 : 2000-5000 live birth
8 % of all major congenital anomalies
The cause of CDH is largely unknown

Prenatal Diagnosis:

Fetal USG shows stomach, intestines, liver, kidneys & spleen in the chest cavity
Abnormal position of certain viscera inside the abdomen
Stomach visualization out of its usual position
Intrauterine growth retardation
Polyhydramnios, Fetal hydrops

Clinical Presentation:

The newborn presents with severe respiratory distress

On examination:

Respiratory distress
Scaphoid abdomen
Auscultation of the lungs reveals poor air entry
Shift of the heart to the side opposite

Diagnosis:

Plain X-ray abdomen (erect) - shows the bowel loops in the thoracic cavity with compression of ipsilateral lung.

Ultrasonography of chest - will confirm the presence of the bowel & other viscera

2-D echo & Renal USG- To rule out associated cardiac & renal anomalies.

Management:

Mechanical ventilation
Nitric Oxide
Surfactant
ECMO
Surgery

Surgical management: By abdominal or thoracic approach the intestinal contents are placed in the abdomen & the hole in the diaphragm is sutured.

Juvenile Polyposis Syndrome

Introduction: Juvenile polyposis syndrome consists of three conditions, diffuse juvenile polyposis of infancy, diffuse juvenile polyposis & juvenile polyposis coli. These syndrome is characterised by multiple polyps in the gastrointestinal tract.

1.Diffuse juvenile polyposis of infancy: This occurs in first few months of life. The child presents with loose motions, blood in stool, protein loosing enteropathy, tendency for intussusception & hypotonia. On investigation, there are multiple polyps through out gastrointestinal tract. The treatment is keeping the baby nil by mouth & starting her/him on total parenteral nutrition.Once the condition stabilizes, then segmental resection. In spite of all the efforts, the prognosis of this condition is dismal.

2.Diffuse juvenile polyposis: This condition occurs at the age of 6 months to 5 years. The child presents with recurrent rectal bleeding & prolapse.There may be signs of malnutrition. The polyps are found in stomach, small intestine & large intestine including rectum. The treatment is either endoscopic polypectomies, open polypectomies and/or segmental resection. The prognosis is better though the need of long term follow-up for recurrence is high.

3. Juvenile polyposis coli: This is a genetic disorder with an autosomal dominant gene. The polyps are present in colon though mainly in sigmoid colon & rectum. The treatment is debatable as one school of thought is total colectomy, rectal mucosectomy & endoanal pull-through while other school of thought is endoscopic resection of polyps & regular colonoscopy. If one finds mucosal changes or dysplasia then resection is done.

In juvenile polyposis syndrome, the chances of development of carcinoma in later life is high even if the treatment is completed so the long term follow-up is mandatory.

Torsion of the testis

Introduction: Rotation of the testis on the spermatic cord causing interference with the blood supply is called torsion of the testis. It is an acute surgical emergency. If not operated within 6 hours, there can be irreversible damage to the testis.

Clinical presentation:
1. Painful swelling of the scrotum with redness
2. Onset is sudden
3. Lower abdominal pain
4. reflex vomiting

Diagnosis:
1. Clinically scrotum is red, tender
2. Doppler of testis reveal decreased blood supply in the affected testis

Treatment: Emergency surgery with derotation of testis and orchiopexy. At the same time other testis is also fixed (orchiopexy) as chances of torsion is high in contra lateral testis.

Differential diagnosis:
1. Epididymo-orchitis
2. Idiopathic scrotal edema

Choledochal cyst

Introduction: Choledochal cyst is congenital anomaly, where there is cystic dilatation of extrahepatic biliary duct, intrahepatic biliary duct or both.

Pathogenesis: Causative factors are

1.Congenital segmental weakness of the wall of biliary duct

2. Inflammatory degeneration of the wall of biliary duct (same as in biliary atresia)

Types:

Type I - Dilatation of entire or segment of common hepatic duct or coomon bile duct

a- Saccular

b- Fusiform

Type II - Diverticulum from common bile duct either sessile or pedunculated

Type III- Also called as choledochocele. Dilatation of intraduodenal portion of CBD

Type IV - Multiple cysts involving both intrahepatic & extrahepatic duct

Type V - Multiple cysts involving only intrahepatic ducts

Clinical presentation:

1. Abdominal pain

2. Mass in upper abdomen

3. Jaundice

Diagnosis:

1. Liver function tests

2. Ultrasonography of abdomen

3. Magnetic resonance cholangiopancreatography

4. Intraoperative cholangiogram

Treatment:

1. Excision of cyst & Roux-en-Y choledochojejunostomy

2. Excision of mucosa & anastomosis between side of the cyst & the jejunum

Tracheoesophageal Fistula

Introduction:

Tracheoesophageal fistula (TEF)is the congenital anomaly where there is abnormal fistulous connection between trachea & esophagus.The incidence of TEF is 1 in 2000-4000 live births.

Associated Anomalies:

Duodenal atresia
Down syndrome,
Ventricular septal defect

Age:

Congenital Tracheoesophageal fistula (TEF) present immediately after birth. H-type of fistula may present in neonates or in infancy.

Classification:

Type 1- Esophageal atresia with distal TEF - 87%
Type 2- Isolated esophageal atresia without TEF - 8%
Type 3- Isolated TEF-4%
Type 4- Esophageal atresia with proximal TEF-1%
Type 5- Esophageal atresia with proximal and distal TEF-1%

Symptoms:

1. Copious white frothy secretions comes out through mouth.
2. Coughing & choking while taking breast feed
3. Cynosis
4. increased respiratory rate

Diagnosis:

1. Plain X-ray chest shows coiling of infant feeding tube in mediastinum.
2. Stomach bubble is not seen in pure esophageal atresia
3. 2-D Echo- for associated cardiac anomalies
4. Ultrasonography of abdomen- to exclude renal / other abdominal anomalies

Treatment:

Thoracotomy, division of fistula & esophago-esophageal anastomosis is the primary line of treatment. If the child is premature with low birth weight & respiratory distress syndrome then one can do the staged procedures. Initially gastrostomy & esophagostomy. After stabilization primary repair. In pure esophageal atresia, initially gastrostomy & esophagostomy then esophageal replacement by gastric pull up,reverse gastric tube, colon transposition or ileal transposition.