Anaphylaxis: an allergic reaction

Anaphylactic shock is an allergic reaction of the body which can at times lead to death if not recognized early. It can be to the drug or food. Anaphylaxis ranges from mild symptom like itching to the fatal death. So one has to be very careful & fully aware of this entity.It is a medical emergency.

It is mediated by Immunoglobulin E (IgE) antibody mediated and causes increased vasodilatation, increased vascular permeability & bronchospasm. Vasodialation occurs in many system of the body like skin & subcutaneous tissue, respiratory system, cardiovascular system & gastrointestinal system resulting in shock. Involvement of heart & lungs can give rise to cardiogenic & respiratory shock which is potentially fatal.

Clinical features:

It starts within few minutes of exposure to the allergic substance and responses well with immediate treatment. Recognision of anaphylactic shock is thus very important.

1. Urticaria
2. Difficulty in breathing
3. Sweating
4. Hypotension
5. Altered sensorium
5. Shock

Immediate treatment:

1. Patient should be immediately admitted in intensive care unit.

2. Airway restoration: monitor airways and if it is compromised then immediate intubation with endotracheal tube & ventilatory support

3. Oxygen by mask/ nasal prongs/ ventilator

4. Circulation: Intravenous access & intravenous fluids according to central line monitoring.

5. Intensive monitoring of pulse, Blood pressure, oxygen saturation, respiratory rate, urine output.

6. Adrenaline:Drug of choice

It causes vasoconstriction, bronchodilatation & relief of angioedema.It should be administered in the doses 0.01 ml/kg of of 1:1000 solution subcutaneously.It can be repeated after 15 minutes if required.

7. Vasopressors for hypotension

8. Anti-histaminics

9. Steroids, Aminophylline

10. Nebulization with bronchodilators

Constipation in Children

Difficulty in passing stool which is firm to hard in consistency is called constipation.If untreated, it can lead to rectal impaction of feces and overflow incontinence (encopresis). Constipation is seen more common in male children than female children.

Causes of Constipation:

1. Dietary cause- Change from breast feeding to bottle feeding or introduction of solid food can cause constipation. Even if the formula feed is not mixed with sufficient quantity of water can give rise to constipation.

2. Cow's milk is itself a constipatory agent. If given in excess, can lead to hard stool.

3. Cystic fibrosis which is a genetic disease can cause constipation.

4. Anorectal problems- Anorectal problems like Anal stenosis, anterior ectopic anus, Anal fissure, Presacral mass can cause constipation.

5. Neurological problems- Meningomyelocele can involve the bowel leading to constipation.

6. Local intrinsic neuronal problems like Hirschsprung's disease, meconium plug syndrome or colonic dysmotility can cause constipation.

7. Metabolic problems like hypothyroidism.

8. Functional Constipation (most common)

Clinical features:

1. Chronic recurrent, nonspecific pain in abdomen
2. Difficulty in passage of stool
3. Eneurosis
4. Soiling of stool
5. Hard stool
6. Perianal pain/ Fissure-in- ano

On Examination:

1. Abdominal Examination: On examination, abdomen will be soft, non-tender, mildly distended. Fecolomas may be palpable.

2. Peri-anal & Anal examination: Fissure, anal stenosis, rectal mucosal prolapse can be seen on perianal examination.

3. Per-rectal examination: Decreased anal tone, shortened anal canal & distended rectal ampulla with full of stools.

Diagnosis:

1. Abdominal X-ray- Fecal matter, vertebral anomalies

2. Contrast Enema- Dilatation of colon & rectum up to the anal verge is suggestive of functional constipation while narrow rectum/ recto sigmoid with dilated proximal colon is suggestive of Hirschsprung's disease.

3. Cinedefecography

4. Anorectal manometry with electromyography can differentiates between Hirschsprung's disease and functional constipation.

5. CT/ MRI- For anatomical problems

6. Rectal full thickness biopsy- To rule out Hirschsprung's disease.

Treatment:

Treatment of underlying cause is the mainstay of management.

1. Correction of anatomical anomalies like dilatation in case of anal stenosis, anoplasty in case of ectopic anal opening.

2. Colostomy followed by pull-through surgery in case of Hirschsprung's disease.

3. Enemas, laxatives, stool softeners, toilet training

4. High fiber diet, plenty of liquids

5. Avoidance of milk or milk related products.

Meningomyelocele or Spina Bifida Manifesta

Introduction:

Herniation of only meninges (covering over the spinal cord) or meninges along with neural elements (spinal cord elements) is called meningocele or meningomyelocele (MMC) respectively. This occurs due to failure of closure of the neural tube during the third week of foetal life. There is defect in the spine through which the meningomyelocele is open to the surface of the skin. The children born with this defect are prone to have bacterial meningitis due to the leak of cerebrospinal fluid (CSF) though the spinal defect. Therefore the child should be operated within 48 hours of delivery to prevent meningitis.The neurological deficit caused by MMC is irreversible and very rarely improves following repair. The further deterioration, however, can be prevented with the surgical repair of MMC.

Clinical presentation:

1. Prenatal diagnosis of MMC by ultrasound during pregnancy is one of the commonest mode of presentation. In such cases maternal serum alpha-fetoprotein is raised.
2. After birth, mid-line spinal swelling which is covered with the meninges. It can be cystic or some neural plaque can be seen into the swelling after trans-illumination test.
3. There may be weakness in the lower limbs i.e. legs or paralysis of lower limbs.
4. Bladder and bowel involvement may be seen in some cases with incontinence of passage of meconium & full bladder.
5. If MMC is associated with hydrocephalus then there is enlarged head with increased head circumference.

Diagnosis:

1. Prenatal - Fetal USG shows the spinal swelling and there is increased maternal serum alpha-fetoprotein.
2. Postnatal- X-ray spine- defect in spinal arch with soft tissue swelling
3. MRI spine- swelling content may be only CSF or neural strands or sometimes whole spinal cord.
4. USG/ CT scan Head- to rule out associated hydrocephalus.

Treatment:

Surgical repair of MMC is done within 48 hours of birth to prevent infection. MMC repair includes dissection & restoration of neural plaque in to spinal cord. Closure of dura followed by skin mobilization & skin closure.If there is associated hydrocephalus then ventriculoperitoneal shunting is also required.

Varicocele Can be Cause of Male Infertility

Introduction:

Varicocele is dilatation of pampiniform plexus (testicular veins). Because of dilatation of veins there is increased temperature inside the scrotum which may cause testicular insufficiency leading to testicular atrophy and subsequent infertility.

In smaller children, varicocele may be caused by kidney tumors like Wilms' tumor, Neuroblastoma or hydronephrosis. The mass effect causes obstruction to the testicular venous return which opens into renal vein.

Clinical presentation:

1. Soft, painless, compressible swelling at the upper part of scrotum above testis.
2. This swelling gives characteristic 'Bag of worms' appearance.
3. Dragging sensation into scrotum.

Etiology:
1. Absence of valves in testicular veins.
2. Perpendicular entry of left testicular vein into high-pressure left renal venous system.
3. External pressure of loaded sigmoid colon on testicular vein.
4. Left adrenal veins also opens into left renal vein exactly opposite to the entry of left testicular vein. Because of adrenaline secreted by adrenal gland, there is spasm at the origin of vein.

Effects of Varicocele:

Testicular atrophy and subsequent infertility in adult life.

Investigations:

The indications for surgical treatment for varicocele are,
1. Chronic pain and discomfort
2. Sub-infertility in adults
3. Atrophy of the testis
4. 10% difference in volume of both testis measured by orchidometer

Surgical treatment:

1. Palomo's procedure:
Mass ligation of testicular vein and artery in the retro peritoneum above the internal ring.

2. Laparoscopic varicocelectomy

Complications:

1. Recurrence
2. Testicular atrophy
3. Nerve Injury
4. Injury to vas
5. Reactive hydrocele

Vitello-intestinal Duct Remnants

Introduction:

Vitello-intestinal duct or omphalo-mesenteric duct connects the midgut to the yolk sac during early embryonic life. It gets obliterated and disappears during fifth to sixth week of intrauterine life.If the remnants persists then following abnormalities can occur:

1. Umbilical Polyp:

Umbilical polyp occur because of persistance of small portion of vitello-intestinal duct epithelium at the base of umbilicus. Clinically, a polyp is seen at the base of umbilicus after the umbilical cord falls off. This differs from umbilical granuloma in the sense that it does not respond to electrical or chemical cauterization like granuloma. The polyp can be pedunculated or sessile and treatment is excision of polyp.

2. Umbilical Sinus:

When umbilical portion of vitello-intestinal duct remains patent, an umbilical sinus forms. There is foul smelling persistent pus or mucoid discharge from umbilicus. On close examination an opening is seen in the umbilicus. Ultrasonography may reveal collection just below the umbilicus. The treatment is exploration and excision of sinus. The peritoneal cavity also opened to look for any fibrous strand connected with ileus.

3. Fibrous remnant of vitello-intestinal duct:

Whole vitello-intestinal duct become fibrous strand but does not disappear. This can cause mechanical intestinal obstruction at any age of the life. Exploratory laparotomy and excision of strand is all that is required.

4. Meckel's Diverticulum:
It is very well known terminology. The ileal portion of vitello-intestinal duct remains patent and form a diverticulum, called as Meckel's diverticulum. This diverticulum characteristically arises from the antimesenteric border of the ileum within 20 cm of ileo-caecal junction. It can present with infection, bleeding or obstruction.

5. Patent Vitello-intestinal duct:
When whole vitello-intestinal duct remains patent cause fistulous connection between umbilicus and ileum. There is discharge of either meconium or flatus or both per umbilicus. This is tackled surgically as an emergency. The treatment is exploratory laparotomy, in toto excision of fistulous tract with the portion of the ileum attached with ileo-ileal anastomosis

Urinary Stones in Children

Introduction:

Childhood urolithiasis or urinary tract stones is common problem in children.The causes are anatomical abnormalities of urinary tract giving rise to stasis of urine, urinary infection, change in pH of urine etc.

Classification:

1. Anatomic

Pelviureteric junction obstruction
Vesicoureteral reflux
Neurogenic bladder
Extrophy of bladder
Ureterocele
Urethral diverticulum

2. Infection:

Stasis of urine causes infection. These infective stones are called as struvite stones.

3. Metabolic:

Cystinuria
Hyperoxaluria
Renal tubular acidosis
Urine acid stones
Hypercalciuria

4. Idiopathic:

In 20% of cases the exact cause of urolithiasis is unknown. Low intake of milk/animal proteins may be one of the cause.

Clinical presentation: Girls> Boys

1. Colicky pain in abdomen
2. Frequency of urination
3. Hematuria
4. Recurrent UTI
5. Passage of stones in urine

Diagnosis:

1. Urine Routine & Microscopy
2. Urine Culture
3. Serum Electrolytes
4. Serum Uric Acid
5. Serum Creatinine
6. Serum Calcium
7. Serum phosphorus
8. Serum Magnesium
9. Ultrasonography- Abdomen & Pelvis
10. Intravenous Pyelography
11. CT Scan Abdomen in some cases)

Treatment:

1. Conservative - in tiny stones. Plenty of liquids orally with antibiotics or IV fluids with diuretics
2. Extracorporeal Shock Wave lithotripsy - ESWL- It is used in renal pelvic stones, calyceal stones or upper ureteral stones which are less than 2 cm in diameter.
3. Percutaneus nephrostolithotripsy- Percutaneus access of calyx by making a puncture then tract is dilated with serial dilators. This tract is used to for use of lithotripter. The PCNL is used for stones more than 2 cm or hard stones. It is also used in combination with ESWL.
4. Ureteroscopic extraction of stone- small stones in lower ureters can be removed with ureteroscopic extraction.
5. Cystolithopaxy- Transurethral defragmentation of bladder stones with electrohydraulic lithotripter.
6. Open surgeries like nephrolithotomy or cystolithotomy.

Colostomy in Anorectal malformation

Colostomy means surgically created stoma through the abdominal wall for the passage of stool.

This is done in many congenital anomalies. To name some of them are anorectal malformation (ARM), Hirschsprung's disease, Necrotizing enterocolitis (NEC) etc. The colostomy in ARM is usually temporary i.e. until the definite anal passage is created in the perineum. Colostomy is done as a first stage in high and intermediate type of ARM.

The types of colostomy are loop colostomy and completely divided skin bridge colostomy according to the way it is created. It can be transverse or high sigmoid colostomy according to the sit. Transverse loop colostomy is simple and rapid to create so preferred in emergency situation. In addition, it leaves large part of the colon for reconstruction. However, majority of times, high sigmoid colostomy is all that needed. High sigmoid colostomy is easy to maintain for longer period and easier for giving a distal colonic wash which is required for definite management of ARM.

The complications of colostomy are skin excoriation, colostomy prolapse, colostomy stenosis, colostomy retraction and pericolostomy herniation.

The distal loop washes are given until the definite management of ARM. Just before the reconstruction, distal loopogram is done to delineate the anatomy, which is useful for the final surgical repair. Colostomy is closed in third stage after 4-6 weeks of final reconstruction.

Omphalocoele

Introduction:

External herniation of abdominal viscera through a central defect into the base of the umbilical cord.It is also called as Exomphalos.It can be minor exomphalos or major exomphalos according to defect size & contents.Umbilical cord is inserted into the crown of covering or sac.Incidence is 1-5,000-10,000 live births.

Etiology:

Because of failure of migration and fusion of cranial, caudal and/or lateral folds because of which there is failure of intestines to return into abdominal cavity.

Associated Congenital Anomalies: 75-80%

trisomy 13, 15, 21
cardiac anomalies
craniofacial
gastrointestinal
Beckwith-Wiedeman Syndrome
Pentalogy of Cantrell
Epigastric omphalocoele
Sternum cleft
Diaphragmatic defect
Ectopia cordis
Cardiac anomaly

Survival: 20% with heart disease
70% without heart disease

Treatment:

1.surgical repair of defect in minor type after repositioning of abdominal contents
inside the cavity.
2.In major type more conservative approach towards slow and continuous
reduction of contents by traction.

Hirschsprung's disease

Introduction:It is due to absence of autonomic ganglion cells in Auerbach's plexus of distal large intestine. It commences at internal sphincter and progresses for variable distance proximally. Lack of peristalsis causes colonic obstruction. It affects 1 in 5000 live births. Male : female ratio 4:1.75% cases confined to recto-sigmoid, 10% cases have total colonic involvement. 80% present in neonatal period with delayed passage of meconium followed by increasing abdominal distension and vomiting.

Symptoms:

Neonatal age:

1. Delayed Passage of meconium
2. Constipation
3. Progressive abdominal distension
4. Vomitings
5. Enterocolitis

Older child:

1. Constipation
2. Abdominal distension

Investigations:

Plain abdominal x-ray will confirm intestinal obstruction.
Barium enema - Contracted rectum, cone shaped transitional zone and proximal dilatation.
Anorectal manometry - No recto-sphincteric inhibition reflex on rectal distension
Rectal biopsy shows: Absent ganglion cells in submucosa, Increased acetylcholinesterase
cells in muscularis mucosa, Increased unmyelinated nerves in bowel wall.

Treatment:

Initial defunctioning colostomy to relieve obstruction & then definitive pull through procedure with excision of aganglionic segment.

Hookworm Infestation in Children

Two species of hookworms commonly infect humans, Ancylostoma duodenale and Necator americanus. These species are found throughout the tropics and subtropics.Most people who are infected are asymptomatic. The most significant risk of hookworm infection is anemia secondary to loss of iron into the gut.

Hookworm larvae emerge from passed eggs within 24 hours. Molt to an infective filariform larval stage in another 24 hours. After molting, larvae are able to penetrate intact skin. Walking barefoot in soil contaminated with feces - most common method of exposure.

After skin penetration, the venous circulation carries larvae to the pulmonary bed, where they lodge in pulmonary capillaries. Within 3-5 days, the larvae break through into alveoli & travel up from the lungs into the bronchi, the trachea, and the pharynx. Upon reaching the pharynx, larvae are swallowed and gain access to the GI tract. Once in the GI tract, worms attach to the wall of the intestine and begin to feed on the blood of the host. Chronic loss of blood and serum proteins leads to hookworm anemia and impaired nutrition.

Though rare, hookworm infestation should be suspected as a cause of gastrointestinal bleeding in infants in communities with a high risk of infestation. Prognosis is excellent with proper antihelminthic treatment.