Skip to main content

Hypertrophic pyloric stenosis of infancy

Incidence of 3/1000 births. 
4 times more in males. 
Aetiology is unknown. In some cases there seems to be a familial association. In such families the mother has suffered from the condition in 50 per cent of cases. Characteristically it is a first-born male child that is most commonly affected. The condition is most commonly seen at 4 weeks after birth ranging from the third week to, on rare occasions, the seventh. Inexplicably, it is the time following birth that seems important and not the child’s gestational age. A premature infant will also develop the condition at about 4 weeks after birth.

Grossly hypertrophied musculature of the pylorus and adjacent antrum, the hypertrophy being maximum in the pylorus itself. The mucosa is compressed such that only a probe can be inserted.

Clinical features:
Vomiting is the presenting symptom that after 2—3 days becomes forcible and projectile. The child vomits milk and no bile is present. Immediately after vomiting the baby is usually hungry. Weight loss is a striking feature and rapidly the infant becomes emaciated and dehydrated. Diagnosis can usually be made with a test feed. This may produce characteristic peristaltic waves that can be seen to pass across the upper abdomen. At the same time, using a warm hand, the abdomen is palpated to detect the lump.

Ultrasonography is the investigation of choice as it can, without difficulty, detect the classical features in the pyloric canal.
Contrast radiology was done in the past but is not necessary now.

Differential diagnosis:
The common D/D are gastro-oesophageal reflux, feeding problems, urinary tract infection and raised intracranial pressure.
 The condition cannot normally be confused with duodenal atresia or intestinal obstruction because of the absence of bile in the milk vomit.

Following diagnosis the first concern is to correct the metabolic abnormalities. Essentially this is the same situation that pertains in adults with the patient being dehydrated, with low sodium, chloride and potassium, and a metabolic alkalosis.
The child should be rehydrated with dextrose—saline and potassium (2.5 per cent dextrose plus 0.45 per cent sodium chloride plus 1 g of potassium chloride per 500 ml of fluid). This will restore the infant’s clinical condition and electrolytes to normal. Following stabilization of the patient, operation is required. 
Conservative treatment has little place in the management of this condition as with appropriate surgical treatment recovery is virtually 100 per cent.

Ramstedt’s operation
1) In preparing the child for operation it is important that the stomach is emptied and washed out with saline, and that hypothermia is avoided. To achieve this, the patient is encased in cotton wool allowing exposure of the upper abdomen. 
2) Operation is performed under general anaesthesia, although it is possible to perform the procedure under a local anesthetic. 
3) The skin is opened through a transverse incision placed in the upper abdomen over the right rectus sheath, which is opened in the same line. The rectus muscle is then split along the line of its fibres and the posterior rectus sheath opened in the line of the skin incision. 
4) The hypertrophied pylorus is delivered and rotated so that its superior surface comes into view. Thus, the least vascular portion can be selected for incision. To ascertain the distal limit of the hypertrophy the surgeon invaginates the duodenum with the index finger. 

5) The incision is made through the serosa only and from this point along the whole length of the pylorus and, importantly, the distal antrum. The hypertrophied pylorus has the consistency of an unripe pear, hence splitting the muscle coats can be accomplished by blunt dissection. On separating the edges with artery forceps the pyloric mucosa bulges into the cleft which has been made in the muscle as shown in the diagram above. 
6) Great care is taken not to penetrate the mucosa. When this injury occurs it is almost always in dividing the most distal part of the constricting fibres which are in the vicinity of the duodenal fornix. To be sure that there is no perforation some air is squeezed from the stomach into the duodenum. If a perforation has occurred it is closed and a piece of omentum placed over the closure. 
7) Haemostasis should be meticulous.

After operation the nasogastric tube can be removed and feeding commenced on the morning after operation. If the infant manages to feed without difficulty it can be discharged early from hospital. If the mucosa is inadvertently opened it is wise to delay feeding for 48 hours and to retain the child in hospital longer.


Popular posts from this blog

Hypokalemia - Potassium replacement calculation

 DEFINITION  Hypokalemia is defined as a serum potassium level of less than 3.5 mmol/L. Normal level= 3.5-5.5 mmol/L. It is encountered in >20% of patients. Patients are usually asymptomatic but severe arrhythmias and rhabdomyolysis can occur. Non-specific complaints include easy fatiguability and skeletal muscle weakness. The preferred method of replacement is via the oral route but at times this is not possible. The article below will give you an idea about how to calculate the amount of KCl to be given I.V. 1) Potassium deficit in mmol is calculated as given below: K deficit  (mmol) = (K normal lower limit  - K measured ) x kg body weight x 0.4 2) Daily potassium requirement is around 1 mmol/Kg body weight. 3) 13.4 mmol of potassium found in 1 g KCl . ( molecular weight KCl = 39.1 + 35.5 = 74.6) Suppose we get an asymptomatic patient of  70 Kg with a serum potassium level of 3.0 mmol/L and he is on nil by mouth but having an adequate diuresis, w

The plantar reflex - Babinski's sign

The plantar response is an important test to identify an upper motor neuron lesion.  PROCEDURE  To elicit it, the muscles of the lower limbs must be relaxed. The outer edge of the sole of the foot is stimulated by firmly scratching a blunt object like a key or a stick along it from the heel towards the little toe. This is what  Joseph Babinski did in the year 1896. He described the 'great toe sign' that year and then in 1903 the 'toe abduction or fan sign'. Nowadays, a final medial movement across the sole of the metatarsus is also done. i.e. we start at the heel to the little toe and finally arcing to the big toe. The final arcing movement is absent in the original Babinski plantar response test. Babinski sign refers to a combination of 'the great toe sign' and the 'fan sign'.  SIGNIFICANCE  The normal response is plantar flexion of the toes (down going) and they are drawn together. More precisely, there is flexion of the big toe and addu

Differences between hyperemia and congestion

Hyperemia and congestion both indicate a local increased volume of blood in a particular tissue. Hyperemia is an active process that result from augmented blood flow due to arteriolar dilation (e.g. at sites of inflammation or in skeletal muscle during exercise). The affected tissue is redder than normal because of engorgement with oxygenated blood. Congestion, on the other hand, is a passive process resulting from impaired venous return out of a tissue. It may occur due to systemic causes like cardiac failure or a local cause like isolated venous obstruction. The tissue is cyanosed because the worsening congestion leads to accumulation of deoxygenated hemoglobin in the affected tissues. 

Apgar scoring - table, mnemonic

 INTRODUCTION  The  Apgar score  was devised in 1952 by Dr Virginia Apgar (anesthesiologist) as a simple and repeatable method to quickly and summarily assess the health of newborn children immediately after birth.  This helps to identify those requiring resuscitation and can also be used to predict survival in the neonatal period.   MNEMONIC  A mnemonic for learning purposes includes: A - Appearance (skin colour) P - Pulse (heart rate) G - Grimace (reflex irritability) A - Activity (muscle tone) R - Respiration  Another mnemonic is also useful:  How -   Heart rate Ready - Respiration Is -        Irritability This -    Tone Child -   Colour Apgar scoring is divided into 1 and 5-min scores.  1-MIN SCORE    Sixty seconds after complete birth, the five parameters specified in the table above must be evaluated and scored. A total score of 10 indicates that the baby is in the best possible condition. A score between 0-3 me

Chickenpox - dew on rose petal appearance

Definition: Chickenpox is a benign viral disease of childhood, characterized by an exanthematous vesicular rash. It is an extremely common and contagious condition. It is caused by the varicella-zoster virus which is a herpes virus and contains a double stranded DNA in its center. Epidemiology: Age group affected- 5 to 9 years. The infection can be there at other ages too but it is less frequent. It is highly contagious and it affects all races and both sexes equally. The attack rate is around 90% among seronegative persons. Pathogenesis: Incubation period- 10 to 21 days but is usually 14 to 17 days. Patients are infectious around 48 hours from onset of vesicular rash, during the period of vesicular formation (around 4-5 days) and until all vesicles are crusted. Transmission occurs by respiratory route. The virus is believed to be localized in the nasopharynx, in the reticulo-endothelial system. It then enters the blood. This stage of viremia is characterized by diffused ski