History case

A child was born in term after normal pregnancy and delivery with body weight of 3600 gr. The early adaptation period was without peculiarities. He was at the breast feeding. In the first month the child gained 700 gr. to body weight. From the age of 1 month the baby began to vomit with curds once twice per day, afterward the abundant projectile vomiting occurred. At the age of 1 month and 10 days was examined by pediatrician who discover a body weight loss of 400 gr. in the last week. At the admission the child is in a poor condition, he is flaccid, the tissue turgescence is decreased, the subcutaneous fat is thin. During the examination the projectile vomiting with a touch of old blood occurred. Heart and lungs without pathology. The abdomen is soft with a distention in the epigastrium, the sand glass-shaped amplified gastric peristalsis is evident. At the examination the child weighted 3700 gr. In the blood analyzes: pH 7,56; pCO2 - 38 mm Hg; BE - +12; Hb 112 g/l; Ht 56%; plasmatic K+ - 3,2 mEq/l; plasmatic Na+ - 135 mEq/l. What is the suspected diagnosis?

Imaging

The ultrasound shows an elarged pylorus ( arrows). The length (green line) was 16mm (upper limit of normal 14mm), and the muscle thickness (orange line) was 4mm (upper limit of normal 3mm). This image also shows the antral nipple sign redundant pyloric mucosa protruding into the gastric antrum, and the cervix sign indentation of the pylorus into the fluid-filled antrum. Was no gastric contents were seen to pass through the pylorus at any time during the examination.

On UGI series the pyloric canal is narrowed and elongated (arrows), and the base of the duodenal bulb is stretched by the pyloric mass. The pyloric canal is narrow, demonstrating a double string sign.

Diagnosis???

Gastroesophageal reflux, Gastroenteritis, Hypertrophic pyloric stenosis Antral web, Foregut duplication cyst, Gastric tumors

N. Testemitanu State University of Medicine and Pharmacy Department of Pediatric Surgery, Orthopedics and Anesthesiology

Hypertrophic pyloric stenosis

Jalba Alexandru, MD, PhD, associate professor

Chisinau 2012

Epidemiology
o Hypertrophic pyloric stenosis (HPS) is one of the most

common surgical conditions of the newborn. o It occurs at a rate of 1 to 4 per 1000 live births in white infants but is seen less in non-white children. o Males are affected more often with a 4:1 male-to-female ratio. o Risk factors for HPS include family history, gender, younger maternal age, being a first-born infant, and maternal feeding patterns. o Premature infants are diagnosed with HPS later than term or post-term infants

Etiology
o
o

The cause of HPS is unknown, but genetic and environmental factors appear to play a large role in the pathophysiology.
Circumstantial evidence for a genetic predisposition includes race discrepancies, the increased frequency in males, and the birth order (firstborn infants with a positive family history). Environmental factors associated with HPS include the method of feeding (breast vs. formula), seasonal variability, exposure to erythromycin, and transpyloric feeding in premature infants. Additionally, there has been interest in several gastrointestinal peptides or growth factors that may facilitate pyloric hypertrophy. Some of these include excessive substance P, decreased neurotrophins, deficient nitric oxide synthase, and gastrin hypersecretion. Thus, the etiology of HPS is likely multifactorial with environmental influences.

Presentation
o The classic presentation of HPS is nonbilious, projectile vomiting in a full-term neonate who is between 2 and 8 weeks old. o Initially, the emesis is infrequent and may appear to be reflux. o However, over a short period of time, the emesis occurs with

every feeding and becomes forceful (i.e., projectile).

o The contents of the emesis are usually the recent feedings, but signs of gastritis are not uncommon (coffee-ground emesis).

Physical examination
o If the diagnosis is made early the neonate usually appears well.
o However, depending on the duration of symptoms and degree of dehydration, the neonate may be gaunt and somnolent. o Visible peristaltic waves may be present in the mid to left upper abdomen. o To palpate the pyloric mass (i.e., olive), the neonate must be relaxed. Techniques for relaxing the patient include bending the neonates knees and flexing the hips and using a pacifier with sugar water. These techniques should be attempted after the stomach has been decompressed with a 10-Fr to 12-Fr orogastric tube (See figure below). o However, if the diagnosis is unclear after a thorough physical examination, radiologic evaluation is warranted.

Palpation

Figure. After palpating the liver edge, the examiners fingertips should slide underneath
the liver in the midline. Slowly, the fingers are pulled back down, trying to trap the olive. Palpating the pylorus requires patience and an optimal examination setting. The definitive diagnosis can be made in a considerable number of patients with this examination.

Diagnosis

o Ultrasonography has become the standard technique for diagnosing HPS and has supplanted the physical examination at most institutions.

o The diagnostic criteria for pyloric stenosis is a muscle thickness of greater than or equal to 3 mm and a length of greater than or equal to 16 mm (See figures below).
o A thickness of more than 3 mm is considered positive if the neonate is younger than 30 days of age. o The study is dependent on the expertise of the ultrasound technician and radiologist.

Diagnosis

o The transverse (A) and longitudinal (B) views of hypertrophic pyloric stenosis are seen here. Muscle thickness greater than or equal to 4 mm on the transverse view or a length greater than or equal to 16 mm on the longitudinal view is diagnostic of pyloric stenosis. On this study, the pyloric wall thickness was 5 mm and the length (arrows) was 20 mm.

Associated conditions
Associated conditions with malrotation
Intestinal atresia Imperforate anus Cardiac anomalies Duodenal web

Incidence (by percent)

5-26% 0-9% 7-13% 1-2%

Associated anomalies common (See table)

are

o Rotational disorders are also known to coexist with heterotaxia but rarely with situs inversus. o Intestinal malrotation occurs along a wide spectrum of anatomic variants and clinical presentations, including midgut volvulus and chronic duodenal obstruction. o In-utero volvulus may lead to intestinal atresia.

Meckels diverticulum
Hernia Trisomy 21

1-4%
0-7% 3-10%

Rare: esophageal atresia, biliary atresia, mesenteric cyst, craniocynostosis, Hirschsprungs disease, intestinal duplication.

Diagnosis
o If the ultrasound findings are equivocal, then an upper gastrointestinal series can be helpful in confirming the diagnosis (See figure below). o It is, however, important to evacuate the contrast agent after the study to reduce the risk of aspiration and deleterious pulmonary complications.

o UGI series. When ultrasound study for pyloric stenosis is not available as well as in some instances, when ultrasound study is equivocal. In this upper gastrointestinal study, note the string sign indicating a markedly diminished pyloric channel (arrow) and subsequent gastric outlet obstruction. It is important to evacuate the contrast material after this study to reduce the risk of aspiration and pulmonary complications.

Diagnosis
o A gasless abdomen is particularly worrisome because the compromised bowel may be filled with fluid alone. o Of particular importance is the presence of rectal gas in the newborn, which essentially excludes duodenal atresia (but not duodenal stenosis or web). o If the diagnosis remains in doubt, an emergent upper gastrointestinal contrast study is needed to document the position of the ligament of Treitz. o Delineation of the duodenojejunal junction remains the most important diagnostic tool with high sensitivity and is preferred over barium enema. o The duodenum should be seen traveling across the spine to the left. Additionally, the lateral film will show the duodenum obtaining a retroperitoneal, posterior position. o Abnormal findings include positioning of the duodenojejunal flexure to the right of the spine, obstruction of the duodenum, and the coil spring, corkscrew, or beak appearance of the obstructed proximal jejunum (See figures below). o In some settings, color Doppler ultrasound imaging is favored and may reveal a dilated duodenum with inversion of the superior mesenteric artery and vein (the whirlpool sign) in cases of acute volvulus. o Moreover, with ultrasonography, it is now possible to diagnose intestinal volvulus in utero.

Differential diagnosis
The differential diagnosis for nonbilious vomiting should be considered. This includes medical causes such as:

gastroesophageal reflux, gastroenteritis, increased intracranial pressure, and metabolic disorders.

Anatomic causes would include:

an antral web, foregut duplication cyst, gastric tumors, or a tumor causing extrinsic gastric compression.

Management
The mainstay of therapy is typically resuscitation followed by pyloromyotomy. There are reports of medical treatment with atropine and pyloric dilation, but these treatments require long periods of time and are often not effective. Once the diagnosis of HPS is made, feedings should be withheld. Gastric decompression is usually not necessary but occasionally may be required for extreme cases. If a barium study was performed, it is important to remove all of the contrast material from the stomach to prevent aspiration.

Management

The hallmark metabolic derangement of hypochloremic, hypokalemic metabolic alkalosis is usually seen to some degree in most patients. Profound dehydration is rarely seen today, and correction is usually achieved in less than 24 hours after presentation. A basic metabolic panel should be ordered and the resuscitation should be directed toward correcting the abnormalities. Most surgeons use the serum carbon dioxide (<30 mmol/L), chloride (>100 mmol/L), and potassium (4.5-6.5 mmol/L) levels as markers of resuscitation. Initially, a 10- to 20-mL/kg bolus of normal saline should be given if the electrolyte values are abnormal. Then D5/12NS with 20 to 30 mEq/L of potassium chloride is started at a rate of 1.25 to 2 times the maintenance rate. Electrolytes should be checked every 6 hours until they normalize and the alkalosis has resolved. Then the patient can safely undergo anesthesia and operation. It is important to appreciate that HPS is not a surgical emergency and resuscitation is of the utmost priority

Surgery

After general anesthesia has been induced, an abdominal examination should be performed to physically check for an olive if one was not detectable preoperatively.

The pyloromyotomy may be performed by the standard open technique or by the minimally invasive approach.
The anesthesiologist should pass and leave a suction catheter in the stomach for decompression and for instilling air after the pyloromyotomy to check for a leak.

Surgery (The open approach)

The typical right upper quadrant transverse incision seems to be used most commonly (See figures below).

o These two children underwent open pyloromyotomy through a right upper quadrant transverse incision. Over time, the cosmetic appearance of their incision is not as attractive as that seen after the laparoscopic operation.

Surgery (The open approach)

The patient is placed in the supine position. A 2.5- to 3-cm long transverse incision is made lateral to the lateral border of the rectus muscle. When supra-umbilical skin fold incision is employed, a circumumbilical incision is made through about two-thirds of the circumference of the umbilicus.

Surgery (The open approach)

The stomach is identified and is grasped proximal to the pylorus with non-crushing clamp and brought through the wound.

Surgery (The open approach)

The pylorus is held with surgeons thumb and forefinger to stabilize and assess the extent of hypertrophied muscle. A seromuscular incision is made over the avascular area of pylorus with a scalpel, commencing 1~2 mm proximal to the pre-pyloric vein along the gastric antrum. The incision should go far enough onto the gastric antrum at least 0.5~1.0 cm from the antropyloric junction where the muscle is thin.

Surgery (The open approach)

The scalpel handle may be used to further split the hypertrophied muscle down to the submucosal layer. Then pyloric muscle is spread widely. Spreader is placed at the midpoint of incision line and muscle is spread perpendicularly and spreading must be continued proximally and distally. Gentle spreading is required to obtain a complete myotomy.

Surgery (The laparoscopic approach)

Surgeon

Video monitor

Anesthesiologist . Scrub nurse

Assistant

For the laparoscopic procedure the patient is placed in the supine position at the end of the operating table (or 90 to the anaesthesiologist). The video monitor is placed at the head of the table, and the surgeon stands at the end of the table with the assistant to the patients right. Intra-abdominal pressure is maintained at 8 mmHg, and insufflation rate is set at 0.5 l/min.

Surgery (The laparoscopic approach)

Pyloric vein

The duodenum is grasped just distal to the pyloric vein (pyloroduodenal junction) and retracted using the atraumatic grasper to expose the avascular surface of hypertrophic pylorus. A seromuscular incision is made over the hypertrophic pylorus with retractable myotomy knife commencing at 12 mm proximal to the pyloroduodenal junction extending to the gastric antrum.

Surgery (The laparoscopic approach)

After the muscle is incised, the blade is then retracted and the laparoscopic pyloromiotomy spreader is used to further split the hypertrophied muscle fibre, as the scalpel handle is used in open procedure, until mucosa is visualized.

Conclusions

Pyloromyotomy is the standard therapy for IHPS. Mortality associated with this operation is very uncommon today. Early diagnosis and proper peri-operative management reduces complications. In spite of these advances, there remains about an 810% incidence of associated peri-operative morbidity such as perforation, wound infection . and wound dehiscence. In an open procedure, essentially right umbilical incision and circumumbilical incision, manipulation of and tension on the pylorus to deliver it through the wound can induce oedema in muscle layer mucosal swelling and, occasionally serosal laceration. A laparoscopic pyloromyotomy (LP) is a less traumatic operation. The tolerance of an early feeding regimen in the LP confirms that there is lack of trauma to the pylorus during the procedure.