Pediatr Surg Int (2014) 30:467–480 DOI 10.1007/s00383-014-3488-8

ORIGINAL ARTICLE

The ins and outs of pyloromyotomy: what we have learned in 35 years Sigmund H. Ein • Peter T. Masiakos Arlene Ein



Accepted: 18 February 2014 / Published online: 14 March 2014  Springer-Verlag Berlin Heidelberg 2014

Abstract Purpose/background The aim of the study is to evaluate a large series of infantile hypertrophic pyloric stenosis (IHPS) patients treated by one pediatric surgeon focusing on their diagnostic difficulties and complications. Methods From July 1969 to December 2003 (inclusive), the charts of 791 infants with IHPS were retrospectively reviewed. Results There were 647 (82 %) males and 144 (18 %) females; mean age was 38 days, median 51 (range 7 days– 10 months). When ultrasonography (US) was routinely used (1990), the age at diagnosis decreased to \40 days. The mean weight before and after routine US was 3.2 kg, median 3 (range 1.5–6). Twenty-five (3.1 %) were premature at diagnosis, mean age 49 days, median 56, (range 1–3 months) and mean weight 2.5 kg, median 2.3 (range 1.5–3.2). Eighty-one (10 %) had a positive family history. Forty-four (5 %) were non-Caucasians. Seventy-five (9 %) had other medical conditions, anomalies and/or associated findings. Sixty (7 %) patients had abnormal preoperative electrolytes. Ten (1.2 %) pylorics occurred after newborn operations. Of the entire total (791) who were treated, there were 13 (1.7 %) not operated on. All operations were done open initially through one of two right upper quadrant incisions, and then through an upper midline incision under general endotracheal anesthesia; 14 (1.7 %) had concomitant procedures. Prophylactic antibiotics (from 1982) decreased the wound infection rate to 3.9 %. There were a

total of 87 (10 %) complications which included 9 (1.1 %) intraoperative, (including mistaken diagnoses) 78 (9 %) postoperative: 59 (2 %) early (\1 month) and 19 (2.4 %) late ([1 month). The 13 (1.6 %) postoperative transfers (12 from non-pediatric surgeons) had 16 (18 %) complications (including 1 death); five (33 %) requiring reoperation (4 incomplete, 1 perforation). There were two deaths. Conclusions IHPS should be considered in any vomiting infant. US allows earlier diagnosis. Serious complications are uncommon and avoidable, but recognizable and easily corrected. Higher surgeon volume of pyloromyotomies ([14 per year) is associated with fewer complications. Keywords Infantile hypertrophic pyloric stenosis  Diagnosis  Complications

Introduction Most series of infantile hypertrophic pyloric stenosis (IHPS) usually involve a collection of cases from several pediatric surgeons. This creates a number of variations due to the different surgeons involved. This series, on the other hand, is the largest one treated by one pediatric surgeon (SHE) and extends over a period of time (35 years), longer than most of the other series. The main purpose of this paper is to report this large series with a focus on the diagnostic difficulties and complications.

S. H. Ein (&)  A. Ein Hospital for Sick Children, Toronto, ON, Canada e-mail: [email protected]

Methods and materials

P. T. Masiakos Massachusetts General Hospital, Boston, MA, USA

From July 1969 to December 2003 inclusive, both the hospital charts and the office charts of 791 babies with

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IHPS were retrospectively reviewed by the senior author (SHE) in reference to age, gestation, sex, weight, family history, racial background, other medical conditions and/or associated findings, investigations, diagnosis and false alarms, size of lump, incision, operation, antibiotics, associated procedures, complications and results. Most of the above information was also available from the 13 patients operated on elsewhere and subsequently transferred to HSC. All of these patients were examined and treated by the senior author and a surgical resident at the Hospital for Sick Children (HSC), Toronto, Ontario, Canada. Of the 791 patients, 778 (98.3 %) were operated on at HSC; the remaining 13 were not operated on: postoperative transfers (8), resolved (4), and focal alveolar hyperplasia (1). This review received HSC Research Ethics Board (REB) approval (1000012009).

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(Table 4). Fourteen (1.7 %) had concomitant procedures (gastrostomy 7, inguinal hernia repair 4, antireflux procedure 1, circumcision 1, release tongue tie 1). Three incisions were used: two types in the right upper quadrant until 1979 and then upper midline. Coincidentally, prophylactic antibiotics were instituted in 1982 which decreased wound infections from 9 to 3.9 %. There were a total of 87 (10 %) complications (Table 5) which included 9 (1.1 %) intraoperative (including mistaken diagnoses) (Table 6), 78 (9 %) postoperative: 59 (7 %) early (\1 month) (Table 7) and 19 (2 %) late ([1 month) (Table 8). There were 13 postoperative transfers (12 from outside general hospitals) with 16 (18 % of the total 87) complications after pyloromyotomy. There were two deaths (0.2 %) (Tables 4, 9).

Discussion Results Of the total 791 neonates and infants in this series, there were 647 (82 %) males and 144 (18 %) females. Their mean age at diagnosis was 38 days, median 51 (range 7 days–10 months). When ultrasonography (US) was routinely used after 1990, the average age at diagnosis decreased to \40 days (Fig. 1). The mean weight before and after routine US was 3.2 kg, median 3 (range 1.5–6). Twenty-five (3.1 %) were premature (\36 weeks gestation and/or weight \2.5 kg) at diagnosis. Their mean age at diagnosis was 49 days, median 56 (range 1–3 months); the mean weight was 2.5 kg, median 2.3 (range 1.5–3.2). Eighty-one (10 %) had a positive family history of IHPS, with the highest incidence (18) being twins (Table 1). Forty-four (5 %) of the 791 patients were non-Caucasians. Seventy-five (9 %) had other medical conditions, anomalies and/or associated findings with jaundice being the most common (15, 1.9 %) (Table 2). Fifty-one (6 %) pyloric sizes were abnormal (either by radiologic evaluation and/or subjectively by physical exam and intraoperatively): small 22, gritty (like scar tissue intraoperatively) 17, and large 12. There were 20 (2.5 %) ‘‘false alarms’’ (admitted with the clinical diagnosis of IHPS but investigations proved otherwise): feeding problem, gastrointestinal reflux (GER), infection, pylorospasm and/or other anomalies. Preoperative electrolytes were abnormal in 60 (7 %) with the highest pH of 7.58 and the abnormal mean: Na 120, K 2.6 and Cl 80 (meq/L) (Table 3). Ten (1.2 %) pyloric stenosis occurred after newborn operations (esophageal atresia 3, small bowel atresia 2, diaphragmatic hernia, fetus-in-fetu, imperforate anus, lung biopsy, malrotation 1) (Table 2). Of the entire total (791) who were treated, there were 13 (1.7 %) with IHPS who were not operated on (postoperative transfers 8, resolved 4, focal alveolar hyperplasia 1)

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Although Gross [1] and others [2–12] in the distant past called this anatomic finding in infants (and occasionally in newborns) ‘‘congenital’’, most authors on this topic in the 1980s, 1990s, and 2000s state that, with few exceptions, almost all patients with pyloric stenosis are not born with it. Therefore, their choice for the correct name of this problem is either ‘‘hypertrophic’’ [13, 14, 16, 19, 20, 22, 27, 28, 35, 38, 40, 42, 44, 45, 49, 50] or ‘‘infantile’’ [15, 17, 18, 21, 24–26, 29–34, 36, 37, 39, 41, 43, 46–48] pyloric stenosis; the latter seems to best describe this entity. Presently, a combination of ‘‘infantile hypertrophic pyloric stenosis (IHPS)’’ seems to be the most popular. In this series the 778 pyloromyotomies, (98.3 % of the total 791) done by the senior author, were 3.5 % of his total operative procedures over his 35-year practice from July 1969 to December 2003 inclusive; this was a mean of 22, median 27, pyloromyotomies/year (range 14–41). This 35-year review focuses on their diagnostic difficulties and complications. Diagnosis The incidence of four males: one female reported in the literature was confirmed in this review [31, 40]. The mean age of 38 days, median 51 (range 7 days–10 months), seems to best describe the age of the patients in this series [28]. This series included 25 (3 %) babies who were premature at diagnosis. In reviewing these 25 premature pylorics, their presenting age was a mean of 49 days, median 56 (range 1–3 months); at least 10 days later than the mean and median of the entire series (38, 51 days, range 7 days– 10 months). The majority of prematures were diagnosed with their pyloric stenosis close to, but still earlier than, their 36-week gestational age. On the other hand, their presenting weight was lower at an average of 2.5 kg,

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469

Fig. 1 Patient age at presentation. Note the decrease of age at diagnosis falls to below 40 days after 1990, when US examination became a routine part of the clinical diagnosis at HSC

HPS

160

140

Age (days)

120

100

80

60

40

20

0 1965

1970

1975

1980

1985

1990

1995

2000

2005

Year of Diagnosis

Table 1 Family history total 81 (10 %) 18 Twins 16 Single 2 Both 1 Down’s 17 Parents––1 couple [50 year 11 Father 6 Mother 1 Brain tumor 1 15 year 1 Drugs 16 Non-specific 6 Multiple family members 10 Brother 8 Uncle 5 Triplets 3 Single 2 All 3 Sister 2 Grandfather 1 Cousin

median 2.3, range 1.5–3.2. Aside from being diagnosed later, their morbidity was the same as the older bigger IHPS baby. It has been written that transpyloric feeding of the premature infant is one of several environmental factors associated with IHPS [17, 29, 39]. Neither this nor other environmental factors (breast feeding, erythromycin exposure), said to be similarly associated with IHPS, were found to be predominant in our premature pylorics. The most important thing to remember when dealing with a premature baby with symptoms and signs of IHPS is that small babies have ‘‘short segment pyloric narrowing’’ [51].

Aside from the above references to prematures, we could find few other references to this group of small babies with IHPS [52], except that younger (\3 weeks) babies with IHPS had a four times (32 %) greater family history [28]. The family history (genetic aspect) of IHPS is quite obvious, but varies in some aspects (Table 1). In this series, 81 (10 %) of our patients had a positive family history, with the highest incidence 18 (22 %) being twins (16 in single twins; 2 in both twins, 1 Down’s syndrome). Yang and co-authors [48] claimed that ‘‘Despite the lack of agreement as to whether the cause of IHPS is genetic, environmental, or both, the high concordance rate seen in twins is indisputable.’’ Of interest, there were also five sets of triplets in this series, but pyloric stenosis only occurred in two complete sets. Yesildag [53] reported four sets of triplets (1 invitro fertilization) in which all of the triplets had IHPS. In spite of the fact that our series showed that almost twice as many patients had a father, rather than a mother, with IHPS, the literature has stated that the reverse is true [6]. In 1953, Gross [1] wrote that ‘‘There is no racial predisposition to the condition.’’ Our incidence of 5 % non-Caucasians coincides somewhat with other similar reports in the literature which report a frequency of 1/3–1/5 that is seen in Caucacians [21, 28]. Mason [30] and O’Donoghue and their co-authors [34] report that the incidence in non-Caucasians is increasing. Most series of IHPS infants do not always (nor seldom completely) list the other medical conditions, anomalies and/or associated findings seen with their patients (Table 2); 75 (9 %) infants had other such occurrences. Jaundice (inconjugated, from diminished hepatic glucuronyl transferase activity and indirect hyperbilirubinemia) was the commonest (15, 1.9 %) in this series; it has also been reported in up to 5 % of similar babies in other papers

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470 Table 2 Other medical conditions, anomalies and associated findings total 75 (9 %)

Pediatr Surg Int (2014) 30:467–480 Table 3 Abnormal preoperative electrolytes total 60 (7 %)

15 Jaundice

Chloride 60 \ 95 Mean 80

14 Developmental delay

Range (52–94) Potassium

4 Down’s 3 Central nervous system, cerebral palsy

5 \ 3.0

3 Enzyme defect

Mean 2.6

2 Menkes syndrome

Range (2.3–2.9)

1 Chromosomal abnormality

Sodium

1 Fetal alcohol syndrome

3 \ 130

10 Congenital heart disease

Mean 120

10 Postoperative newborn 3 Esophageal atresia 2 Small bowel atresia 1 Diaphragmatic hernia

Range (117–127) All values (except pH) meq/l. Some values belong to same patients

pH Highest 7.58

1 Fetus-in-fetu 1 Imperforate anus 1 Lung biopsy

Table 4 Pyloric stenosis not operated on total 13 (1.6 %)

1 Malrotation

8 Postoperative transfers 3 Perforation

6 Respiratory 3 Bronchopulmonary dysplasia

2 Sepsis

1 Focal alveolar hyperplasia

1 Apnea (electrolytes)

1 Respiratory acidosis apnea

1 Recurrence 1 Vomiting

1 Respiratory Distress Syndrome 3 Genitourinary

4 Resolved (2‘–7 months)

1 Hypospadius 1 Non-functioning pelvic kidney

1 Focal alveovar hyperplasia (died in hospital)

1 Ureteropelvic obstruction 2 Cleft palate, Pierre Robin syndrome 2 Jehovah witness 2 Preoperative sepsis 2 Preoperative transfusion 1 Arthrogryposis

Table 5 Complications total 87 (10 %) 9 Intraoperative (1.1 %) 78 Postoperative (9 %) 59 Early (\1 months) (7 %)

1 Hearing impaired

19 Late ([1 months) (2.4 %)

1 Hypothyroid 1 Laryngeal cleft

16 (18 %) complications in 13 (1.6 %) postoperative transfers

1 Malignant hyperthermia 1 Meningomyelocele 1 Necrotizing enterocolitis 1 Thin (\3rd percentile)

Table 6 Intraoperative complications total 9 (1.1 %)

1 Ventriculoperitoneal shunt

6 Perforation 4 ? 1 Reoperationa 2 ? 2 Reoperation 3 Mistaken diagnosis 2 Normal

[21, 40]. The jaundice resolved spontaneously within 1 week of their pyloromyotomy. There are several papers debating the possible association (up to 6 %) of congenital genitourinary (GU) anomalies with IHPS [3, 15, 16], but this was only seen in three cases in this series (Table 2). In spite of this, ten babies who underwent surgical repair of their congenital anomalies (none of them involving the GU system), while recovering from their newborn operation, developed IHPS requiring pyloromyotomy (Table 2) [54].

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a

Postoperative transfers

1 Duodenal duplication

Although cystic fibrosis (CF) was reported [25] to have a nine times greater incidence (2.7 %) in babies with IHPS than the general population (0.3 %), CF was not present in any of our babies. A Department of Pediatric Radiology study showed that the ‘‘clinical skill of the examiner (and)…the size of the

Pediatr Surg Int (2014) 30:467–480 Table 7 Early (\1 month) postoperative complications total 59 (7 %)

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([90 % Vomiting) 46 (5 %) Wound infection 4 Incomplete pyloromyotomya 4 Reoperation 2 Bleedinga 2 Sepsisa 1 Apnea (electrolytes)a 1 Deatha 1 Slow to open up (10 days) 1 Vomiting

a

Postoperative transfers (1 patient had 3; 1 patient had 2)

1 Wound dehiscence

Table 8 Late ([1 month) postoperative complications total 19 (2.4 %) 13 Gastroesophageal reflux 7 Operation (1‘ months–13 months) 6 No operation (6 months–12 years) 2 Recurrence 1 Redo pyloromyotomy 1 Dilatationa 2 Slow gastric emptying (2 years, 9 years) 1 Gastric ulcer (14 months) 1 Ventral hernia a

Postoperative transfer

Table 9 Postoperative transfers total 13 (1.6 %) Operation

Complicationsa

4

/ 4 Incomplete pyloromyotomy

1

/ 4 Perforation ?

3

2 Bleeding ?

2

2 Sepsis ?

2

1 Apnea (electrolytes) ?

1

1 Death ?

1

1 Recurrence ? 1 Vomiting ?

1 1

Total 16a

Total 11 (8 patients)a

Total 5 (5 patients) a

No operation

1 Patient had 3; 1 patient had 2

hypertrophied pylorics determine and affect the clinician’s ability to palpate the pyloric mass’’ [28, 35]. Size and texture of the pyloric lump (tumor, olive) have received some attention in the literature, although only 51 (6 %) of the pyloric lumps in this series were deemed abnormal in size and/or texture either by radiologic evaluation and/or subjectively by preoperative physical exam and intraoperatively. While somewhat subjective, these extreme pyloric

lump sizes certainly do exist and pose problems in both diagnosis and treatment. Generally speaking, we found that in our series there was no correlation between the size of the pyloric lump and the symptoms of the baby. The only two studies that we are aware of that actually defined the sizes of the pyloric lump were from Spain in 2005 [19] and Pittsburgh in 2008 [28]. The former study measured the operative size of the pyloric ‘‘olive’’ in 145 consecutive cases and reported: small \20 mm (14 %), medium 21–30 mm (51 %), large [31 mm (35 %); they concluded that pyloric olive size correlated with age, weight and duration of vomiting. In the latter 2008 study, the pyloric channel length increased from 15 mm in the babies \14 days of age to 19 mm at 36–42 days. In our series, the small pyloric lump was 43 % of our abnormal sizes and textures and it increased the difficulty of making the diagnosis both clinically and radiologically, in spite of the accepted fact that younger babies as well as small babies have small pylorics [51]. The description (diagnosis) of a ‘‘gritty’’ pyloric lump was made in 17 of our patients and was noted only at operation, when the pyloric lump was incised and felt like scar tissue. The myotomy split was usually done with some difficulty, although successfully in all 17 cases. It was always associated with an older pyloric that had been present longer than the usual time. While the extreme sizes and unusual texture of the above pyloric tumors made the operative pyloromyotomy more difficult, there were no mucosal perforations and no postoperative problems. In spite of the fact that ‘‘In well over 95 percent of cases, the pyloric ‘tumor’ can be felt through the abdominal wall…’’ [1], in a 2008 study, the pyloric olive was less likely (30 %) to be palpated in younger (\21 days) newborns than in older ([22 days) (70 %) [28]. Nowadays, there is a trend toward increased reliance on imaging as a substitute or complement to physical examination [14]. Furthermore, Cosper et al. [55] reported that ‘‘Surgeons who have undergone focused training to perform (US) ultrasonography for pyloric stenosis can diagnose the condition without confirmatory testing by a Radiologist’’. To complement and confirm this, a 2004 study from a Pediatric Radiology Department concluded that ‘‘No significant change in imaging volume occurred following initiation of a guideline which recommended clinical evaluation for palpation of the olive prior to ordering imaging studies’’ [20]. Nonetheless, there was a threefold increase in the use of imaging between the 1970s and the late 1980s, with a concomitant decrease in cases diagnosed by palpation from 87 to 49 % [56]. Schwartz in 2006 [40] stated that ‘‘a definitive diagnosis can be made in 75 % of infants with IHPS by careful physical examination of the upper part of the abdomen, but this is becoming a lost skill… Eighty-two percent of pediatric surgeons would

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proceed with an operation with a positive US study even if the results of physical examination under anesthesia were negative… This indicates that US has essentially supplanted physical examination as the diagnostic test of choice for IHPS’’. Suffice it to say, each of the patients in this series was examined by the surgical team and, with very few exceptions, a pyloric lump was clinically palpable. Even if this was not accomplished, and the diagnosis was established by radiologic means, under general anesthesia, more often than not, the pyloric lump still could not be palpated. In such instances, the baby was almost always larger than normal and the diagnosis of IHPS was confirmed at operation. In two of the three instances in this series in which the pylorus turned out to be normal at laparotomy (false positive), all three pyloric ‘‘lumps’’ were felt preoperatively (misdiagnosed) to be ‘‘small but consistent with IHPS’’; moreover, in two of the three cases, their diagnosis had also been confirmed by US (in one) and barium upper gastrointestinal investigation (UGI) (in the second). Therefore, to avoid the possibility of a falsepositive normal pylorus being found at laparotomy, some surgeons have resorted to intraoperative endoscopy for confirmation of their diagnosis [57, 58]. In a study of 150 pyloric patients, ‘‘olives were appreciated in 111 (74 %) with one false positive (0.7 %) and no false negatives; sonography and UGI were equally accurate’’. The authors’ conclusion was that ‘‘Although highly sensitive, imaging is superfluous if an olive is palpable’’ [44]. Nonetheless, US has become the most common imaging technique for the diagnosis of IHPS and is now the gold standard [40, 55]. Ultrasonographic diagnosis of IHPS was first described in 1977, but there continues to be some debate about what the exact pathological pyloric measurements should be. In 2008, Leaphart et al. [28] said that ‘‘It is now generally accepted that muscle thickness (MT) [4.0 mm and pyloric channel length (CL) [15 mm are consistent with the diagnosis of IHPS, with presenting between 3.0 and 4.0 mm as ‘borderline’’’. However, these measurements are from term newborns presenting at 4–6 weeks of age. Two paragraphs later in the paper, the authors then wrote that ‘‘Standard controls for normal measures of pyloric MT and CL were 3 and 15 mm respectively…’’. In 1994, Hernandez and Schulman [22] wrote that accuracy approaches 100 % in experienced hands; the same authors stated 9 years later (2003) that ‘‘the actual numeric value (of the length of the hypertrophied canal and the muscle thickness) is less important than the overall morphology of the canal and the real-time observations… The unequivocal diagnosis of IHPS is made by identifying the hypertrophied pylorus; in these patients, the muscle thickness, although variable during the examination, will be three mm or more throughout the examination’’ [21]. Generally speaking, most radiologists seem more concerned, especially in

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smaller babies about how the hypertrophied pyloris looks and acts (‘‘dynamic assessment’’ [23]) more than its exact measurements, supplemented by the relationship between the pyloric channel and weight [22, 24]. At HSC, during the later years of this study, the Diagnostic Imaging Department generally used a length of 15 mm and a thickness of 3–5 mm as their radiologic standards for IHPS. The first US that was done in this series was in 1979; prior to that, all imaging for IHPS was done by barium UGI series. After 1979, the latter radiological investigation was slowly replaced by US; when it was routinely used at HSC (1990), the age at diagnosis decreased to\40 days (Fig. 1). Preoperative serum electrolytes were abnormal in 60 of our patients (7 %) (Table 3), but similar results from other series have rarely been available for comparison. These results, while they were the mean for all our patients with abnormalities in their electrolytes, seem somewhat extreme; however, these babies often presented late and were quite sick and dehydrated enough to produce these severe electrolyte disturbances. The electrolyte results of Chen et al. [59] with their IHPS patients in 1996 showed ‘‘metabolic alkalosis or acidosis (10 %), hypokalemia (5 %), hypochloremia (3 %) and dehydration (9 %),’’ which compares equally with our patients. We found the plasma (serum) chloride (Cl) to be the most reliable electrolyte for the assessment, monitoring and correction of the preoperative hypochloremic alkalosis during the diagnosis and resuscitation in IHPS [60]. In infants, a plasma Cl concentration of 90 meq/L has been suggested as normal [8]; however, it was found in one series that 38 % of infants with a Cl concentration of 90 meq/L still remained alkalotic [27]. Uncorrected alkalosis may cause preoperative apnea and may also delay recovery from anesthesia and cause postoperative apnea [41, 61]. In our series, there were three patients with abnormal electrolytes who had apnea due to the compensatory respiratory acidosis from their metabolic alkalosis: the first was in 1974, when a healthy baby boy who had an apparently uneventful pyloromyotomy for pyloric stenosis under general anesthesia at a general hospital by an adult general surgeon and arrested 2 h postoperatively. His admission chlorides were 78 but were only improved to 85 before operation. He had an unsuccessful resuscitation and died the next day from disseminated intravascular coagulopathy (DIC). The second case of apnea was in 1988 and was preoperatively; this baby did not require intubation, and the pyloromyotomy several days later went well. The third was also a postoperative transfer of an infant whose metabolic alkalosis was not adequately corrected. We required the chlorides to be at least at 90 meq/L. Some have recommended that an appropriate target for plasma Cl concentration be at least 106 meq/L [62]. A plasma Cl concentration [105 meq/L corresponds to a urine Cl concentration[20 meq/L and the

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latter may be used to confirm that the extracellular fluid volume is restored. Various intravenous fluid regimens have been described in the literature, including correction with 0.45 % saline, 5 % dextrose and added potassium [14]; however, a 2008 practice was to use 0.45 % saline, 10 % dextrose and 10 mmol of potassium at 170 mls/ kg day [45]. The latter authors found that with their standardized fluid regimen, the rate of correction of the electrolyte derangement in IHPS followed a direct linear pattern and the time to correction could be predicted [45]. Nowadays, the relatively low incidence of presenting electrolyte abnormalities confirms the earlier presentation and diagnosis of babies with IHPS as was also observed in our series, certainly aided using US. Two studies, 30 years apart, also observed that with earlier diagnosis, they were encountering fewer patients who were in a state of severe dehydration, alkalosis and malnutrition [5, 62]. There is no doubt that ‘‘the clinical presentation varies with the length of symptoms’’, [21] as well as ‘‘the number of episodes of postoperative emesis and time to goal feeds’’ [63]. The 60 (7 %) patients in this series that had preoperative electrolyte imbalance with the coexisting alkalotic dehydration were diagnosed later and vomited longer. For that very reason, Chen et al. [59] claim that ‘‘ultrasonographic confirmation is obtained in the majority of cases, often before clinical evaluation by the surgeon… Because so little time had elapsed, water and electrolyte imbalances were not present, and the patients could be operated on within hours of admission’’. In spite of the above philosophy of early operation, we feel that the emergency is over, once the diagnosis has been made; there should be no rush to do an immediate pyloromyotomy. More often than not, the IHPS baby has been seen several times by his/her pediatrician, family doctor, walk-in clinic and/or all three before the correct diagnosis was made. This was often enough to exhaust the baby, the mother and the family. This, as well as the occurrence of almost 100 % postoperative vomiting, may be the reasons why the immediate operative and outpatient surgery treatment of IHPS have never gained popularity at HSC. As it is, one study of 100 IHPS patients during 1986 and 1987 showed that the time from diagnosis to the time of surgery was 23 ± 12 h (range 2–72 h) [62]. As expected, there was a delay in recognizing the ten babies who presented with pyloric stenosis after their newborn surgery [esophageal atresia (3), small bowel atresia (2), diaphragmatic hernia, fetus-in-fetu, imperforate anus, lung biopsy, malrotation (1)] [54]. Their mean gestational age was 36 weeks, birth weight 3.2 kg, and age at newborn operation was 2 days. Their initial postoperative feeds started on the sixth day (mean), and the pyloric stenosis vomiting started on the 14th day. It took a mean of 12 days to make the diagnosis of pyloric stenosis, and this was done entirely by diagnostic imaging. Mean age at

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pyloromyotomy was 3‘ weeks. A new incision was used in seven patients. Postoperative pyloric feedings started on the first day; all did well. There were three complications (bowel obstruction), all requiring operation. Although nonoperative management was never the intended mode of therapy for babies with IHPS in this series, there were 13 (1.6 %) babies with clinically and radiologically proven IHPS who were not operated on (Table 4); none were treated with atropine [12, 14, 42, 47]. Eight infants with 11 postoperative complications were postoperative transfers and were also managed nonoperatively (Table 9). Four babies presented later with a mean age of 5 months (range 2‘–7) and with a longer than usual history, but with less projectile vomiting than the typical IHPS; by the time the diagnosis was made by history, exam and confirmatory radiologic studies, their symptoms and signs were decreasing enough to postpone their pyloromyotomy and all four continued to improve and did well without surgery. One may speculate that if the wait is long enough, the IHPS muscle thickness will become normal again. Muramari et al. [33] reported that the normal muscle wall thickness (approximately 2 mm) returns within 8 months and the diameter (10 mm) within 1 year; however, the channel length decreases more rapidly than the other two parameters. These authors concluded that ‘‘the morphologic resolution of the pylorus after surgery did not correlate with the prompt improvement of symptoms’’ [33]. The fact that these four unoperated patients’ clinical pictures were improving enough for surgical non-intervention to be continued showed that there indeed is ‘‘a slow decrease (in muscle thickness) that reaches normal thickness (\3 mm) by 5 months’’ [49]. In our series, one baby girl was diagnosed with irreparable focal alveolar hyperplasia before her diagnosis of IHPS was made, but pyloromyotomy was not an option. Once again, our search of the literature failed to find any discussion of the above situations, as specific, albeit rare, entities. Of the 778 patients who had a pyloromyotomy at HSC, all were operated on under general endotracheal anesthesia with muscle relaxation and via a small laparotomy incision. The actual pyloromyotomy incision was made with a # 15 scalpel blade from the antrum to the pyloric vein of Mayo at the pylorus for at least 2 cm. The pyloric split (spread) was carried out with a pyloric (Benson) spreader making certain that it extended proximally from the normal inner circular muscle layer distally to the pyloric vein of Mayo to allow the redundant duodenal mucosa to bulge out. The usual excess air in the dilated stomach was squeezed into the duodenum to check for a mucosal perforation. This was the operative routine that the senior author was taught and it did not change throughout his practice. The last operation he did in his practice was a pyloromyotomy. Nasogastric tubes were not routinely used either pre or postoperatively.

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Very rarely, when vomiting postoperatively was both large in amount and mucoid, a nasogastric tube was carefully passed into the stomach (to wash it out with lactated Ringers’ solution, which is mucolytic) and then removed. Early in this series postoperative feeding followed a routine Division protocol of slow progression (if no vomiting) to full feeds over a few days, to minimize postoperative vomiting. When it was realized by both the nursing and surgical staffs that whatever routine (or not) was used did not seem to make any difference, for most of this series, all the pyloromyotomy babies were fed (by bottle and/or by breast) ad lib Q4H starting at 6 h postoperatively. Discharge was allowed when feeds reached 45–60 cc Q4H with only the occasional vomiting. It was observed that once ad lib feedings were instituted, the length of hospital stay decreased from postoperative days 2–3 to postoperative days 1–2. There were five other procedures (gastrostomy 7, inguinal hernia repair 4, circumcision 1, antireflux procedure 1, tongue tie release 1) done concomitantly with 14 (1.7 %) of our pyloromyotomies; this did not hamper their overall recovery in any way. The gastrostomies were all done in babies with significant developmental delay who were unable to attain the majority of their feeds by mouth. Another newborn was diagnosed both clinically and radiologically as having significant GER and in spite of aggressive medical treatment in the neonatal intensive care unit (NICU), there was no improvement. At operation for an antireflux procedure, to everyone’s surprise, a classic IHPS lump was found. It was decided to proceed with the planned antireflux procedure along with a pyloromyotomy. To the embarrassment of the radiologists, the pylorus had never been imaged once the GER was noted; a good teaching lesson for all doctors investigating vomiting in a baby or infant. The above paragraphs show that IHPS can occur in infants of any age and size from any racial and/or familial background with or without any medical condition, anomaly and/ or associated finding. It can also be mimicked by anything that causes vomiting. The diagnosis can be made in several ways, depending upon the size of the patient and the pyloric lump, as well as the presence or absence of any other associated problems. As with other diagnoses in medicine, if one does not think about them, they are often overlooked or forgotten about. The follow-up was almost 100 % with a minimum of 1 month and was continued until the infant was back to normal in all respects. No attempt at a longer follow-up was made; however, it was expected that if there was a problem, thought to be related to the pyloromyotomy, the patient would return to the original surgeon. This seemed to be the best that could be achieved under the circumstances. Suffice it to say, in Toronto (population, 3 million) and Ontario (10 million), the pediatric referral patterns and patient base

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mostly relate to HSC. Moreover, if one of the original pyloromyotomy patients had another problem or was operated on elsewhere, more often than not, that information would be transmitted back to the HSC surgeon [64]. Complications One must be cautious in discussing complications (Table 5) because they mean different things to different authors, especially when dealing with pyloromyotomy. Gross [1] only mentioned ‘‘perforating the muscosa’’ under ‘‘complications’’ and later ‘‘mortality’’ and ‘‘incomplete (pyloromyotomy)’’ under ‘‘results of therapy’’. The longer the duration of obstructive symptoms in IHPS does not seem to have a significant impact on postoperative complications [13]. In reviewing our 87 (10 %) total complications (Table 5), we included 9 (1.1 %) intraoperative (including mistaken diagnoses) (Table 6); 78 (9 %) postoperative, 59 (7 %) early (\1 month) (Table 7) and 19 (2.4 %) late ([1 month) (Table 8). The results seem to match favorably with complications reported in other series [1, 14, 38, 40, 46, 65–67] although very few series calculated and reported their total complications as completely as we have. Thirteen (1.6 %) of the 791 patients were postoperative transfers (12 from non-pediatric surgeons) and they had 16 (18 %) of all the complications (Table 9). The commonest intraoperative complication is a perforation (Table 6). In our series, of the nine (1.1 %) intraoperative complications, six (0.7 %) were perforations, but only two of these six belonged to the HSC 778 patients of this series and both occurred proximally on the pyloric lump. Our teaching has been that most incomplete myotomies are a result of failure to extend the myotomy far enough proximally onto the antrum until one sees the normal inner circular muscle fibers. These two proximal perforations were closed with interrupted 4–0 silk sutures for the mucosa covered with a (Graham) patch of omentum [1]. Gross [1] stated (and it is still generally believed) that the commonest area of perforation is distally on the duodenal side of the pyloromyotomy, where the pyloric musculature ends abruptly and the redundant duodenal mucosa tends to double back. This latter perforation can be prevented by not carrying the distal part of the myotomy past (distal to) the pyloric vein of Mayo. Furthermore, Gross [1] stated that ‘‘it is our custom to keep the baby on constant gastric suction for twenty-four hours after operation before starting any feedings’’. In all the cases of perforation in this series, the nasogastric tube was kept in place for 5 days, and before it was removed, a contrast study was done to make sure that the perforation was closed. If a peritoneal drain was present, it was slowly removed after a normal contrast X-ray. The rationale for this approach was the same as for the closure of a perforated duodenal ulcer. As a

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matter of fact, one of the four perforations that were transferred to our care postoperatively also had an intraperitoneal drain, which was not such a bad idea since their closure of the distal myotomy perforation leaked, but the leakage was captured by the drain. The four intraoperative myotomy perforations that were transferred to HSC were all done by non-pediatric surgeons. One of these required reoperation and re-closure as described above; the other three were treated nonoperatively because their closure was done when recognized intraoperatively before their transfer. All six babies with the perforations recovered well with no undue sequelae. There were three patients who also had five early complications (2 bleeds, 2 with sepsis and 1 death). These combinations are almost always associated with a mucosal perforation at the pyloromyotomy site that is either closed and leaks, or is missed. In a district general hospital (with non-pediatric surgeons) a perforation rate of 19 % was considered acceptable after open pyloromyotomy, because the perforations were immediately recognized and no morbidity resulted [14]. This approach seems worrisome. However, a 1996 study [66] compared the perforation rate of 12 % by non-pediatric surgeons with 0 % by a pediatric surgeon and supported the recommendation that infants with IHPS should be managed by a pediatric surgeon, or by a non-pediatric surgeon with experience with this procedure. The six (0.7 %) perforations in our entire series compare favorably with, if not better than, the mean of 4 % (range 0–6 %) from another series of six authors’ 600? cases [40], and with 39 (4 %) perforations from 901 infants from two pediatric surgical centers over a 25-year period (1969–1994) similar to the same time line of this paper [14]. Also considered as an intraoperative complication, three (0.3 %) mistaken diagnoses were noted at the time of operation (Table 6). All three were initially diagnosed as IHPS at HSC with a combination of clinical exam, US, and UGI. In spite of the above, at operation, the pylorus was normal; two had no other pathology, but to ensure that a small IHPS was not missed, a pyloromyotomy was done. The third infant had a duodenal duplication which obstructed the duodenal sweep just distal to the pylorus and was unroofed (rather than excised, which would have required a Whipple procedure). All three infants did well postoperatively. These three mistaken diagnoses show that all diagnostic modalities offer an 80–90 % guarantee of a correct diagnosis, but it has yet to be determined if any combination will make the correct diagnosis 100 % of the time. A mistaken diagnosis was not discussed in many recent chapters and papers on IHPS [1, 14, 17, 19–21, 38, 44, 46, 59, 65–67]. It is quite possible that, as a last resort, if confirmation of IHPS cannot be confirmed, intraoperative endoscopy may provide the definitive answer [57, 58].

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Many series report postoperative vomiting as an early (\1 month) complication (Table 7), but [90 % of the 778 HSC patients vomited for 1–3 days afterwards and as such, this vomiting was not considered a true complication. Under ‘‘Complications’’ Schwartz [40] said that ‘‘Vomiting, frequently in the early postoperative period, is thought to be secondary to gastroesophageal reflux or discoordination of gastric peristalsis or gastric atony and should not be considered a complication.’’ The closest occurrence to an incomplete myotomy in this series (in spite of all 778 babies having the exact same supervised anatomical pyloromyotomy) was a baby who vomited for 10 days, before settling down without any further feeding problems. One of the 16 postoperative transfer complications was vomiting, but it was excessive and worrisome enough to the referring non-pediatric surgeon to warrant the transfer. No specific cause was found for this postoperative vomiting and it settled down in a few days with intravenous therapy and no oral feeding. It is not difficult to conclude that vomiting, secondary to an obstructed pylorus, can be easily misconstrued as primary GER, but in the vast majority of cases all the vomiting disappears after pyloromyotomy. It is obvious that other authors (from 1999 to 2005) also seem unclear about the actual occurrence of postoperative vomiting, reporting their incidence from 3 to 60 % with no mention as to its primary or secondary origin [14]. The commonest early postoperative complication in this series was a wound infection and there were 46 (5 %) of them. Seventy-seven percent of these infections was caused by the common skin flora (Staphylococcus epidermidis) as also reported from several other pediatric centers [32]. This wound infection rate is larger than desired, especially for a clean operation; the senior author’s inguinal hernia infection rate (for comparison) over the same 35-year time span as this series was 1.2 % [64]. Nonetheless, this series’ pyloromyotomy wound infection results compare with the mean wound infection rate of 4 % (range 0–7 %) for open pyloromyotomies in five series (1995–2003) [40]. In our series, three incisions were used for the 778 consecutive pyloromyotomies: 1.

2.

1969–1979 (231 pyloromyotomies): right upper quadrant (subcostal gridiron muscle-splitting lateral to rectus––(Robertson), and transverse rectus dividing or muscle splitting); the number of infections was 21 (9 %). 1980–2003 (547 pyloromyotomies): upper midline. The senior author found that this was an easier incision to teach from; both the pyloric lump and the stomach can be more easily and safely brought out of the abdomen to do the pyloromyotomy. The number of infections was 25 (4.5 %).

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In contrast to the above, in 2008, umbilical pyloromyotomy is used by most pediatric surgeons in the United Kingdom and Ireland, while the right upper quadrant incision is now used by a minority of surgeons; [32, 69] laparoscopy is increasingly popular in minimally invasive centers. All wounds in this series were closed with a synthetic suture (Dexon1, Vicryl2) with a subcuticular closure of the skin from 1969 to 1987; there were 391 pyloromyotomies with 40 wound infections (10 %). In 1987–2003, the skin incision was made with the cutting cautery and staples closed the skin, which was then infiltrated with a local anesthetic; there were 547 pyloromyotomies with 25 wound infections (4.5 %). Podevin [70] showed that these three different incisions (right upper quadrant and midline/ umbilical) had the same wound infection rate, but van den Ende [46] revealed a three times greater wound infection rate for the right upper quadrant incisions. Our series had a two times greater wound infection rate for the right upper quadrant incision. In this series, there were two eras of prophylactic antibiotics (non-use and use) for our 778 consecutive pyloromyotomies: 1.

2.

1969–1981 (272 pyloromyotomies): no prophylactic antibiotics; the number of wound infections was 26 (9 %). 1982–2003 (506 pyloromyotomies): prophylactic antibiotics (cloxacillin, ancef); the number of wound infections was 20 (3.9 %). Cloxacillin was used from 1982 to 1987 (141 pyloromyotomies): the number of wound infections was 16 (11 %). Ancef was then used from 1988 to 2003 (365 pyloromyotomies): the number of wound infections was four (1 %).

This remains both surprising to, and unexplained by, the authors of this paper. Considering that the routine use of US at HSC occurred around 1990, which produced an earlier diagnosis of pyloric stenosis, (along with possibly less repeated abdominal examinations), as well as the institution of routine prophylactic antibiotics in 1982, it is open to speculation which of the above, or both, contributed to an overall decrease in postoperative wound infections in this series. Antibiotic prophylaxis is now common with only the umbilical incision, since it carries a 7 % increased infection risk. Nonetheless, more than 50 % of pediatric surgeons presently do not routinely use prophylactic antibiotics regardless of their choice of incision [32]. For pain relief, we started infiltrating 2 cc of 1/4 % local anesthesia (bupivicaine without adrenaline) around the upper midline incision in 1987. This may well have decreased the need for anything more than acetaminophen 1 2

Polyglycolic acid, Davis & Geck. Polyglactin 910, Ethicon.

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Table 10 Redo pyloromyotomy total 7 (0.8 %)

4 Incomplete 4 Reoperationa 2 Recurrent 1 Reoperation 1 Dilatationa 1 Perforationa

a

Postoperative transfers

Reoperation

for pain relief; narcotics were seldom required in this series. Habre et al. [71] similarly concluded that there was generally a low consumption of analgesics after pyloromyotomy because infiltration of the wound with local anesthesia delayed the need for, and time to administer, the first postoperative analgesia. Aside from acetaminophen for postoperative pain a few times, these babies are quite comfortable postoperatively and are more intent on, and content with, feeding, some vomiting not withstanding. Of all the serious complications of pyloromyotomy, next to death and intraoperative perforation, in our estimation, redo pyloromyotomy ranks third. In this series of 791 pyloromyotomies, there were seven (0.8 %) that required reoperation in the form of a second (redo) pyloromyotomy (Table 10). The commonest reason for a redo pyloromyotomy in our series, and in others, was an incomplete pyloromyotomy. This did not happen with any of the 778 HSC patients in this series, but 4 of the 12 postoperative transfers from non-pediatric surgeons had an incomplete pyloromyotomy, all of which required reoperation. Incomplete (open) pyloromyotomy has been reported to vary between 0 and 1.9 %; [14] the series from Boston Children’s Hospital by Gross [1] from 1915 to 1952 with 1,787 cases of IHPS had only four (0.2 %) incomplete pyloromyotomies. There was one wound dehiscence, which occurred when a synthetic polygycolic acid 2–0 suture was found to be broken in the middle of a running one-layer closure of an upper midline incision. The cause of this could not be determined. This happened in 1985 and, from then on, all closures were done in an interrupted fashion to avoid another similar occurrence. Postoperative pyloromyotomy wound dehiscence of 6 % was reported from a 10-year experience of non-pediatric surgeons in a large general hospital; when the management of IHPS was taken over by a pediatric surgeon in the same general hospital, the wound dehiscence rate was 0 % [66]. Although Podevin et al. [70] claimed that wound dehiscence in their patients was not different in incidence regardless of the right upper quadrant or umbilical incision, we could not find any series in which the upper midline incision was used for pyloromyotomy as in our series. There were two deaths (0.2 %) out of the entire 791 patients. The first, in 1974, (which was possibly avoidable),

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was a 34-day-old 3.5-kg boy who apparently had an uneventful pyloromyotomy for pyloric stenosis under general anesthesia at a general hospital by an adult general surgeon. His admission chlorides were 78, which were corrected only up to 85 before operation. Two hours postoperatively he arrested and was thought to have aspirated. It took 30 min of resuscitation to obtain a heartbeat after which he was transferred to the HSC ICU intubated with bilateral pneumothoraces. Upon arrival at HSC, bilateral chest tubes relieved the pneumothoraces; his abdomen was softly distended with decreased bowel sounds and on X-ray, there was an ileus pattern with fluid between bowel loops. He was on a respirator and his neurological function was in question. He was bleeding from everywhere and his blood work indicated DIC. The latter was felt to have developed from his prolonged postoperative cardiac arrest which produced severe respiratory acidosis, hypovolemia, hypocirculation, hypothermia and shock. Sepsis was also suspected as a possible cause of the DIC and the appropriate antibiotic therapy was given. It was felt that surgery for possible postoperative intraabdominal bleeding was not feasible. With aggressive therapy, no improvement ensued and he died the next day. There was no autopsy. The second death in 1991 was in a 1-month-old 3.2-kg girl with biopsy proven focal alveolar hyperplasia, who soon thereafter developed pyloric stenosis. Because of this irreparable lung pathology, pyloromyotomy was not an option and comfort care was given until her death within a few weeks in hospital. Hernandez– Schulmann [21] said that ‘‘Mortality for babies with pyloric stenosis reflects one of the great success stories in modern medicine… In the United States prior to 1904, the mortality was 100 %. Within 10 years of the advent of the Ramstedt procedure, published mortality rates for this operation had decreased to 10 %; this rate further diminished to 2 % by 1931 and is well below that value today.’’ By 1952 Gross [1] reported a mortality rate of 0.6 %. This series was 0.2 %. There were 19 (2.4 %) late ([1 month) postoperative complications (Table 8). Thirteen of these 19 patients (1‘ months–12 years) developed significant vomiting and were diagnosed with GER. We defined ‘‘significant’’ GER if their symptoms lasted more than 1 week, were proven by contrast study, ph probe study, and/or esophagoscopy and required medication. Of these 13 infants and children, seven (1‘–13 months) required antireflux surgery and six (6 months–12 years) required only medical treatment for a few weeks. There were two rare true recurrent IHPS babies, one of which required reoperation (Table 10). Both recurred 8 weeks after an initial successful pyloromyotomy was done and the infants were discharged home on regular feeds, which they tolerated for 1 month before they

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suddenly developed a rare recurrent IHPS [72]. In spite of 2 weeks of medical treatment, including two attempts at intraluminal balloon dilatation by interventional radiology (IR), one required a redo pyloromyotomy (below the previous pyloromyotomy site which had healed over with scar); he recovered well. The second recurrent IHPS (who was a transfer from another pediatric general Surgeon at HSC) was successfully dilated by IR. Both remain well long-term. The third reason for a redo pyloromyotomy (after incomplete and recurrent) was also a transfer from a nonpediatric surgeon who perforated the pyloromyotomy, as well as doing it incompletely; the perforation in the distal part of the initial pyloromyotomy was closed and the original incomplete pyloromyotomy was completed proximally in the usual fashion. All seven redo pyloromyotomies did well. For some strange reason, two of the pyloromyotomy patients in this series returned with the sudden onset of symptomatic slow gastric emptying at the age of 2 and 9 years. This was confirmed with contrast UGI series which showed no anatomical abnormality at the previous pyloric stenosis area; this was successfully treated with a prokinetic (Maxeran, Metachlopramide) and disappeared after a week or two. The authors have not seen the equivalent situation mentioned during their review of the literature or do we have an explanation for this unusual occurrence; it may not even have been related to the previous pyloromyotomy of infancy. Both remain well longterm. Although there have been scattered reports suggesting that peptic ulcers are more likely to develop in patients with successfully treated pyloric stenosis [18, 36], this happened only once in this series (0.1 %). A 14-month-old developed a symptomatic gastric ulcer, which was treated medically with no further sequelae. The cause for this, other than chance, remains a mystery. He has remained well. In this series, there was only one ventral (incisional) hernia which was seen in follow-up several months after an uneventful pyloromyotomy. This occurred in 1985 through a right upper quadrant transverse muscle-cutting incision, which had healed without an infection. The repair was closed in running layers of polyglycolic acid 2–0 suture material. After a few months, the ventral hernia healed without surgical intervention. Interestingly, once the upper midline incision was routinely used in this series, many parents questioned an upper midline incisional bulge postoperatively as a possible ventral hernia. We felt it was the very common midline bulge from diastasis recti, which in all such cases disappeared after 6 months of age. Very few pyloromyotomy series reported ventral hernias, but one series (256 patients) had a 1 % incidence after a 6 %

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wound infection rate [46] in mostly right upper quadrant incisions. Sixteen (18 %) of the 87 complications occurred in 13 (1.6 %) patients who were postoperative transfers (Table 9); 12 of these 13 infants were from non-pediatric surgeons [73]. Eleven (69 %) of these transfers had 16 complications which did not require reoperation (including 1 death); five (31 %) needed reoperation. In spite of the fact that in a 2006 survey only 27 % of more than 11,000 pyloromytomies were done in children’s hospitals [74], 92 % of pediatric surgeons believe that pyloromyotomy should be performed only by pediatric surgeons [75]. Nonetheless, a 2000 study by Ly and co-workers [76] showed that higher surgeon volume of pyloromyotomies ([14 per year) was associated with fewer complications, regardless of the specialty training of the surgeon. The lessons learned from this large series can be summarized as follows. The emergency should be over when the diagnosis is made. The baby must have its dehydration, fluid balance and electrolytes back to normal before any operation is scheduled. The operation should not be done at night and both the anesthesiologist and surgeon must be experienced in, and capable of, treating infants with this problem, as well as the nursing staff on both sides of the operation. Intraoperatively, it is essential that the pyloromyotomy extends from the pyloric vein of Mayo proximally to the normal inner circular muscle fibers of the antrum. The postoperative feeding regime does not seem to be important since they all vomit after the operation, whether they are fed or not, or even how or what they are fed, but they all stop.

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The ins and outs of pyloromyotomy: what we have learned in 35 years.

The aim of the study is to evaluate a large series of infantile hypertrophic pyloric stenosis (IHPS) patients treated by one pediatric surgeon focusin...
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