WordNet
- abnormal narrowing of a bodily canal or passageway (同)stricture
- relating to or near the pylorus
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/08/05 10:33:44」(JST)
wiki en
| Pyloric stenosis | |
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Outline of stomach, showing its anatomical landmarks, including the pylorus.
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| Classification and external resources | |
| Specialty | General surgery |
| ICD-10 | K31.1, Q40.0 |
| ICD-9-CM | 537.0, 750.5 |
| DiseasesDB | 11060 29488 |
| MedlinePlus | 000970 |
| eMedicine | emerg/397 radio/358, article/929829 |
| MeSH | D046248 |
Pyloric stenosis or pylorostenosis is narrowing (stenosis) of the opening from the stomach to the first part of the small intestine known as the duodenum, due to enlargement (hypertrophy) of the muscle surrounding this opening (the pylorus, meaning "gate"), which spasms when the stomach empties. This condition causes severe projectile non-bilious vomiting. It most often occurs in the first few months of life, when it may thus be more specifically labeled as infantile hypertrophic pyloric stenosis.[1] The thickened pylorus is felt classically as an olive-shaped mass in the middle upper part or right upper quadrant of the infant's abdomen. In pyloric stenosis, it is uncertain whether there is a true congenital anatomic narrowing or whether there is merely a functional hypertrophy of the pyloric sphincter muscle. This condition typically develops in male babies in the first 2 to 6 weeks of life.
Pyloric stenosis also occurs in adults, where the cause is usually a narrowed pylorus due to scarring from chronic peptic ulceration. This is a different condition from the infantile form.
Contents
- 1 Signs and symptoms
- 2 Cause
- 3 Diagnosis
- 4 Pathophysiology
- 5 Treatment
- 5.1 Surgery
- 6 Epidemiology
- 7 References
- 8 External links
Signs and symptoms
Babies with this condition usually present any time in the first weeks to months of life with progressively worsening vomiting. The vomiting is often described as non-bile stained ("non bilious") and "projectile vomiting", because it is more forceful than the usual spittiness (gastroesophageal reflux) seen at this age. Some infants present with poor feeding and weight loss but others demonstrate normal weight gain. Dehydration may occur which causes a baby to cry without having tears and to produce less wet or dirty diapers due to not urinating for hours or for a few days.[2] Constant hunger, belching, and colic are other possible signs that the baby is unable to eat properly.
Cause
Pyloric stenosis seems to be multifactorial, with some genetic and some environmental components. It is four times more likely to occur in firstborn males.[3] Rarely, infantile pyloric stenosis can occur as an autosomal dominant condition.[4]
Diagnosis
Diagnosis is via a careful history and physical examination, often supplemented by radiographic imaging studies. Pyloric stenosis should be suspected in any young infant with severe vomiting. On physical exam, palpation of the abdomen may reveal a mass in the epigastrium. This mass, which consists of the enlarged pylorus, is referred to as the 'olive',[5] and is sometimes evident after the infant is given formula to drink. Rarely, there are peristaltic waves that may be felt or seen (video on NEJM) due to the stomach trying to force its contents past the narrowed pyloric outlet.
Most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the thickened pylorus. Muscle wall thickness 3 millimeters (mm) or greater and pyloric channel length 14 mm or greater are considered abnormal in infants younger than 30 days.
Although somewhat less useful, an upper GI series (x-rays taken after the baby drinks a special contrast agent) can be diagnostic by showing the narrowed pyloric outlet filled with a thin stream of contrast material; a "string sign" or the "railroad track sign". For either type of study, there are specific measurement criteria used to identify the abnormal results. Plain x-rays of the abdomen sometimes shows a dilated stomach as shown here.
Although UGI endoscopy would demonstrate pyloric obstruction, physicians would find it difficult to differentiate accurately between hypertrophic pyloric stenosis and pylorospasm.
Blood tests will reveal low blood levels of potassium and chloride in association with an increased blood pH and high blood bicarbonate level due to loss of stomach acid (which contains hydrochloric acid) from persistent vomiting. There will be exchange of extracellular potassium with intracellular hydrogen ions in an attempt to correct the pH imbalance. These findings can be seen with severe vomiting from any cause.
Pathophysiology
The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum. As a consequence, all ingested food and gastric secretions can only exit via vomiting, which can be of a projectile nature. While the exact cause of the hypertrophy remains unknown, Rogers has assembled compelling evidence that neonatal hyperacidity is involved.[6]
This physiological explanation for the development of clinical pyloric stenosis at around 4 weeks and its spontaneous long term cure without surgery if treated conservatively, has recently been further reviewed.[7] The vomited material does not contain bile because the pyloric obstruction prevents entry of duodenal contents (containing bile) into the stomach.
Persistent vomiting results in loss of stomach acid (hydrochloric acid). The chloride loss results in a low blood chloride level which impairs the kidney's ability to excrete bicarbonate. This is the significant factor that prevents correction of the alkalosis.[8]
A secondary hyperaldosteronism develops due to the decreased blood volume. The high aldosterone levels causes the kidneys to avidly retain Na+ (to correct the intravascular volume depletion), and excrete increased amounts of K+ into the urine (resulting in a low blood level of potassium).
The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.
Treatment
Vertical Pyloromyotomy scar (large) 30 hrs post-op in a one-month-old baby
Horizontal Pyloromyotomy scar 10 days post-op in a one-month-old baby
Infantile pyloric stenosis is typically managed with surgery;[9] very few cases are mild enough to be treated medically.
The danger of pyloric stenosis comes from the dehydration and electrolyte disturbance rather than the underlying problem itself. Therefore, the baby must be initially stabilized by correcting the dehydration and the abnormally high blood pH seen in combination with low chloride levels with IV fluids. This can usually be accomplished in about 24–48 hours.
Intravenous and oral atropine may be used to treat pyloric stenosis. It has a success rate of 85-89% compared to nearly 100% for pyloromyotomy, however it requires prolonged hospitalization, skilled nursing and careful follow up during treatment.[10] It might be an alternative to surgery in children who have contraindications for anesthesia or surgery, or in children whose parents do not want surgery.
Surgery
The definitive treatment of pyloric stenosis is with surgical pyloromyotomy known as Ramstedt's procedure (dividing the muscle of the pylorus to open up the gastric outlet). This surgery can be done through a single incision (usually 3–4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference.[11]
Today, the laparoscopic technique has largely supplanted the traditional open repairs which involved either a tiny circular incision around the navel or the Ramstedt procedure. Compared to the older open techniques, the complication rate is equivalent, except for a markedly lower risk of wound infection.[12] This is now considered the standard of care at the majority of children's hospitals across the US, although some surgeons still perform the open technique. Following repair, the small 3mm incisions are hard to see.
The vertical incision, pictured and listed above, is no longer usually required, though many incisions have been horizontal in the past years.
Once the stomach can empty into the duodenum, feeding can begin again. Some vomiting may be expected during the first days after surgery as the gastrointestinal tract settles. Rarely, the myotomy procedure performed is incomplete and projectile vomiting continues, requiring repeat surgery. Pyloric stenosis generally has no long term side-effects or impact on the child's future.
Epidemiology
Males are more commonly affected than females, with firstborn males affected about four times as often, and there is a genetic predisposition for the disease.[13] It is commonly associated with people of Scandinavian ancestry, and has multifactorial inheritance patterns.[4] Pyloric stenosis is more common in Caucasians than Hispanics, Blacks, or Asians. The incidence is 2.4 per 1000 live births in Caucasians, 1.8 in Hispanics, 0.7 in Blacks, and 0.6 in Asians. It is also less common amongst children of mixed race parents.[14] Caucasian babies with blood type B or O are more likely than other types to be affected.[13]
Infants exposed to erythromycin are at increased risk for developing hypertrophic pyloric stenosis, especially when the drug is taken around two weeks of life[15] and possibly in late pregnancy and through breastmilk in the first two weeks of life.[16]
References
- ^ Hulka F, Campbell TJ, Campbell JR, Harrison MW (1997). "Evolution in the recognition of infantile hypertrophic pyloric stenosis". Pediatrics 100 (2): E9. doi:10.1542/peds.100.2.e9. PMID 9233980.
- ^ "Pyloric stenosis: Symptoms". MayoClinic.com. 2010-08-21. Retrieved 2012-02-21.
- ^ http://pediatrics.georgetown.edu/documents/Pyloric%20Stenosis.pdf. Missing or empty
|title=(help); Missing or empty|url=(help) - ^ a b Fried K, Aviv S, Nisenbaum C (November 1981). "Probable autosomal dominant infantile pyloric stenosis in a large kindred". Clin. Genet. 20 (5): 328–30. doi:10.1111/j.1399-0004.1981.tb01043.x. PMID 7333028.
- ^ Shaoul R, Enav B, Steiner Z, Mogilner J, Jaffe M (2004). "Clinical presentation of pyloric stenosis: the change is in our hands" (PDF). Isr Med Assoc J 6 (3): 134–7. ISSN 1565-1088. PMID 15055266.
- ^ Rogers, Ian; Vanderbom, Frederick (2014-02-26). The Consequence and Cause of Pyloric Stenosis of Infancy. More Books Lembert Academic Publishers. ISBN 978-3-659-52125-6.
- ^ Rogers, I.M. (2014). "Pyloric stenosis of infancy and primary hyperacidity-the missing link.". Acta. Paediatrica 103: e558–e560.
|accessdate=requires|url=(help) - ^ Kerry Brandis, Acid-Base Physiology. Retrieved December 31, 2006.
- ^ Askew, Nathan (October 2010). "An overview of infantile hypertrophic pyloric stenosis.". Paediatric nursing 22 (8): 27–30. PMID 21066945. Retrieved 30 August 2012.
- ^ Aspelund G, Langer JC (February 2007). "Current management of hypertrophic pyloric stenosis". Semin. Pediatr. Surg. 16 (1): 27–33. doi:10.1053/j.sempedsurg.2006.10.004. PMID 17210480.
- ^ "Medical News:Laparoscopic Repair of Pediatric Pyloric Stenosis May Speed Recovery - in Surgery, Thoracic Surgery from". MedPage Today. 2009-01-16. Retrieved 2012-02-21.
- ^ Sola JE, Neville HL (August 2009). "Laparoscopic vs open pyloromyotomy: a systematic review and meta-analysis". J. Pediatr. Surg. 44 (8): 1631–7. doi:10.1016/j.jpedsurg.2009.04.001. PMID 19635317.
- ^ a b Dowshen, Steven (November 2007). "Pyloric Stenosis". The Nemours Foundation. Retrieved 2007-12-30.
- ^ Pediatrics, Pyloric Stenosis at eMedicine
- ^ Maheshwai, Nitin (March 2007). "Are young infants treated with erythromycin at risk for developing hypertrophic pyloric stenosis?". Archives of Disease in Childhood 92 (3): 271–3. doi:10.1136/adc.2006.110007. PMC 2083424. PMID 17337692. Retrieved 30 August 2012.
- ^ Kong YL, Tey HL (June 2013). "Treatment of acne vulgaris during pregnancy and lactation". Drugs 73 (8): 779–87. doi:10.1007/s40265-013-0060-0. PMID 23657872.
External links
- 00675 at CHORUS
- UCL Institute of Child Health
- Peristaltic waves video on NEJM
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UpToDate Contents
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- 1. 幼児の肥厚性幽門狭窄症 infantile hypertrophic pyloric stenosis
- 2. 悪心および嘔吐を呈する幼児または小児に対するアプローチ approach to the infant or child with nausea and vomiting
- 3. 6ヵ月以下の乳幼児における体重減少の評価 evaluation of weight loss in infants six months of age and younger
- 4. 新生児におけるChlamydia trachomatis感染症 chlamydia trachomatis infections in the newborn
- 5. 胃排出遅延の原因 pathogenesis of delayed gastric emptying
English Journal
- No association between the SNPs (rs56134796; rs3824934; rs41302375) in the TRPC6 gene promoter and infantile hypertrophic pyloric stenosis in Chinese people.
- Ju JJ, Gao H, Li H, Lu Y, Wang LL, Yuan ZW.SourceKey Laboratory of Health Ministry for Congenital Malformation, Shengjing Hospital, China Medical University, Shenyang, 110004, People's Republic of China.
- Pediatric surgery international.Pediatr Surg Int.2011 Aug 6. [Epub ahead of print]
- PURPOSE: Our aim is to verify the association of three Single nucleotide polymorphisms (SNPs) (-218A/G, -254C/G, -361A/T) in the promoter of TRPC6 in 168 sporadic cases with infantile hypertrophic pyloric stenosis (IHPS) and 164 controls in Chinese people.METHODS: All participants were genotyped usi
- PMID 21822655
Japanese Journal
- 幽門狭窄症 (特集 これが大切! 1カ月以内の新生児疾患) -- (見落としてはならない重症疾患・対応に迷う疾患)
- 内田 広夫
- 小児科診療 74(4), 669-672, 2011-04
- NAID 40018758361
Related Links
- Pyloric stenosis (or infantile hypertrophic pyloric stenosis) is a condition that causes severe vomiting in the first few months of life. There is narrowing (stenosis ) of the opening from the stomach to the intestines, due to enlargement ...
Related Pictures
★リンクテーブル★
| リンク元 | 「肥厚性幽門狭窄症」 |
| 関連記事 | 「hypertrophic」「pyloric」 |
「肥厚性幽門狭窄症」
概念
- 幽門輪状筋の肥厚により幽門部の狭窄、通過障害をきたす。
- 生後2-3週より胃液、ミルクを嘔吐(無胆汁性)
病因
- 幽門の輪状筋の肥厚。生後には肥厚は存在せず、徐々に肥厚
疫学
- 500出生に一人。男児が女児の4-5倍多い (PED.1017)
病態
- 生後2-3週頃より嘔吐が始まり、回数、量が次第に増加し、1-2週間で噴水状となる(SPE.487)
- 生後2-4週で発症(PED.1017)
症状
- 噴水状嘔吐、低Clアルカローシス
- 脱水、電解質異常、低栄養
身体所見
- 胃： 胃蠕動の亢進
- 腹部：肥厚した幽門筋は上腹部正中ないし右側に母指頭大の硬い腫瘤として触知：オリーブ状腫瘤
検査
- 血算、血液生化学、動脈血ガス：低Cl性代謝性アルカローシス、低K血症、脱水による血液濃縮
- 腹部単純X線：胃の拡張・蠕動亢進、小腸ガスの減少
- single bubble sign：胃泡の著明な拡大
- 上部消化管造影検査：肥厚した幽門を認める
- string sign：狭小化した胃幽門部
- umbrella sign：狭小化した胃幽門部の肛門側に認められる
- beak sign
- 腹部超音波検査：細菌は幽門肥厚を直接描出して確定診断できる(PED.1017)
- doughnut sign：肥厚した幽門輪がlow。粘膜がhigh
- owl's eye sign：？
診断
- 身体所見(オリーブ状腫瘤)
- 検査(超音波：幽門部の筋層の厚さが4mm以上、長さが16mm以上)
治療
- 粘膜外幽門筋切除術 幽門筋切除術 Ramstedt手術
参考
- 1. 写真
国試
「hypertrophic」
- adj.
- 肥大性の、肥大型の、肥厚性の、肥大症の
「pyloric」
- 幽門の
- 関
- pylorus