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Table of Contents   
ORIGINAL ARTICLE
Year : 2021  |  Volume : 26  |  Issue : 6  |  Page : 416-420
 

Congenital Pyloric Atresia: Experience with a Series of 11 Cases and Collective Review


1 Department of General Surgery, DCMS, Hyderabad, Telangana, India
2 Department of Anesthesia, DCMS, Hyderabad, Telangana, India

Date of Submission24-Aug-2020
Date of Decision20-Sep-2020
Date of Acceptance27-Nov-2020
Date of Web Publication12-Nov-2021

Correspondence Address:
Dr. Gungi Raghavendra Prasad
3-9-14, Sharada Nagar, Ramanthapur, Hyderabad - 500 013, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_295_20

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   Abstract 


Introduction: Pyloric atresia is a rare cause of congenital gastric outlet obstruction. It is often associated with epidermolysis bullosa (EB). Rarity and experience with 11 cases are the reason for this publication.
Aims and Objectives: The aim and objective of this study is to present our experience of 11 cases of congenital pyloric atresia and correlate with available literature.
Materials and Methods: This was retrospective cohort of 11 cases correlative comparative study. Data of all the 11 cases from 1982 to 2019 were collected, reviewed, and analyzed. The parameters studied included age, gender, antenatal diagnosis, postnatal diagnosis, preoperative management, intraoperative findings, postoperative course outcome, associated anomalies, and any genetic studies if done. All these parameters were compared with published data.
Results: There were 11 cases in the present series with six boys and five girls. Most of them presented at varying periods from birth to day 1 of life.
Eight cases of type 1 pyloric atresia, two cases of type 2 pyloric atresia, and one case of type 3 pyloric atresia constituted the cohort.
Five out of 11 cases were associated with EB. Two out of six cases with isolated pyloric atresia and four out of five cases with EB died.
Discussion: Congenital pyloric atresia may be isolated or associated with EB. Three varieties of pyloric atresia were described. Association with EB increases the mortality.
Conclusions: Review and analysis of 11 cases of pyloric atresia compared with published literature is being reported.


Keywords: Epidermolysis bullosa, polyhydramnios, pyloric atresia


How to cite this article:
Prasad GR, Subba Rao J V, Fatima F, Anjum F. Congenital Pyloric Atresia: Experience with a Series of 11 Cases and Collective Review. J Indian Assoc Pediatr Surg 2021;26:416-20

How to cite this URL:
Prasad GR, Subba Rao J V, Fatima F, Anjum F. Congenital Pyloric Atresia: Experience with a Series of 11 Cases and Collective Review. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2021 Dec 4];26:416-20. Available from: https://www.jiaps.com/text.asp?2021/26/6/416/330377





   Introduction Top


Pyloric atresia is a rare cause of congenital gastric outlet obstruction. It may be associated with epidermolysis bullosa (EB). EB when associated with congenital pyloric atresia contributes to increased mortality. Experience with 11 cases over 37 years is the reason for this publication.

Aims and objectives

To report, review, analyze, and correlate 11 cases of congenital pyloric atresia with published literature.


   Materials and Methods Top


This is retrospective cohort of 11 cases and a correlative comparative study. The data of all cases of pyloric atresia were retrieved from senior author's database from 1982 till 2019. The various parameters collected were age, gender, antenatal diagnosis, postnatal diagnosis, preoperative management, intraoperative findings, postoperative course, outcome, associated anomalies, and any genetic studies done.

Similar parameters from the published data were tabulated and compared with the present series with particular attention to the association of pyloric atresia with EB.

The diagnostic modalities included antenatal ultrasonography (USG), plain radiograph, barium series, and imaging for associated anomalies.


   Results Top


There were 11 cases in the present series with six boys and five girls. Most of them presented at varying periods from birth to day 1 of life. Pyloric atresia was suspected antenatally based on polyhydramnios and single bubble sign. Few cases underwent chromosomal analysis with normal karyotyping in most of them. Contrast study was done in all.

All cases managed with care of fluid and electrolyte balance, nasogastric decompression, prevention of sepsis, prevention of exposure-related hypothermia, particularly in cases associated with EB along with standard new-born care.

Following was the observation in the present series: four cases presented at birth, three cases presented <12 h, and three in <24 h. There were six boys and five girls.

There were eight cases of type 1 pyloric atresia, two cases of type 2, and one case of type 3 pyloric atresia.

Five out of 11 cases were associated with EB.

Five out of 11 cases survived and six out of 11 cases died. Two out of six cases (33.3%) with isolated pyloric atresia died. Four out of five cases (80%) with EB died.

In the present series, one case of EB has survived and is growing satisfactorily. Follow-up included a child of 11 years. Skin started healing and the child continues to take extra caution to prevent trauma to the skin. Children with isolated pyloric atresia's are thriving well.

One child of gastroduodenostomy presented with obstruction. Laparotomy revealed multiple intestinal adhesions and narrowed stricture of gastroduodenostomy. It was managed by adhesiolysis and revision gastroduodenostomy.

Isolated cases of pyloric atresia did not show any anomalies. However, pyloric atresia with EB showed various renal anomalies, cardiovascular anomalies such as PDA and VSD. The present series did not have any syndromic EB.

[Table 1] shows comparative analysis of the chosen parameters with present series and published literature.
Table 1: Comparative analysis of the chosen parameters with present series and published literature

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   Discussion Top


Pyloric atresia is a rare cause of gastric outlet obstruction. Incidence varies approximately 1 in 100000 live births.[1],[2] It is a developmental error. Many hypotheses have been proposed to explain its occurrence ranging from noncanalization to intrauterine vascular accident.[3],[4] Other explanations included recurrent mucosal ulceration and healing leading to scar formation. This was described as a part of effect of altered expression of alpha 6 beta integrin which leads to adherence of epidermis to tissues at the level of dermo-epidermal junction as a result of mutations in ITGA6 and ITGB4 genes.[4],[5],[6]

It can be suspected antenatally on high resolution maternal USG. Their findings included polyhydramnios and single bubble sign.[1],[7],[8] In the present series, 7 out of 11 cases were diagnosed antenatally [Figure 1]. In the published literature, 5 out of 11 cases showed antenatal suspicion.
Figure 1: (a) Clinical picture of a new-born with epidermolysis bullosa showing exfoliated skin of almost the entire body. (b) Plain radio-\graph showing single bubble with total paucity of gas distally. Single bubble sign

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Most of the published data were single case reports, Gupta et al. published experience with 11 cases.[1]

EB is often associated with pyloric atresia.[4],[8],[9],[10] It is a mechanobullous disorder characterized by increased skin fragility and blister formation.[11],[12] The association is classical and characteristic.[1],[2],[7],[8],[9] EB is associated with type 6 aplasia cutis congenita.[13],[14] They are usually caused by genetic errors.[4],[5] Five out of 11 cases in the series were associated with EB.

Four types of EB were described.[15] EB simplex (EBS) and dystrophic EB are usually due to autosomal dominant (AD) genes and hence may have familial inheritance. Whereas junctional EB and kindler syndrome are inherited in an autosomal recessive pattern.[10] EB acquisita is rare and usually not associated with pyloric atresia.

The treatment of pyloric atresia includes (1) Preoperative care for gastric outlet obstruction, (2) relief of obstruction, and (3) establishment of gastro-duodenal continuity.[1],[2],[9],[10]

Associated EB requires additional care - To prevent evaporative hypothermia and fluid loss, prevention of sepsis, evaluation of associated anomalies, protective dressing to prevent further exfoliation, avoiding trauma. An attempt was made to take care of all the above aspects in the case series with EB. The primary treatment of pyloric atresia is operative intervention.[4]

Three anatomical types of PA's were described.[1],[2],[5],[7],[9],[10] Types of Pyloric Atresia–(1) Type 1– Pyloric membrane or web (57%). (2) Type 2 – Pyloric tissue replaced by solid cord/tissue (34%). (3) Type 3 – Pyloric atresia with a gap between the stomach and duodenum (9%).

The present series had eight type 1, two type 2, and one type 3 pyloric atresia's. The published literature showed similar incidences 65.6% of type 1, 12.5% of type 2, and 15.6% of type 3 pyloric atresia.

Type 1 usually managed by excision of diaphragm and Heineke Mikulicz or Finney's type pyloroplasty [Figure 2]. In the present series, all Type 1 PA's were managed by Heineke Mikulicz type pyloroplasty. The authors routinely added feeding jejunostomy and/or double gastrostomy tube with one feeding tube across the anastomoses into the jejunum for feeding purposes.
Figure 2: (a) Operative picture showing type 1 pyloric atresia. (b) Completed Heineke Mikulicz type of pyloroplasty

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Type 2 and Type 3 were managed by gastroduodenostomy. A two-stage procedure was recommended by Sonal Nagra and Jitaco KC.[16]

Gastrojejunostomy was not done in present series. Published literature suggested gastro-jejunostomy previously but not scientifically advisable/desirable procedure for gastric outlet obstruction. Multiple anomalies were described including multiple intestinal atresia's.[17]

Chromosomal karyotyping was done in eight out of 11 cases in the present series, with normal results. Additional genetic test includes gene analysis of ITBG4 gene. The present series did not do genetic analysis. Published literature showed the presence of novel mutation in the ITGB 4 gene.[15]

Comparing the outcome, present series had 54.5% of mortality, splitting further isolated pyloric atresia with 33.3% mortality from pyloric atresia associated with EB showing increased mortality (80%). Similar experience is published by many.[1],[3],[7],[9],[10],[15],[18]


   Conclusions Top


Pyloric atresia is a rare cause of gastric outlet obstruction.

Association with EB is often characteristic. EB with pyloric atresia is often fatal.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gupta R, Soni V, Mathur P, Goyal RB. Congenital pyloric atresia and associated anomalies: A case series. J Neonatal Surg 2013;2:40.  Back to cited text no. 1
    
2.
Zecca E, Corsello M, Pintus C, Nanni L, Zecca S. Peculiar type 1 congenital pyloric atresia: A case report. Ital J Pediatr 2010;36:3.  Back to cited text no. 2
    
3.
Kim JH, Park HY, Lee HJ, Eom M, Choi EH. Case of epidermolysis bullosa with pyloric atresia. Ann Dermatol 2011;23 Suppl 1:S41-4.  Back to cited text no. 3
    
4.
Pfendner EG, Lucky AW. Epidermolysis Bullosa with Pyloric Atresia. 2008 Feb 22 [Updated 2017 Sep 7]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews®. Seattle (WA): University of Washington, Seattle; 1993-2020.  Back to cited text no. 4
    
5.
Brown TA, Gil SG, Sybert VP, Lestringant GG, Tadini G, Caputo R, et al. Defective integrin alpha 6 beta 4 expression in the skin of patients with junctional epidermolysis bullosa and pyloric atresia. J Invest Dermatol 1996;107:384-91.  Back to cited text no. 5
    
6.
Chung HJ, Uitto J. Epidermolysis bullosa with pyloric atresia. Dermatol Clin 2010;28:43-54.  Back to cited text no. 6
    
7.
Milad N, Sanjad S, Charafeddine L. Pyloric atresia: An unusual presentation. J Case Rep 2016;6:104-8.  Back to cited text no. 7
    
8.
Hassan ME, Ali KA, Khalaf M, Taryam L. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: A case report and literature review. Ann Pediatr Surg 2013;9:84-86  Back to cited text no. 8
    
9.
Purnachandra D, Prathvi S. Carmi syndrome associated with gastric perforation. Ann Pediatr Surg 2013;9:122-3.  Back to cited text no. 9
    
10.
Parelkar SV, Kapadnis SP, Sanghvi BV, Joshi PB, Mundada D, Shetty S, et al. Pyloric atresia–three cases and review of literature. Afr J Paediatr Surg 2014;11:362-5.  Back to cited text no. 10
[PUBMED]  [Full text]  
11.
McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Dermatol 1991;30:481-4.  Back to cited text no. 11
    
12.
Lin AN, Carter DM. Epidermolysis bullosa: When the skin falls apart. J Pediatr 1989;114:349-55.  Back to cited text no. 12
    
13.
Blionas A, Giakoumettis D, Antoniades E, Drosos E, Mitsios A, Plakas S, et al. Aplasia cutis congenita: Two case reports and discussion of the literature. Surg Neurol Int 2017;8:273.  Back to cited text no. 13
    
14.
Frieden IJ. Aplasia cutis congenita: A clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60.  Back to cited text no. 14
    
15.
Okulu E, Durmaz CD, Tunc G, Guzel A, Kutlay NY, Erdeve O, et al. A novel mutation in ITGB4 gene in a newborn with epidermolysis bullosa, pyloric atresia, and aplasia cutis congenita. Egypt J Med Hum Genet 2020;21:16  Back to cited text no. 15
    
16.
Nagra S, Cama JK. Pyloric atresia in a healthy newborn. J Ped Surg 2014;2:12-4.  Back to cited text no. 16
    
17.
Pujar VC, Kurbet S, Kaltar DK. Pyloric atresia in association with multiple colonic atresias in a neonate: An unreported association. J Neonatal Surg 2012;1:6.  Back to cited text no. 17
    
18.
Ksia A, Zitouni H, Zrig A, Laamiri R, Chioukh F, Ayari E, et al. Pyloric atresia: A report of ten patients. Afr J Paediatr Surg 2013;10:192-4.  Back to cited text no. 18
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