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CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 2  |  Page : 133-135

A rare case of congenital pyloric atresia with situs inversus abdominalis


Fortis La Femme Hospital, Greater Kailash – II, New Delhi, India

Date of Web Publication25-Apr-2019

Correspondence Address:
Dr. Avadhesh Ahuja
Fortis La Femme, S.549, Greater Kailash - II, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.JCN_27_19

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  Abstract 


Congenital pyloric atresia and situs inversus are rare conditions. The combination of congenital pyloric atresia and situs inversus is extremely rare, only two such cases reported in the literature so far. We present third such case of a term, male baby born to a primigravida mother. All antenatal ultrasound up to 31 weeks were unremarkable. Follow-up antenatal ultrasound was suggestive of duodenal atresia. The baby was noted to have respiratory distress at birth requiring respiratory support in the form of nasal continuous positive air pressure. X-ray abdomen revealed a single bubble sign, but toward the right side of the abdomen, cardiac shadow being on the left side, suggestive of congenital pyloric atresia along with situs inversus abdominalis. The baby was operated, laparotomy done, findings confirmed, and retrograde side-to-side gastroduodenostomy was done. Postoperatively, feeds were started and baby discharged on day 17 of life. Isolated congenital pyloric atresia has a good prognosis and all attempts should be made to identify other possible associations (since there are different anomalies associated with congenital pyloric atresia such as junctional epidermolysis bullosa, renal anomalies [hydroureteronephrosis, multicystic kidney, absent bladder], aplasia cutis congenita, and colonic atresia).

Keywords: Congenital pyloric atresia, single bubble sign, situs inversus abdominalis


How to cite this article:
Ahuja A, Sikriwal D, Mallaiah R. A rare case of congenital pyloric atresia with situs inversus abdominalis. J Clin Neonatol 2019;8:133-5

How to cite this URL:
Ahuja A, Sikriwal D, Mallaiah R. A rare case of congenital pyloric atresia with situs inversus abdominalis. J Clin Neonatol [serial online] 2019 [cited 2019 May 22];8:133-5. Available from: http://www.jcnonweb.com/text.asp?2019/8/2/133/257137




  Introduction Top


Congenital pyloric atresia is a rare condition, the incidence of the condition being around 1 in 100,000 newborns worldwide. Dextrocardia and situs inversus are also similarly rare conditions. The combination of these two distinct entities is quite rare. We present here a rare case of congenital pyloric atresia along with situs inversus abdominalis. Pyloric atresia is also known to be associated with other anomalies such as renal anomalies, aplasia cutis congenital, colonic atresia and much be inspected.


  Case Report Top


A male baby was born at term (37 + 5 weeks of gestation) to a primigravida mother. The mother was antenatally diagnosed to have gestational diabetes mellitus (controlled on oral hypoglycemic-metformin) and had a history of polyhydramnios. Antenatal ultrasound done at 31 weeks showed a fetus with persistently distended stomach on the level of the duodenal cap. Another antenatal ultrasound scan done at 33 completed gestations weeks showed that the fetal stomach bubble was adequately distended and not overdistended. All prior prenatal ultrasonography before 31 weeks had not revealed fetal abnormality.

The baby was born through Elective LSCS (Indication: Polyhydramnios with Gestational Diabetes with? Duodenal Atresia). Baby received one course of antenatal steroids (2 doses of Betamethasone 12 h apart). Baby was born with a birth weight of 2.6 kg (14th centile–) and cried immediately after birth, APGAR scores being 7, 9, 9 at 1, 5 and 10 min, respectively.

The baby had meconium-stained amniotic fluid. Baby was noted to have tachypnea and respiratory distress soon after birth. SpO2 (preductal) on room air was ~76% even after 10 min. Baby was shifted to the neonatal intensive care unit immediately after birth. Blood gas revealed respiratory acidosis.

The baby was started on nasal continuous positive air pressure (CPAP) with peak end expiratory pressure (PEEP) 5 cm H20 and FiO2 30%.

Chest X-ray showed haziness in bilateral lung fields. FiO2 requirement decreased gradually, respiratory distress settled on nasal CPAP. No murmur was heard on cardiac auscultation.

After birth, the baby was started on intravenous fluids and kept nil by mouth. X-ray of the abdomen was done and showed a single gastric bubble on the right side; suggestive of pyloric atresia with situs inversus abdominalis [Figure 1] and [Figure 2]. Upper gastrointestinal contrast study was done and confirmed pyloric stenosis/pyloric atresia.
Figure 1: X-ray abdomen showing a large single bubble on the opposite side of cardiac shadow

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Figure 2: Another X-ray showing large single bubble on the right side of abdomen and nasogastric tube seen entering the bubble

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All neonatal reflexes were normal. Cranial ultrasound was done and showed a small 0.7 cm × 0.5 cm choroid plexus cyst in the posterior horn of the left lateral ventricle. Two-dimensional echocardiography was done to rule out any other associated anomaly and levocardia with fossa ovalis atrial septal defect (ASD) and rest normally. Physical examination and renal ultrasound did not reveal any other abnormality.

Pediatric surgery reference was sought, and baby operated. Exploratory laparotomy was done, findings were confirmed, and retrograde side-to-side gastroduodenostomy was done. Postoperatively, feeds started on day 6, which was tolerated well. Repeat contrast study was done and showed no holdup or leak of the contrast. Baby discharged on full feeds on day 17 of life.


  Discussion Top


Gastric outlet obstruction is divided into two types – pyloric atresia and antral webs.[1]

Congenital pyloric atresia is a rare condition, the incidence being around 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias.[2]

Dextrocardia with situs inversus has incidence of 0.09/1000 live births.[3] The combination of these two rare and entirely distinct entities is extremely rare. The combination of these two conditions occurs rarely and only two cases[4],[5] have been reported worldwide. We are reporting here the third case of the combination of these two conditions. In our case, it was Congenital pyloric atresia and Situs inversus abdominalis.

Congenital pyloric atresia is thought to result from the developmental arrest between the 5th and 12th weeks of intrauterine life.[6] Calder reported the first case of congenital pyloric atresia in 1749 and turoff performed the first successful operation in 1940.[7] Commonly, congenital pyloric atresia occurs as an isolated lesion, which has good prognosis, but it can also be seen in association with other malformations, which can have a bad impact on the ultimate outcome.[8]

Congenital pyloric atresia is divided into the following three types anatomically:

  • Type 1 – Pyloric membrane or web (57%)
  • Type 2 – Pyloric tissue replaced by solid cord/tissue (34%)
  • Type 3 – Pyloric atresia with a gap between stomach and duodenum (9%).


Nonbilious vomiting is the most common feature in congenital pyloric atresia. Neonates present with episodes of apnea, respiratory distress, or aspiration due to excessive secretions. The diagnosis of congenital pyloric atresia is based on the presence of a single large gastric air bubble with no gas distally on plain X-ray abdomen.

There can be different anomalies associated with congenital pyloric atresia such as junctional epidermolysis bullosa, renal anomalies (hydroureteronephrosis, multicystic kidney, and absent bladder), aplasia cutis congenita, and colonic atresia. Intraoperatively, it is necessary to check the patency of the remaining intestines using a catheter which is passed distally, and saline injected to rule out hereditary multiple intestinal atresias of the gastrointestinal tract.[9]

The treatment of congenital pyloric atresia depends on the type of atresia. Pyloric diaphragms are treated by excision and Heineke–Mikulicz pyloroplasty (involves incising the canal longitudinally, excising membrane, and transverse closure of pylorus). Congenital pyloric atresia without a gap is also treated by excision and pyloroplasty. For pyloric atresia with a gap, the treatment is gastroduodenostomy.

Isolated congenital pyloric atresia has good prognosis. The sometimes familial occurrence of congenital pyloric atresia, with a high frequency of consanguinity and an equal sex incidence, suggest a genetic predisposition, with an autosomal recessive mode of inheritance.[10],[11] A history of polyhydramnios is present in the majority of these patients.

In all the cases of congenital pyloric atresia, all efforts should be made to identify other associations, which have an impact on the overall prognosis of the child. Furthermore, the possibility of situs inversus totalis or situs inversus abdominalis should also be ruled out.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bawazir OA, Al-Salem AH. Congenital pyloric atresia: Clinical features, diagnosis, associated anomalies, management and outcome. Ann Pediatr Surg 2017;13:188-93.  Back to cited text no. 1
    
2.
Muller M, Morger R, Engert J. Pyloric atresia: Report of two cases and review of literature. Pediatr Surg Int 1990;5:276-9.  Back to cited text no. 2
    
3.
Taeusch HW, Ballard RA, Avery ME, Freed MD. Congenital cardiac malformations in diseases of the newborn. In: Taeusch HW, Ballard RA, Avery ME, editors. Schaffer and Avery's Diseases of the newborn. 6th ed. Philadelphia: WB Saunders; 1991. p. 91-633.  Back to cited text no. 3
    
4.
Harrington B, Chambers T, Grier D. A diagnosis obscured: Pyloric stenosis with situs inversus. Arch Dis Child 1997;76:385.  Back to cited text no. 4
    
5.
Gupta AK, Yadav L, Pathak M. A rare association of pyloric stenosis and situs inversus: Impact on diagnosis and treatment. J Clin Neonatol 2014;3:51-2.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Puri P, Fujimoto T. New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg 1988;23:221-5.  Back to cited text no. 6
    
7.
Sencan A, Mir E, Karaca I, Günşar C, Sencan A, Topçu K, et al. Pyloric atresia associated with multiple intestinal atresias and pylorocholedochal fistula. J Pediatr Surg 2002;37:1223-4.  Back to cited text no. 7
    
8.
Ilce Z, Erdogan E, Kara C, Celayir S, Sarimurat N, Senyüz OF, et al. Pyloric atresia: 15-year review from a single institution. J Pediatr Surg 2003;38:1581-4.  Back to cited text no. 8
    
9.
Lambrecht W, Kluth D. Hereditary multiple atresias of the gastrointestinal tract: Report of a case and review of the literature. J Pediatr Surg 1998;33:794-7.  Back to cited text no. 9
    
10.
Bar-Maor JA, Nissan S, Nero S. Pyloric atresia. J Med Genet 1972;9:70-2.  Back to cited text no. 10
    
11.
Olsen L, Grotte G. Congenital pyloric atresia: Report of a familial occurrence. J Pediatr Surg 1976;11:181-4.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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