|Year : 2023 | Volume
| Issue : 5 | Page : 421-424
Nonhypertrophic pyloric stenosis in a neonate: A rare presentation
TK Jayakumar1, Kirtikumar J Rathod1, Arvind Sinha1, Taruna Yadav2
1 Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
|Date of Submission||29-Oct-2022|
|Date of Decision||22-Jan-2023|
|Date of Acceptance||03-Mar-2023|
|Date of Web Publication||25-Apr-2023|
Kirtikumar J Rathod
Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Gastric outlet obstruction in neonates due to nonhypertrophic pyloric stenosis (NHPS) is a rare cause. We report the case of a 37-day-old baby boy who presented with complaints of vomiting for the last 2 weeks and an inconsolable cry over the last 2 days. He has been vomiting seven to eight times a day, a few hours after breastfeeding. On ultrasonography, the stomach was distended, while the pylorus was not hypertrophied. An upper gastrointestinal (GI) contrast study was done, which was suggestive of gastric volvulus. We performed a laparoscopy for the same. Intraoperatively, the volvulus was already resolved. We performed gastropexy. Postoperatively, he had persistent symptoms, for which an upper GI endoscopy was performed. It demonstrated a narrow pylorus, consistent with the NHPS. We performed a laparoscopic Heineke–Mikulicz pyloroplasty. The patient's symptoms had improved postoperatively. He was discharged after 5 days.
Keywords: Gastric outlet obstruction, laparoscopy, neonate, non-hypertrophic pyloric stenosis
|How to cite this article:|
Jayakumar T K, Rathod KJ, Sinha A, Yadav T. Nonhypertrophic pyloric stenosis in a neonate: A rare presentation. J Indian Assoc Pediatr Surg 2023;28:421-4
|How to cite this URL:|
Jayakumar T K, Rathod KJ, Sinha A, Yadav T. Nonhypertrophic pyloric stenosis in a neonate: A rare presentation. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Oct 2];28:421-4. Available from: https://www.jiaps.com/text.asp?2023/28/5/421/374696
| Introduction|| |
Idiopathic/infantile hypertrophic pyloric stenosis is the most common cause of gastric outlet obstruction in infants. It is an acquired condition usually seen beyond the neonatal period. Congenital causes of gastric outlet obstruction are rare. The reported incidence is <1% of all congenital gastrointestinal (GI) obstructions. Congenital causes of gastric outlet obstruction include gastric atresia (of antrum and pylorus), prepyloric web, annular pancreatic tissue, and antral duplication cysts.,
Nonhypertrophic pyloric narrowing as a primary cause of gastric outlet obstruction was reported in infants, young children, and even adults.,,, The terminology is inconsistent and has changed over the years. The initial classification provided by Moore was modified by Sharma et al. and later by Pathak et al.,,
However, nonhypertrophic pyloric stenosis (NHPS) as a cause of gastric outlet obstruction has never been reported in a neonate.
| Case Report|| |
A 37-day-old baby boy was brought to our emergency room with the complaint of nonbilious vomiting for 15 days, and an inconsolable cry for the last 2 days. He was apparently fine until he developed the symptoms. He had 7–8 episodes of vomiting per day, almost every time he was breastfed. The vomiting occurred after 1–2 h. Of feeding, and it contained breastmilk. For the last 2 days, he has been experiencing discomfort and had episodes of inconsolable crying.
The antenatal and perinatal history was unremarkable. He has been on exclusive breastfeeding since birth. There was no treatment history before the symptoms appeared, and he was never given any herbal medicines, milk from animal sources, or artificial feeds. On examination, he had signs of mild dehydration. The upper abdomen was distended and tender. No lump/olive-like swelling was palpable.
He was resuscitated with intravenous fluids. A nasogastric (NG) tube was inserted, and the aspirates contained gastric fluid.
An ultrasonography (USG) study demonstrated a distended stomach. The pyloric wall thickness was normal (2.2 mm). An upper GI contrast series was suggestive of gastric volvulus, with a pyloroduodenal junction at the same level as the gastroesophageal junction, with a thin streak of contrast passing into the duodenum. The duodenojejunal flexure was located on the left side. A delayed image showed persistent contrast within the stomach [Figure 1]a, [Figure 1]b, [Figure 1]c. The patient was taken up for a diagnostic laparoscopy. During the surgery, the gastric volvulus had already resolved by itself. The stomach was distended and appeared edematous. A gastropexy procedure was done.
|Figure 1: Upper GI contrast study (a-c). (a) Contrast outlining lower esophagus and stomach. (b) Study shows features of gastric volvulus (pyloroduodenal junction is at the same level of GE junction) with a thin streak of contrast passing into the duodenum. DJ flexure is normal in the location on the left side. (c) Delayed image shows persistent contrast in the stomach with slow transit. (d) High-resolution ultrasound image (transverse view) of the stomach. The air column is seen in the pyloric lumen (arrows) with normal thickness of the pyloric wall and distended stomach. (Length of pylorus 1.97 cm, the thickness of the wall 0.22 cm). GI: Gastrointestinal|
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During the postoperative period, the patient had persistent symptoms, which compelled us to review the case. On postoperative USG of the abdomen, the findings were similar, showing a distended stomach and a pylorus with normal wall thickness [Figure 1]d. Following this, an endoscopy (performed by a gastroenterologist with the smallest endoscope available) could demonstrate a stenosed, nondistending pylorus. The flexible endoscope could not be negotiated into the pylorus [Figure 2]a. The esophagus and stomach mucosa were normal, with no signs of inflammation. The radiological and endoscopy findings were suggestive of NHPS.
|Figure 2: (a) Endoscopic appearance of the narrow pyloric lumen, (b) Laparoscopic appearance of distended stomach and the pylorus|
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The patient was operated on again. During the laparoscopy, the stomach was distended, and there was a distinct groove between the antrum and the pylorus, indicating the proximal level of stenosis [Figure 2]b. The gastropexy was not taken down. A wide incision (about 1.5–2 cm) was given along the axis of the pylorus extending it over the antrum and duodenum. Distally, there were no webs or membranes within the duodenum. Pyloroplasty using the Heineke–Mikulicz procedure was done by intracorporeal suturing using absorbable sutures.
Postoperatively, feeds were started through an NG tube on day 2. Full feeds were achieved by day 5. The patient's symptoms had subsided, and he was discharged. He remained asymptomatic during the follow-up.
| Discussion|| |
Due to the rarity of the cases, gastric outlet obstruction in children, due to primary causes other than infantile hypertrophic pyloric stenosis or congenital gastric atresias, were called by different names. The primary acquired gastric outlet obstruction, also called the “Jodhpur disease” was first described by Sharma et al. All of the cases they reported had a narrowing of the pyloric canal without any muscular hypertrophy, histologically proven normal pylorus, with no other secondary causes. Such similar cases have been reported later as well. The increased frequency of the reporting of this new entity had fueled the argument that the classification proposed by Moore et al., for the causes of gastric outlet obstruction in children is inadequate. Sharma et al. provided a new classification that was more clinically relevant. Later, primary acquired gastric outlet obstruction due to cicatrization, which is a new entity, was as well reported., After this, the classification of GOO was revised again.
In our case, the patient had an onset of symptoms during the 3rd week of life. He did not have any history suggestive of infection, consumption of drugs, ingestion of herbal medicine, or any caustic/corrosive substances. He did not have symptoms during the initial 2 weeks of his life. This suggests that the narrowing of the pyloric canal is an acquired cause. As this condition was never described in a neonate before, our clinical suspicion of primary acquired gastric outlet obstruction was low. The gastric volvulus was initially diagnosed as an idiopathic event, and he was treated accordingly. As the symptoms were persistent in the postoperative period, he was reevaluated.
The approach to such cases would be to get a USG study to rule out the hypertrophy of pylorus.
An upper GI contrast study or barium follow-through, is done to demonstrate the gastric dilatation and narrow outlet at the pylorus. In our case, the patient had gastric volvulus at the time of the upper GI contrast study, which is an unusual finding which was not reported before. There was a faint amount of contrast within the small bowel in our case. Depending on the amount of obstruction, the degree of contrast within the small bowel can vary from almost being nil to the delayed passage of contrast into the small bowel. We reviewed the images published in the literature and found similarities with the report of Srivastava et al., where there was a faint presence of contrast within the small bowel in a case of GOO. Although it is rare, the gastric volvulus has probably occurred due to pyloric obstruction.
Endoscopy was the next investigation of choice. In our case, there was a distinct narrowing of the pyloric opening. Similar findings were reported in previous studies as well.,, The finding is subjective, and there is no standard cutoff value to determine the stenosis.
Among the treatment modalities, Heineke–Mikulicz pyloroplasty has been the most widely practiced operation.,, In cases of cicatrization, Pathak et al. reported that V-Y advancement antropyloroplasty was better than the Heineke–Mikulicz technique, as the cicatrized tissue makes it difficult for a transverse incision to be closed in a vertical fashion. Pneumatic dilatation of the narrow pylorus via endoscopy is another described technique for the management of these cases., Minimally invasive techniques were not reported before, and ours is the only case to have been operated on by laparoscopy. During the operation, we could distinctly see the narrowing between the antrum and pylorus while the stomach was distended. The Heineke–Mikulicz procedure was accomplished laparoscopically and proved to be effective in our case. Although there is no way of deciding which treatment modality is superior, the management must be decided based on the available facilities and expertise of the treating surgeon.
Primary acquired gastric outlet obstruction or NHPS can present even from the neonatal period. These cases can be easily missed while being treated symptomatically with medications. While our patient presented early, such cases could present much later with severe fluid imbalances, malnutrition, and weight loss. It is necessary to have a clinical suspicion and evaluate when there are no secondary causes and the pylorus is not hypertrophic.
| Conclusion|| |
Primary acquired gastric outlet obstruction or NHPS is a rare condition. It was never described in neonates before. Diagnosis is made after ruling out secondary causes and proceeding further with a combination of investigations like USG study, upper GI contrast study, and endoscopy. Laparoscopy can be both diagnostic and therapeutic as we demonstrated in our case.
Declaration of patient consent
Authors hereby declare that proper consent has been obtained from the patient's parents for publishing the clinical/radiological/operative images and clinical information without revealing the identity of the patient.
Financial support and sponsorship
Conflict of interest
There are no conflicts of interest.
| References|| |
Berrocal T, Torres I, Gutiérrez J, Prieto C, del Hoyo ML, Lamas M. Congenital anomalies of the upper gastrointestinal tract. Radiographics 1999;19:855-72.
Moore CC. Congenital gastric outlet obstruction. J Pediatr Surg 1989;24:1241-6.
Till H. Congenital pyloric stenosis, webs and strictures. In: Till H, Thomson M, Foker JE, Holcomb GW 3rd
, Khan KM, editors. Esophageal and Gastric Disorders in Infancy and Childhood. Berlin, Heidelberg: Springer; 2017. p. 1353-4.
Sharma KK, Agrawal P, Toshniwal H. Acquired gastric outlet obstruction during infancy and childhood: A report of five unusual cases. J Pediatr Surg 1997;32:928-30.
Maylard AE. Congenital narrowness of the pyloric orifice a cause of chronic gastric disease in the adult, illustrated by seven cases successfully treated by operation. Br Med J 1904;1:416-9.
Hameury F, Mcheik J, Lardy H, Gaudin J, Petit T, Ravasse P, et al.
Acquired non hypertrophic pyloric stenosis in children. Arch Pediatr 2007;14:330-3.
Srivastava P, Gangopadhyay A, Upadhyaya V, Sharma S, Jaiman R, Kumar V. Unusual acquired gastric outlet obstruction during infancy: A case report. Cases J 2008;1:237.
Sharma KK, Ranka P, Goyal P, Dabi DR. Gastric outlet obstruction in children: An overview with report of Jodhpur disease and Sharma's classification. J Pediatr Surg 2008;43:1891-7.
Pathak M, Saxena R, Patel H, Sinha A. Primary acquired cicatrizing gastric outlet obstruction in children. J Indian Assoc Pediatr Surg 2022;27:38-41. [Full text]
Kajal P, Bhutani N, Kadian YS. Primary acquired gastric outlet obstruction (Jodhpur disease). J Pediatr Surg Case Rep 2019;40:6-9.
Ratan SK, Dhua AK, Sinha S, Mathur M, Rao S, Aggarwal S. Acquired nonspecific cicatrizing inflammation causing pyloric stricture and gastric outlet obstruction in infancy: Is it Jodhpur disease? Trop Gastroenterol 2013;34:274-7.
Nazir Z, Arshad M. Late-onset primary gastric outlet obstruction – An unusual cause of growth retardation. J Pediatr Surg 2005;40:e13-6.
Bartlett ES, Carlisle EM, Mak GZ. Gastric outlet obstruction in a 12 year old male. J Pediatr Surg Case Rep 2018;31:57-9.
Lin JY, Lee ZF, Yen YC, Chang YT. Pneumatic dilation in treatment of late-onset primary gastric outlet obstruction in childhood. J Pediatr Surg 2007;42:e1-4.
Karnsakul W, Cannon ML, Gillespie S, Vaughan R. Idiopathic non-hypertrophic pyloric stenosis in an infant successfully treated via endoscopic approach. World J Gastrointest Endosc 2010;2:413-6.
[Figure 1], [Figure 2]