Gastric Deserosalization: What Lies Behind Closed Doors.

Congenital intestinal malrotation has a wide spectrum of presentation varying from incidental detection to recurrent episodes of benign abdominal pain to frank gastrointestinal obstruction, mid-gut volvulus, and bowel gangrene. Herein, we report the first case of congenital intestinal malrotation leading to gastric deserosalization. Intraoperative findings were conspicuous by the presence of midgut volvulus and gastric perforation in the posterior wall. There are a few more dimensions uncovered by this case, a brief reference to each has been considered necessary.

INTRODUCTION

We report the first case of gastric deserosalization in a case of congenital intestinal malrotation and the most plausible (behind closed doors) cause-effect sequence.

There are a few more dimensions uncovered by this case; a brief reference was considered mandatory.

CASE REPORT

The baby presented on 6th-day of life with bilious-vomiting, abdominal distention, and tachypnea.

The baby was born 600 km from our tertiary care center. He developed refusal to feed with vomiting on 2nd day of life. The vomitus was initially nonbilious and subsequently bilious. There was upper abdomen distention without visible peristalsis.

Barium study performed in another center (@age 4 days) revealed massive distention of stomach up to 4 h [Figure 1]. Contrast could be appreciated in distal small and large bowel loops also.

Figure 1

Barium meal follow through showing huge distention of the stomach with contrast in the distal bowel loops after 5 min, 30 min, 2 h, and 4 h of administering the contrast. Dilatation of duodenum and proximal jujenum and position of duodenojejunal junction in relation to the spine is not clearly elicited in the radiographs

Clinical condition at presentation

Dehydration (moderate to severe), sepsis, and shock.

He was pale and icteric with weak pulses, hypotension, prolonged capillary filling time, parietal edema, and sclerema. There were no nasogastric or orogastric tubes or urinary catheter. The intravenous access was displaced during transportation. X-ray was suggestive of pneumoperitoneum. After Intensive Care Unit admission, the baby was resuscitated, and bilateral flank glove-drains were inserted.

The baby was explored by a right upper transverse incision after 48 h.

Operative findings

(1) Fecal contamination of peritoneal cavity [Figure 2], (2) malrotation (3) midgut volvulus (one-and-half turns), (4) Abrupt change in caliber of bowel 1 cm distal to duodenum-jejunal junction which was being compressed by the coil of mid-gut volvulus, persistence of compression band despite derotation of volvulus (5) Hugely distended stomach with deserosalization of proximal half of stomach involving both walls, (6) Massive ballooning of the deserosalized-mucosa displacing the left hemidiaphragm, (7) Lesser sac distended with gastric contents with a small rent limiting its free release into the peritoneum, and (8) 1 cm × 1.5 cm perforation of posterior stomach wall (deserosalized).

Figure 2

Intraoperative images showing (a) anterior view of stomach revealing deserosalization, i.e., separation of serosa from the mucosa. Due to decompression of the stomach cavity, the ballooning of the mucosal bag is less evident. (b) Posterior view of stomach after opening the lesser sac showing deserosalization and perforation of the gastric mucosa in the deserosalized region. (c) Proximal jujenum just distal to duodenojejunal junction showing persistence of nonobstructing constricting band in the region compressed by the volvulus loop (zoomed appearance in inset for better visualization). (d) Postrepair of stomach serosa by imbrication

Surgical procedures

(1) Derotation of volvulus, (2) Peritoneal toileting, (3) Ladd's procedure without appendectomy, (4) Foley's test to rule out duodeno-jejunal web, (5) Repair of gastric perforation, (6) Primary repair of serosal edges by mucosal imbrications (without any loss of mucosal surface and contained glands), and (7) Feeding jejunostomy (to initiate early feeding).

Postoperative period

The postoperative period was prolonged but showed consistent improvement.

Peroral dye study (day 10) of surgery was satisfactory. The stomach silhouette, capacity, and emptying time were acceptable. The patient showed complete recovery.

DISCUSSION

Neonatal gastric perforation, rare though is an important cause of neonatal pneumoperitoneum. Other common causes include gastric acidity in the first few days of life, posttraumatic, asphyxia-ischemia sequence, vascular shunting, and lack of pacemaker and C-KIT mast cells.[1] Isolated case reports have documented gastric perforation in patients with malrotation.[234]

A preliminary literature search suggested that gastric deserosalization in a case of malrotation is yet to be reported. Deserosalization of bowel[5] has occasionally been reported in trauma cases.

It seems that in the index case, the primary pathology was congenital intestinal malrotation.

The development of mid-gut volvulus led to gastrointestinal obstruction. The persistent change in caliber of the bowel in the jejunum immediately distal to the duodenum-jejunum junction in the absence of signs of vascular insult to the mid-gut loops suggest that the volvulus was chronic and possibly antenatal in origin.

The gastrointestinal obstruction led to progressive distention of the stomach and the duodenum-jejunum proximal to the site of obstruction; the distention was being propagated by feeds, saliva, and other gastrointestinal secretions. The vomiting episodes and the partial distal decompression must have been the respite.

A tear in the gastric mucosa may have been initiated by vigorous gastric peristalsis – antegrade or retrograde or during an unnoticed abdominal trauma in the setting of massive gastric distention. Subsequent gastric peristalsis and the act of vomiting must have triggered progressive gastric deserosalization. The deserosalized portion of the stomach underwent ballooning in response to progressive distention. This is supported by the huge distention of stomach in the contrast study in the absence of pneumoperitoneum

Once dilated to its limit, the final event was the rupture of the gastric mucosa in the posterior wall of the stomach and leak of the gastric contents into the lesser sac. Three mechanisms for perforation of the stomach have been described as follows: traumatic (including iatrogenic), ischemic, and spontaneous.[3]

Herein, we report the fourth mechanism – augmented intragastric pressure from gastrointestinal obstruction, gastric distention, retrograde gastric peristalsis (vomiting), simultaneous and coordinated contraction of diaphragm, and abdominal wall muscles in the setting of gastric deserosalization. Shaw et al.[6] reported mechanical pneumatic rupture of the stomach in his experiment after he tied both ends of stomach and insufflated it with air (Laplace's Law). Supporting factors include lack of coordinated esophageal peristalsis in the neonate[7] and neonatal immaturity of the vomiting mechanism making it possible to increase the intragastric pressure to its limit.[8]

CONCLUSION

Herein, we report the first case of gastric deserosalization in intestinal malrotation with mid-gut volvulus and the most plausible cause-effect sequence.

We also report a new mechanism of neonatal gastric perforation – augmented intragastric pressure from gastrointestinal obstruction, gastric distention, and retrograde gastric peristalsis (vomiting) along-with simultaneous and coordinated contraction of diaphragm and abdominal wall muscles in the setting of gastric deserosalization.

This case also highlights the potential of congenital intestinal malrotation to result in catastrophic sequel with high morbidity and mortality.

An inquisitive eye will also find a hint toward the need for safe, supervised, and well-organized transport of the surgical neonate.

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.