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Cureus Mar 2024Lymphatic malformations frequently present as benign masses in the neck and clavicle region among infants and young children. Cystic hygroma represents an...
Lymphatic malformations frequently present as benign masses in the neck and clavicle region among infants and young children. Cystic hygroma represents an often-encountered form of lymphatic malformation. This case report details the medical history of a one-year-old girl characterized by a multifaceted medical background, initially exhibiting symptoms of persistent cough, cold, and neck swelling. Further investigations revealed more severe conditions: complex congenital heart defects, including large atrial septal defect (ASD), large ventricular septal defect (VSD), and aorta arising from the right ventricle with cystic hygroma and annular pancreas. The patient underwent various diagnostic tests, including chest X-rays, ultrasound, magnetic resonance imaging (MRI), and computed tomography pulmonary angiogram (CTPA), leading to multidisciplinary treatment involving sclerotherapy for cystic hygroma and supportive therapies. The case underscores the challenges in diagnosing and managing pediatric patients with overlapping conditions and the critical need for continuous follow-up.
PubMed: 38659571
DOI: 10.7759/cureus.56852 -
Case Reports in Gastroenterology 2024Portal annular pancreas (PAP) is a congenital anomaly resulting from aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein (PV). PAP was...
INTRODUCTION
Portal annular pancreas (PAP) is a congenital anomaly resulting from aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein (PV). PAP was classified into three types by Joseph et al., based on the location of the main pancreatic duct around the PV. The presence of PAP is important for the surgical procedure because it is associated with the postoperative pancreatic fistula. There are no standardized surgical procedures of resection and reconstruction for PAP.
CASE PRESENTATION
We report 2 cases of subtotal stomach-preserving pancreatoduodenectomy in patients with PAP. One case of PAP was discovered coincidentally intraoperatively, and the other case was diagnosed before surgery. The first case was an 84-year-old male patient who underwent surgery for distal bile duct cancer. PAP was noticed intraoperatively when the uncinate process of the pancreas was detached from behind the PV. The second case was an 84-year-old female patient who also underwent surgery for distal bile duct cancer. We recognized PAP from preoperative computed tomography images. In both cases, the ductal anatomy was consistent with type IIIA PAP, and the dorsal pancreas was resected using a stapling device. During the postoperative period, there was no clinically relevant postoperative pancreatic fistula.
CONCLUSION
PAP is rarely encountered intraoperatively; however, it is important to recognize it before surgery and take it into consideration when deciding upon the procedures for resection and reconstruction.
PubMed: 38628808
DOI: 10.1159/000538370 -
The American Journal of Case Reports Mar 2024BACKGROUND Bilious vomiting in a child potentially portends the dire emergency of intestinal malrotation with volvulus, necessitating prompt surgical management, with...
BACKGROUND Bilious vomiting in a child potentially portends the dire emergency of intestinal malrotation with volvulus, necessitating prompt surgical management, with differentials including small-bowel atresia, duodenal stenosis, annular pancreas, and intussusception. Although the upper-gastrointestinal series (UGI) is the diagnostic investigation of choice, up to 15% of the studies are inconclusive, thereby posing a diagnostic challenge. CASE REPORT We report a case series of 3 children referred for bilious vomiting, whose initial UGI was inconclusive and who were eventually confirmed to have intestinal malrotation at surgery. The first child was a female born at 37 weeks with antenatally diagnosed situs inversus and levocardia, who developed bilious vomiting on day 1 of life. The duodenojejunal flexure (DJ) could not be visualized on the UGI because of faint opacification on first pass of the contrast and subsequent overlap with the proximal jejunal loops. The second child was a male born at 36 weeks, presenting at age 4 months with bilious vomiting of 2 days duration. The third child was a female born at 29 weeks, presenting with bilious aspirates on day 3 of life. UGI for all 3 showed persistent hold-up of contrast at the proximal duodenum with no opacification of the distal duodenum or small bowel.Adjunctive techniques during the UGI and ultrasound examination helped achieve a preoperative diagnosis of malrotation in these children. CONCLUSIONS Application of diagnostic adjuncts to an inconclusive initial UGI may help elucidate a preoperative diagnosis of intestinal malrotation in infantile bilious vomiting.
Topics: Female; Humans; Infant; Infant, Newborn; Male; Duodenum; Intestinal Atresia; Intestinal Volvulus; Nausea; Vomiting
PubMed: 38483097
DOI: 10.12659/AJCR.943056 -
Frontiers in Pediatrics 2024A congenital duodenal diaphragm (CDD) is a rare cause of duodenal obstruction in infants and children. The traditional approach to treatment has been surgical...
BACKGROUND
A congenital duodenal diaphragm (CDD) is a rare cause of duodenal obstruction in infants and children. The traditional approach to treatment has been surgical intervention either with excision and duodenoplasty or with bypass through a duodenoduodenostomy, which is invasive and carries the risk of anastomotic leakage.
AIMS
To summarize the key points of differential diagnosis and treatment of a CDD under gastroscopy and to evaluate its efficacy and safety.
METHODS
The clinical data of patients with a duodenal obstruction diagnosed and treated by gastroscopy in our hospital between January 2019 and December 2022 were retrospectively analyzed. The gastroscopic features of the CDD were summarized. The clinical outcomes of patients with CDD treated by gastroscopy were collected and investigated.
RESULTS
A total of 13 children with a duodenal obstruction underwent a gastroscopic examination and/or treatment, and of these, 10 were diagnosed as having a CDD and 3 were finally diagnosed as having an annular pancreas. A dome-shaped structure during inspiration (9/10) and a guidewire that could be inserted through the opening into the distal lumen (6/10) were the gastroscopic features of the CDD, while the annular pancreas had none of these features. The 10 patients, 4 men and 6 women with the CDD, were treated through endoscopic diaphragmotomy and dilation. The age and weight at operation was 15 days to 7 years (M: 2.25 years) and 2.85-22 kg (M: 13.6 kg), respectively. A total of 11 endoscopic operations were performed in the 10 patients. The time of single operation was 15-55 min (M: 38 min). The patients did not experience complications such as bleeding, pneumoperitoneum, and duodenal papilla injury during the operation. Feeding was started 12-24 h after surgery, and the patients were discharged 2-10 days (M: 7 days) postoperatively. The patients were followed up for 3-36 months (M: 17 months), and none of them had a recurrence of vomiting. However, three showed a recurrence of stenosis in upper gastrointestinal imaging, one of whom underwent a partial diaphragm resection again 2 months after the primary treatment.
CONCLUSIONS
A CDD can be confirmed by endoscopy based on its characteristic features. Endoscopic diaphragmotomy and balloon dilation are safe and effective, which can be a new option for minimally invasive treatment of a CDD.
PubMed: 38445081
DOI: 10.3389/fped.2024.1298748 -
Radiology Case Reports May 2024Etiologies underlying the relatively infrequent third-trimester sonographic depiction of dilated fetal bowel include (functional or mechanical) bowel obstruction,...
Etiologies underlying the relatively infrequent third-trimester sonographic depiction of dilated fetal bowel include (functional or mechanical) bowel obstruction, intestinal atresia, volvulus, annular pancreas, intestinal malrotation, intussusception, gastrointestinal duplications, cystic fibrosis-associated meconium ileus, congenital chloride diarrhea, microvillus inclusion disease, intestinal neuronal dysplasia, and meconium plug syndrome. Fetal bowel obstruction may be associated with aneuploidy (mostly Trisomy 21 in association with esophageal or duodenal atresia), and rarely select microduplications or deletions. We present unusual sonographic findings associated with transient marked proximal fetal bowel dilatation in association with concurrent development of oligohydramnios, in a growth-restricted fetus at 35 weeks' gestation. This case supports that upon observation of dilated loops of fetal bowel, while not negating the potential need for delivery secondary to potential bowel compromise, consideration should be given for observation in anticipation of potential spontaneous resolution of this condition, especially among growth-restricted fetuses with decreased amniotic fluid volume in prematurity.
PubMed: 38384702
DOI: 10.1016/j.radcr.2024.01.069 -
Journal of the Korean Society of... Jan 2024Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while... (Review)
Review
Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while nonspecific symptoms such as recurrent abdominal pain may be present in older children. Malrotation can be associated with duodenal obstruction caused by an abnormal peritoneal fibrous band or congenital anomalies, such as an annular pancreas or a preduodenal portal vein. Volvulus can lead to bowel ischemia and a life-threatening condition, thus prompt and accurate diagnosis is crucial. Diagnosis can be made through upper gastrointestinal series, ultrasonography, and CT, with ultrasonography being preferred as a screening tool due to its rapid and accurate diagnosis, without radiation exposure, in children. This pictorial essay discusses the key imaging findings and diagnostic approaches for malrotation and midgut volvulus, as well as diagnostic pitfalls based on actual cases.
PubMed: 38362395
DOI: 10.3348/jksr.2023.0002 -
Journal of the Belgian Society of... 2024A complete annular pancreas, a rare congenital anomaly, can lead to duodenal obstruction, causing recurrent symptoms like vomiting and often requiring surgical...
A complete annular pancreas, a rare congenital anomaly, can lead to duodenal obstruction, causing recurrent symptoms like vomiting and often requiring surgical intervention for relief.
PubMed: 38312145
DOI: 10.5334/jbsr.3432 -
HPB : the Official Journal of the... Apr 2024The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum.
METHODS
The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP.
RESULTS
A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%).
CONCLUSION
The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.
Topics: Adult; Child; Humans; Prevalence; Pancreas; Pancreatic Diseases; Duodenal Obstruction; Digestive System Abnormalities
PubMed: 38310080
DOI: 10.1016/j.hpb.2024.01.006 -
Gastrointestinal Endoscopy Jul 2024
Topics: Humans; Pancreatic Ducts; Recurrence; Pancreatitis; Acute Disease; Male; Cholangiopancreatography, Endoscopic Retrograde; Choristoma; Female; Middle Aged
PubMed: 38253210
DOI: 10.1016/j.gie.2024.01.018 -
Journal of Indian Association of... 2023Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful...
Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful management of a child with BEF discovered as an intraoperative surprise and associated with the short gut, annular pancreas, and disorder of intestinal fixation.
PubMed: 38173639
DOI: 10.4103/jiaps.jiaps_79_23