An effective method for preoperative distinguishing Gastric duplication cysts and Adrenal cysts in children

doi:10.21203/rs.3.rs-2506621/v1

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An effective method for preoperative distinguishing Gastric duplication cysts and Adrenal cysts in children

https://doi.org/10.21203/rs.3.rs-2506621/v1

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Background: Gastric duplication cysts (GDC) are a rare congenital disease primarily found in the left adrenal region in neonates and children. Due to a lack of typical clinical manifestations, GDCare often preoperatively treated as adrenal cysts (AC).

Methods: We retrospectively report six child cases of retroperitoneal cysts presenting as left adrenal masses between January 2012 and January 2022. Patients aged from 10 months to 10 years, including three females and three males, were all treated by surgery and discharged without serious complications. Clinical data such as medical history, clinical manifestations, auxiliary examination, operation process, and postoperative pathological diagnosis were examined in detail.

Results: Only one male had mild epigastric pain that could be relieved spontaneously, and the rest had no obvious clinical manifestations. Endocrinological evaluations indicated no positive findings. The stomach-cyst distance in computerized tomography (CT) scans and cyst-kidney distance in ultrasound scans were significantly different in the patient with GDC. All patients underwent surgical resection and were preoperatively diagnosed with a left AC. One patient underwent open cystectomy, and the others underwent laparoscopic cystectomy. Surgical access was transperitoneal in five patients and retroperitoneal in one patient. Mean operative time was 129.7±15.46 min, mean oral feeding time was 9.0±2.58hours, and mean length of hospital stay was 6.5±0.96 days. No serious complications were observed, and their pathological diagnosis was AC, except for one case of GDC. All patients recovered and had no complications during follow-up.

Conclusion: GDC is easily misdiagnosed as left AC.Accurate diagnoses are mainly based on intraoperative findings and postoperative pathological examinations. However, in addition to gastrointestinal symptoms, such as abdominal pain and vomiting, the stomach-cyst distance in CT scans and cyst-kidney distance in ultrasound scans can be used as critical evidence for preoperative diagnosis GDC or AC. Laparoscopic surgery is safe and effective, and a transperitoneal approach is recommended for GDC.

Gastric duplication cyst

Adrenal cyst

Preoperative diagnosis

Pediatric surgery

Gastic duplications are a rare congenital malformation primarily found in neonates and children, with an incidence of 1 in 100,000 live births [1]. Gastric duplication cysts (GDC) are extremely rare, accounting for 4–6% of gastric duplication malformations [2]. Patients with GDC are often admitted to the pediatric surgical ward for cystic lesions in the left adrenal area. Notably, due to a lack of typical symptoms and clinical manifestations, GDC are usually misdiagnosed as adrenal cysts (AC) before surgery [3]. Furthermore, the final diagnosis is still based on the surgical findings and pathological examination [4]. An unclear diagnosis may affect the surgical plan and increase surgery risk. Therefore, this study focused on the specific symptoms and indicators in preoperative examinations to help pediatric surgeons to improve their understanding of GDC, reduce the preoperative misdiagnosis rate, and better formulate surgical plans to reduce the operation risk.

This retrospective study was approved by the ethics committee of an affiliated hospital of Zunyi Medical University (Zunyi, Guizhou Province, People’s Republic of China). We identified six child cases from the diagnosis medical record index with retroperitoneal cysts presenting as left adrenal masses admitted to the Pediatric Urology Ward between January 2012 and January 2022. The patients were aged 5 months to 10 years (median of 4.4 years), including three females and three males (Table 1).

Table 1

Clinical GDC/AC characteristics.
Case	1	2	3	4	5	6
Gender	Female	Male	Female	Female	Male	Male
Age (years)	10 Mon	6	3	10	2	5
Mass location	Left adrenal region	Left adrenal region	Left adrenal region	Left adrenal region	Left adrenal region	Left adrenal region
Clinical symptoms	Asymptomatic	Abdominal pain	Asymptomatic	Vomiting	Diarrhea	Asymptomatic
Endocrinological examination	Normal	Normal	Normal	Normal	Normal	Normal
Tumor-related biomarkers	Normal	Normal	Normal	Normal	Normal	Normal
Preoperative diagnosis	AC	AC	AC	AC	AC	AC
Surgical procedure	Open	Laparoscopic	Laparoscopic	Laparoscopic	Laparoscopic	Laparoscopic
Surgical access	Transperitoneal	Transperitoneal	Transperitoneal	Transperitoneal	Transperitoneal	Retroperitoneal
Operation time (min)	158	125	125	140	120	110
Oral feeding time (h)	14	10	9	8	7	6
Length of hospital stay (d)	8	7	6	7	6	5
Complications	N/A	N/A	N/A	N/A	N/A	N/A
Pathological examination	AC	GDC	AC	AC	AC	AC

All patients underwent ultrasound examination in the Outpatient Department. After admission, endocrinological evaluation, blood pressure measurement, and tumor-related marker examination were performed to exclude cystic pheochromocytoma and other adrenal gland tumors, such as cystic teratoma. In addition, computerized tomography (CT) scans were performed routinely to clarify the relationship between the cyst and the surrounding tissues and blood vessels to improve the formulation of the surgical plan.

The pathological diagnosis criteria for GDC were: (1) A GDC cyst wall containing smooth muscle tissue similar to gastric wall smooth muscle; (2) Attachment of the gastric epithelium or other intestinal mucosa epithelial tissue types to the cyst wall. The pathological diagnostic criteria for AC were: (1) A smooth and glistening AC surface with an internal lining varying from smooth to slightly rough; (2) An AC wall containing fibrous tissue instead of smooth muscle [5–7].

Patient Characteristics

The clinical presentation of all patients was atypical. A 6-year-old male presented with abdominal pain without apparent cause and was admitted with a cyst in the left adrenal gland. Body temperature was normal, and there was no bloody stool. Abdominal pain was mostly intermittent and located mainly in the upper abdomen, and accompanied by acid reflux, belching, and occasional vomiting. It could be relieved on its own, had no obvious relationship with eating, and was not present at night. Physical examination showed normal growth, development, and body hair, and no central obesity signs such as a full moon face. Cardiopulmonary examination showed no abnormalities. There was deep tenderness under the xiphoid process in the abdomen, but no definite mass was detected by touch, and no abnormalities were present in other regions. The other five patients had no significant gastrointestinal symptoms or other relevant medical histories, such as hypertension or abnormal hypercortisolism signs. All patients were diagnosed with an AC mass during their outpatient physical examination.

Blood count, amylase, serum liver, and renal function tests were performed and within the normal range. No abnormalities were found in routine testing of tumor-related (e.g., alpha-fetoprotein and neuron-specific enolase), endocrinological (e.g., dopamine, epinephrine, and norepinephrine), and plasma (e.g., metanephrine, 3-methoxytyrosine, metanephrine, and methoxy- norepinephrine) biomarkers (Table 1).

Ultrasonography has the benefits of non-invasiveness, low cost, and no radiation and is used routinely in early screening examinations of abdominal space-occupying lesions in outpatient clinics. The direction of the ultrasound probe was varied during ultrasound examinations. The cyst wall was closely related to the ipsilateral kidney (Table 2). There was no obvious difference in distance between the two tissue structures in the AC group. However, the cyst wall was thicker, and the cyst-kidney distance was more obvious in the GDC patient than in the AC group (Fig. 1A-B).

Table 2

Changes in cyst location with ultrasonography or CT examination.
Case	Pathological examinations	Cyst-kidney distance in ultrasonography (mm)	Cyst-stomach distance in CT scan (mm)
1	AC	0	10
2	GDC	15	0
3	AC	0	8
4	AC	0	12
5	AC	0	9
6	AC	0	7

CT scans can show the relationship between cysts and surrounding tissues from the sagittal and horizontal planes. Enhanced scans can be used to determine whether the cyst components are enhanced and understand cyst structure and the relationship between the cyst and surrounding tissue components. The cyst components in each patient were homogeneous in the plain CT scan. No cyst component enhancement was found in the enhanced CT scan. Notably, there was an apparent stomach-cyst distance in the AC group (Table 2). However, the gastric wall was close to the cyst with no apparent distance between them in the GDC patient in both the horizontal and coronal CT direction (Fig. 2A-H).

Operative Treatments

All six patients were operated on as AC. One underwent open surgery, and the other five underwent laparoscopic surgery. Surgical access was transperitoneal in five patients and retroperitoneal in one patient. During the operation, the adrenal region was explored first. Five patients were confirmed to have AC, with cysts close to the upper pole of the kidney. The other patient had an intact adrenal gland with no cyst during exploration. The exploration continued upward to the lower edge of the greater curvature of the stomach based on the preoperative CT scan, where a cyst was found. Blunt dissection of the cyst showed the cyst wall was close to the greater curvature of the stomach, and located in the muscular layer of the gastric wall. After the cyst was completely removed, we found the cystic cavity did not interact with the gastric cavity, and the gastric wall was sutured with an absorbable suture (Fig. 3A-D).

Cysts were completely removed in all cases. The mean operative time was 129.7 ± 15.46 min. One AC case was successfully excised using the retroperitoneal laparoscopic technique with an operative time of 110 min. The GDC case used the transperitoneal approach, involving a longer intraoperative exploration time, with an operative time of 125 min. However, there was no significant difference in operative time between GDC and AC cases.

The mean time to oral intake was 9.0 ± 2.58 hours. The mean hospital stay was 6.5 ± 0.96 days(Table 1). No serious complications were observed, and a liquid diet was resumed the day after the operation. All six children in this study had no postoperative complications and were discharged from the hospital with full recovery. In the outpatient follow-up of 1 month, 3 months and 6 months, the surgical area healed well and the abdominal pain, vomiting and diarrhea disappeared completely.

Postoperative general and microscopic pathological findings showed a grey-red capsule wall tissue macroscopically. Smooth muscle on the cyst wall, covered with cubic or short columnar epithelium, a few lymphocytes and plasma cells below the epithelium, and dilated and congested interstitial blood vessels were seen microscopically.

GDC are a particular type of gastrointestinal tract cyst that occurs mostly in infants and young children [8]. GDC can be divided into tubular cysts (20%) and cystic cysts (80%) based on their morphological features. Tubular cysts are connected to the gastric cavity, while cystic cysts are not [9]. GDC can occur anywhere in the stomach but are mainly found in the pylorus near the greater curvature of the stomach[10]. Because GDC are often found in the left adrenal region and lack specific symptoms and auxiliary examinations, GDC patients are often admitted to the hospital for an AC and incorrectly undergo surgical AC treatment [11–12].

The clinical symptoms of GDC vary according to their size and location [13]. When the cyst is located in the greater curvature of the stomach near the pylorus, epigastric discomfort, nausea, and vomiting may occur. If it interacts with the gastrointestinal tract, symptoms such as gastrointestinal bleeding may also occur [14]. Vomiting and epigastric discomfort are the most common symptoms [15]. The GDC symptoms in this study were only intermittent abdominal pain in the upper abdomen, which often does not attract parental attention, leading to delayed treatment and complicating surgery. Notably, most of Adrenal cysts are clinically asymptomatic and are found accidentally, a few larger adrenal cysts can produce compression symptoms, such as distension and pain in the lower back and abdomen and gastrointestinal discomfort, which is only detected during surgical exploration[16]. Therefore, the clinical presentation of both diseases is atypical, which makes them extremely easy to misdiagnose clinically.

Abdominal ultrasonography is an important method for early GDC detection but lacks specificity for GDC [17]. Recent studies have shown that endoscopic ultrasonography (EUS) and fine needle biopsy are more sensitive for preoperative GDC diagnosis [18]. However, therapeutic EUS uses in pediatrics focus on endoluminal therapy for pancreaticobiliary disease, eosinophilic esophagitis, inflammatory bowel disease, and liver disease. In addition, the complexity of the surrounding tissues of cysts requires sedation or even anesthesia for routine puncture examination. Therefore, this approach can only be qualitative and cannot remove cysts, limiting its clinical application [19].

CT examination can determine the cyst’s location, size, and relationship to surrounding tissue but cannot be qualitative. Some studies have found that under enhanced CT, lesions with thicker cyst walls can appear as “halo signs.” The capsule wall mucous membrane forms the inner low-density ring, and smooth muscle forms the outer high-density ring. However, this typical “halo sign” can only be seen with high-definition equipment, limiting its application [10, 20].

After a detailed analysis of the patients’ preoperative ultrasound and CT results, we found that the cyst’s location was significantly abnormal in those with GDC or AC. Ultrasound showed that the distance between the cyst wall and the upper pole of the left kidney was significantly greater in GDC than in AC patients. In addition, in the CT results, while the GDC wall was close to the stomach wall, the distance between the AC wall and the stomach was significantly greater. Moreover, the GDC was closer to the greater curvature of the stomach and farther from the upper pole of the kidney. Given that the relationship between probe and body position will cause errors in the results, the patient was placed in the supine position, and the analyses are based on the horizontal position data of the CT scan. Ultrasound has changes in probe direction and position and is only used to supplement CT results. However, based on the results, ultrasound observations were completely consistent with CT observations. Therefore, ultrasound and CT examinations can show the positional changes of the cyst, the gastric wall, and the upper pole of the left kidney, allowing the surgeon to distinguish between GDC and AC before surgery.

Currently, transperitoneal or retroperitoneal approaches can be used in surgery for masses in the adrenal region [21]. Since the surgical sites of GDC are in the abdominal cavity near the greater curvature of the stomach, a retroperitoneal approach is not conducive to exposing the cyst. The transabdominal approach can better expose the upper renal pole and the greater curvature of the stomach and avoid injury to surrounding tissues or peripheral organs, such as the pancreas and spleen. Therefore, the transabdominal approach is more suitable for GDC resection. However, the retroperitoneal approach is recommended for patients with ACs since it interferences less with the intraperitoneal bowel and better exposes the adrenal gland than the intraperitoneal approach.

In this study, one AC case chose the retroperitoneal approach instead of the transabdominal approach. Notably, compared with the transperitoneal approach, the operation time, oral feeding time and hospital stay length were shorter in the retroperitoneal approach. Therefore, if GDC is considered before surgery, we recommend the transperitoneal approach, which can obtain better space and field of vision and reduce surgery risk and operative time. If AC is considered before surgery, the retroperitoneal approach is also suitable.

This retrospective study preoperatively diagnosed GDC and AC based on comprehensive ultrasound and CT examination analyses. Cysts closer to the stomach wall and far from the left adrenal gland are more likely GDC. Laparoscopic cystectomy is a safe and reliable surgical method, and a transabdominal approach is recommended for GDC.

Conflict of interest

None.

Declaration of funding

This study was supported by the National Natural Science Foundation of China (grant no. 82060277), the Guizhou provincial Health Committee support program (grant no. gzwjkj2019-1-005), and the Doctoral Startup Fund of the Affiliated Hospital of Zunyi Medical University, Project Number: 2020-04.

Ethics approval and informed consent

The studies involving human participants were reviewed and approved by the Ethics Committee of the Affiliated Hospital of Zunyi Medical University.Written informed consent to participate in this study was provided by the participants' legal guardian.

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No competing interests reported.

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An effective method for preoperative distinguishing Gastric duplication cysts and Adrenal cysts in children

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Abstract

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Introduction

Materials And Methods

Results

Patient Characteristics

Operative Treatments

Discussion

Conclusions

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References

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