Abdominal Masses in Infants and Children

Approximately 60% of abdominal masses in children are due to non-surgical diseases. These are; Leukemia, Lymphoma, cirrhosis, Portal Hypertension, and enlargement of the Liver and Spleen due to other systemic diseases. The remaining approximately 40% are due to diseases that require surgery. These masses can be divided into tumoral masses and non-tumoral masses. Most of the masses are located retroperitoneally. Approximately 2/3 of retroperitoneal masses are due to renal and 1/3 to non-renal causes.

Approximately 65% of the masses seen in the neonatal period are retroperitoneal and 35% are intraperitoneal. Also 90% of retroperitoneal masses are of renal origin.

The most common renal masses encountered in the neonatal period are as follows; The most common renal masses of benign origin encountered in the neonatal period include pathologies such as multicystic kidney, in-fantile polycystic kidney, hydronephrosis due to ureteropelvic obstruction, renal vein thrombosis (RVT), mesoblastic nephroma, and adrenal hemorrhage.

Malignant tumors of this period (13%) include neuroblastoma, Wilms tumor, sacrococcygeal teratomas with presacral location or presacral extension, and malignant retroperitoneal teratomas.

Benign Kidney Masses in Newborns and Infants;

• Multicystic kidney
• Polycystic kidney
• Hydronephrosis
• Pyonephrosis
• Renal vein thrombosis

Tumoral Kidney Masses in Newborns and Infants;

• Wilms tumor
• Neuroblastoma
• Intrarenal teratoma

Non-renal benign masses seen in newborns and infants

• Intestinal duplications
• Mesenteric cyst
• Mid-intestinal volvulus
• Meconium pseudocyst
• Hydrocolpos
• Ovarian cysts
• Choledochal cyst
• Mesenteric lymphangioma
• Adrenal hemorrhage

Non-renal tumoral masses seen in newborns and infants;

• Neuroblastoma
• Retroperitoneal teratoma
• Neuroblastoma
• Ganglioneuroma
• Ganglioneuroblastoma
• Presacral teratoma
• Mesoblastic nephroma

Most of the diseases listed so far have been mentioned in their own topics. Here, we will talk about topics that we have not examined under another heading. These are; Gastrointestinal Duplication Cysts, Mesenteric cysts, Hydrocolpos, Adrenal hemorrhage.

Gastrointestinal Duplications

Duplication is defined as two of the same or similar anatomical structures (doubling). 
A gastrointestinal duplication is an abnormal part of the intestine that is attached to or directly related to the normal intestine. In other words, it is a structure that looks like a normal intestine but is not really an intestine.
This is also called a duplication cyst. This duplication cyst or duplicated part of the intestine is usually covered with intestinal cells.

 

 

Why do gastrointestinal duplications occur, what is the cause?

Although there are some theories about its formation, it is not known for certain.

Which part of the digestive system is affected by intestinal duplications?

Duplications of the digestive tract can occur in any part of the esophagus, stomach, small and large intestine.
They vary in length and size. They can be spherical or in the shape of a tube. The cell structure is very similar to normal intestinal cells. The main features are as follows;

• The presence of a well-developed smooth muscle sheath
• Covered with an epithelium similar to the epithelium of part of the digestive tract
•  In close contact with (adjacent to) the gastrointestinal tract.

Duplications

• Cystic
• They can be tubular shaped

When and how often is intestinal duplication seen?

It occurs in 1 in 9000 births, so it is quite rare. One third of patients are diagnosed in the neonatal period. They are more common in white males. There is no familial incidence.

In which anatomical regions are gastrointestinal duplications found?

Duplications can be found anywhere in the digestive tract from the mouth (tongue) to the anus. Duplications are usually located paramesenterically. This distinguishes them from diverticula (e.g. Meckel's). Diverticula, on the other hand, are located antimesenterically. About 45% of duplications occur in the jejunu-ileal, 20% in the esophagus, 15% in the colon and the rest in other gastrointestinal areas. Small intestine (jejunoileal) duplications are most common. Duodenal duplications (intraabdominal ones) are the least common.

• Most common, Small intestine (ileum)
• Least common, Cervicol

Synchronized duplication is present in approximately 5-15% of cases.

What are the General Characteristics of Gastrointestinal Duplications?

They may be cystic or tubular, they are located in the meseneteric part of the intestine, they are more common in males, clinical findings vary according to the size and anatomical localization of the duplication, some are asymptomatic and do not show symptoms until adulthood, if left untreated, they may become cancerous in adulthood, additional anomalies are common.

What are the Associated Anomalies of Gastrointestinal Duplications?

Associated anomalies are common: Vertebral anomalies are seen in almost all cases, intestinal atresia, malrotation, urinary system anomalies are other anomalies.

• Many researchers have found an association between vertebral anomalies (bifid or fused spine) and thoracic and thoracoabdominal duplications. This is consistent with the “Split Notochord”, one of the proposed mechanisms of embryogenesis of duplications.
• Enteric cystic duplications have been documented in association with intestinal atresia or malrotations.
• Tubular end-intestinal duplications are frequently associated with genitourinary and genital malformations.

What are the clinical findings of gastrointestinal duplications and how are they diagnosed?

With the development of prenatal follow-up and diagnosis, the diagnosis is made by ultrasonography performed in the womb in some patients. Postnatal clinical findings vary according to the location and size of the duplication and the presence of heterotopic gastric mucosa. 67% of cases are diagnosed before the age of 1 year and 85% before the age of 2 years.

Some of the duplications are detected during routine ultrasonographic examination performed after birth. In some of them; pain, signs of obstruction in the digestive system and bleeding may also occur. Symptoms may occur due to stretching with the enlargement of the duplication, or they may cause various complaints by compressing other neighboring organs.

Ulcers, severe bleeding and perforation may occur with acid release of different cells (e.g. gastric cells) within the duplication. Pyloric and duodenal duplications may mimic hypertrophic pyloric stenosis. Intestinal duplications may provide a focal focus for volvulus or invagination.

If within the rib cage, they may cause dysphagia by compressing the esophagus and respiratory distress or difficulty by compressing the trachea.

Colonic or presacral duplications may cause constipation, prolapse or a picture mimicking perirectal abscess.

How is Duplication Diagnosed?

The main basis of the diagnosis is history and physical examination. While the above-mentioned complaints are present in the history, findings migrating to the region where the duplication is located are detected in the systemic examination. Physical examination may be normal or coarsening of respiratory sounds, inability to swallow, palpable mass may be detected.

Imaging methods include direct radiographs, ultrasound, contrast-enhanced radiographs, computed tomography, magnetic resonance imaging and endoscopy.

The characteristic “Enhancing Rim” (i.e., a clear edge-wall-wall) on computed tomography is important in the diagnosis of duplication. It helps to differentiate them from neoplasia.

Contrast-enhanced studies show the “mass” effects of duplications and the “displacements” they cause in the normal anatomical structure. Technetium scintigraphy is important to show heterotopic gastric mucosa in cases where bleeding is predominant. Ultrasonography is helpful in the diagnosis of duplication. It is also very useful in the detection of associated genitourinary anomalies in colonic duplications.

Most intestinal duplications are diagnosed at surgery.

How are gastrointestinal duplications treated?

The treatment is surgical removal of the duplication cyst. Removal may sometimes require staged surgery. The surgical method depends on the part of the organ where the duplication is located, its size and the general condition of the patient. Cysts located in the thoracic cavity are removed with thoracic surgery. Cysts originating from the digestive system in the abdomen are removed with abdominal surgeries. Depending on the location of the cyst, it may also be necessary to remove a section of the intestine, in which case the intestine is reconnected and its continuity is ensured. During the operation, depending on the proximity of the mass, there may be damage to the intestine and esophagus, which are repaired.

Can Duplication Surgeries Be Performed Closed?

Yes, this method has many advantages. You can read our article on Laparoscopy on the subject.

1.Cervical Duplications:

It is the least common type of gastrointestinal duplications. Cases are usually diagnosed under the age of 5 years, most of them under the age of 1 year. They usually present with dysphagia and dyspnea and sometimes with swelling in the neck. Recently, some cases may be diagnosed in the womb.

2.Thoracic or Thoracoabdominal Duplications:

It constitutes 25% of all gastrointestinal duplications. In 1/3 of thoracic duplications there is a second or third duplication cyst under the diaphragm. Vertebral anomalies are present in almost all patients. CNS involvement may be present. Heterotopic gastric mucosa is present in 1/3 of thoracic duplications.

Clinical signs of dysphagia and dyspnea are usually seen. Recently, some of the cases may be diagnosed in utero.

Lower cervical and upper thoracic esophageal duplications associated with the spinal cord are also called neurocentric cysts.

 

 

3.Gastric Duplications:

Gastric duplications are usually cystic. They are located in the greater curvature and have no relationship with the stomach. In most cases, vomiting and mass occur at < 1 year of age. The diagnosis is easily made with upper gastrointestinal system study. The mucosa of the cyst is often gastric. Technetium scintigraphy is helpful in the diagnosis.

 

 

4.Duedenal Duplications:

It is the least common of all intraabdominal duplications. It is difficult to treat because of its proximity to bile and pancreatic ducts. Duodenal duplications present with jaundice or pancreatitis with signs of upper GI obstruction.

Approximately 15% of these have mucosa. They are located on the medial margin of the 2nd continent of the duodenum. Differential diagnosis includes choledochal cysts and pancreatic cysts.

5.Jejunoileal Duplications:

The most common site of duplications is the small intestine (especially ileum). Most of the duplications are cystic. But they may also be tubular. They are located on the mesenteric margin. They may be confused with mesenteric cysts. They may cause invagination or volvulus. They may contain heterotopic gastric mucosa.

 

6.Large Intestine (Colonic) Duplications:

Colonic duplications account for 20% of gastrointestinal duplications. These cysts may be isolated only in the colon or may be fistulous to the perineum and urinary system. They can be classified according to their anatomical leukalization and shape.

• Type I (Ia, Ib, Ic): Cystic > above the perineal reflexion.
• Type II (IIa, IIb, IIc): Tubular > Below the peritoneal reflection.

 

 

7.Rectal Duplications

Rectal duplications are always located in the retrorectal space. In 45% of cases they open as fistulas into the anal canal or perineum.  Split notochord theory is valid in their embryogenesis due to the association of spinal anomalies.

Which diseases should be considered in the differential diagnosis of duplication cysts?

• Anterior sacromeningomyelocele
• Dermoid cyst
• Sacrococcygeal teratoma
• Chordoma
• Leomyosarcoma
• Carcinoma.

These are the main pathologies to be considered

How long will my child stay in the hospital after surgery?

After the surgery, the normal functioning of the intestines and normal defecation time varies between 2-3 days. During this period, they are supported with serum and antibiotic treatments continue. After bowel movements start and normal defecation occurs, feeding begins. Separation from the IV and full oral feeding is completed in 5-6 days and the patient can be discharged.

However, this situation is valid for isolated and limited cysts. The situation may be different for complicated cases. In this case, the doctor's advice and information are important.

Mesenteric Lymphangioma
What are Lymphangiomas (Lymphovenous Malformations)?

Lymphangiomas are an anomaly characterized by dilatation of the lymph vessels caused by the interruption of the lymphatic system. Today, it is called Lymphatic Malformation. It is the second most common benign vascular tumor of childhood. The incidence is approximately one in 60 000 infants. The incidence is equal in boys and girls. There is no familial predisposition.

What is the lymphatic system and what is its function?

The lymphatic system or lymphatic system is an organ system consisting of the lymph transport system and lymphatic tissue (lymph gland). The lymphatic system, which can be defined as a second circulatory system, is still very different from the circulatory system in terms of its structure. It works independently of the circulatory system, but at the end it joins the general systemic circulation through the veins. In general, it has two tasks: to transfer inter-tissue fluids to the general circulatory system and to contribute to the body's immune system.

What is Mesenteric Lymphangioma?

Lymphangiomas located on the mesosoma of the intestines (the structure where the intestinal vessels are located) in the abdomen. More information about lymphangiomas can be found in the Lymphangioma Topic.

 

 

How is Mesenteric Lymphangioma Diagnosed?

The diagnosis is made with history and physical examination. Cystic swelling in the abdomen is seen on Ultrasonography performed in the womb and is suspected.

Mesenteric Lymphangiomas may be asymptomatic most of the time and are detected on ultrasonography performed during examination for other reasons. Sometimes it may cause recurrent abdominal pain, very rarely it may cause acute abdomen due to bowel rotation, intussusception or perforation and emergency surgery may be required.

How is mesenteric lymphangioma treated?

Treatment involves medication or surgical removal. The location and structure of the cyst and the age of the patient determine which one will be applied. If the cyst is small and can be removed without damaging neighboring organs, it can be excised. Surgical and drug treatment of mesenteric lymphangiomas located in the abdomen can be performed by laparoscopy or open surgery.

In addition, drug treatment may be preferred in recurrent cases after surgery. Local drug treatment can be applied especially in cysts that are widespread and surrounding neighboring organs, which should be preferred. In recent years, successful results have been reported with bleomycin and picibanil (OK-432) sclerosing therapy in the treatment of lymphangioma. Sometimes laser or even radiation therapy may be required in these patients. However, the last two treatments have serious complications. The choice of treatment option is determined by the structure and location of the cyst, the age of the patient, the relationship of the cyst or cysts to vital organs and the experience of the physician.

What are the complications of surgical removal of mesenteric lymphangioma and drug treatment?

When surgically removed; If the cyst or cysts cause vital organ injury or nerve damage as a result of infiltration, long-term lymphatic drainage requirement, infection, recurrence, complications may occur. In addition, postoperative recurrence is around 5-15% in cases. It may not be possible to remove the entire cyst or cysts, especially in multiple cases.

There are no serious complications of OK-432 yet. However, Bleomycin may have serious complications. These complications may occur in the short or long term and you should be informed by your physician. However, since the doses administered in these patients are very low, these complications are almost non-existent.

*** The information provided here, the content of the website is organized for the purpose of informing the visitor, especially the families. No information should be considered as advice by visitors and should not lead to any decision or action. Families should definitely have their patient examined by a pediatric surgery specialist, consult with him/her and make a decision by consulting his/her one-on-one information.