Lymphatic malformations are benign congenital malformations of the lymphatic system that occur predominantly in children. Most lymphatic malformations occur in the head and neck region, with those in abdominal locations, such as the mesentery, omentum and retroperitoneum, being less common, accounting for fewer than 5% of lymphatic malformations in children. This study analyzed the clinicopathologic characteristics and treatment outcomes of abdominal lymphatic malformations in children.
The medical records of 12 pediatric patients treated for abdominal lymphatic malformations at our institution between April 1999 and September 2017 were retrospectively reviewed. Demographic and clinical characteristics, including gender, age, symptoms and signs, diagnostic modalities, and treatment results, were analyzed.
The 12 patients included 11 boys and 1 girl, ranging in age from 3 months to 17 years (median 36.5 months) at presentation. The primary signs and symptoms included abdominal pain, abdominal mass and abdominal distention. Other symptoms and signs included fever, vomiting, scrotal pain and mass, and right inguinal mass. All patients were diagnosed by abdominal ultrasonography and computed tomography, and all underwent surgical excision with or without bowel resection. The lymphatic malformations occurred in the retroperitoneum (n=4), omentum (n=4), jejunal mesentery (n=2), and retroperitoneum and mesentery (n=2). Seven patients underwent complete mass excision, including 3 who underwent laparoscopic excisions, and 3 who underwent mass excision with segmental resection of the adjoining bowel. Two patients underwent incomplete excision because the lesion was extensive and invaded the superior mesenteric vessels. There was no major perioperative morbidity in any patient. At a median follow-up of 50 months (range, 8–183 months), only 1 patient experienced recurrence.
Although abdominal lymphatic malformations are benign, most children present with acute abdominal symptoms, necessitating early surgical treatment.
Single-port laparoscopy-assisted surgery is being performed for various operations in pediatric patients recently. The aims of this study were to prove the safety and find well-matched indications of small bowel resection using single-site umbilical laparoscopic surgery (SSULS).
From 2011 to 2016, 29 pediatric patients underwent SSULS. Medical records were retrospectively reviewed.
A total of 29 patients were included and 30 SSULS were performed in this study. The mean age at operation was 5.7 years, and the mean weight was 21.9 kg. Meckel's diverticulum was the most common indication for SSULS, followed by small bowel intussusception due to leading point mass, small bowel duplication, and Crohn's disease. In most cases, estimated blood loss was negligible except in Crohn's disease with severe inflammation. While answering post-discharge questions about scars, most parents responded that they were satisfied with the postoperative wound.
SSULS is a useful operation to try even for surgeons who do not have advanced laparoscopic skills. Complication rates of single-port operations do not differ from those of conventional laparoscopic operations. Most lesions of the small bowel could be indications of SSULS. Careful attention is required when performing SSULS in patients with Crohn's disease.
Gardner-associated fibroma (GAF) is a benign lesion of soft tissue which has recently been described and is exceedingly rare in children. GAF is associated with adenomatous polyposis coli gene mutation, familial adenomatous polyposis and desmoid. We report a case of patient with soft tissue tumor on her lower back which was turned out to be GAF. The patient was a 19-month-old female who visited out-patient clinic with palpable mass on her lower back and we performed surgical excision. The tumor was located at subcutaneous and we excised the tumor including surrounding soft tissue. She discharged without any complication on surgery day. The pathologic report showed dense collagenous tissue with spindle cell and adipose tissue, suggestive of GAF. We are planning to check gene study and to perform endoscopy and abdominal ultrasonography for at the age of 4.
A 19-month-old boy suffered from stridor and dysphagia. He was taking asthma medication for a few months, but symptoms did not improve. After admission, a chest CT showed a posterior mediastinal mass, which compressed the trachea and esophagus. The removed mass via open thoracotomy was a bronchogenic cyst on histopathology. Postoperatively, stridor and dysphagia disappeared. In case of persistent and refractory stridor or dysphagia in children, congenital lesions including bronchogenic cyst need to be ruled out.
An 1-month old female newborn was admitted to our hospital because of jaundice which occurred at 2 days after birth. Plain chest X-ray and chest CT revealed a collapsed right middle lobe and lobar emphysema was suspected. Right upper lobectomy of the lung was done and pathologic findings showed an infantile lobar emphysema. After the operation, the newborn was discharged without complication and was followed up through the out patient clinic. Infantile lobar emphysema is rare and male dominant. Left upper lobe of the lung is the most prevalent site. Patients with infantile lobar emphysema complain of respiratory symptoms. We report one case of infantile lobar emphysema on right upper lobe of lung, in a female with no respiratory symptoms.
A one day old female infant was brought to the emergency room suffering from shortness of breath. An x-ray revealed the gastrointestinal tract in the right thoracic cavity. An emergency operation demonstrated eventration of the diaphragm, and a plication was performed. The baby was discharged without complication and has been followed up in the out patient clinic. Congenital diaphragmatic eventuation requiring emergency operation is rare.
Annular pancreas is a rare congenital anomaly with the descending duodenum encircled by a ring of pancreatic tissue, which may cause partial or complete obstruction of the duodenum. In newborn, the symptoms can be those of duodenal stasis resulting from partial intestinal obstruction secondary to some degree of duodenal stenosis. A male newborn weighing 2.0 Kg was born by C-section delivery at 37 weeks' gestation to a 27-year-old mother who had a hydramnios. He was in relatively good condition at birth except regurgitation of saliva and intermittent apnea. A plain film of the abdomen showed the double-bubble of gas filled stomach and proximal duodenum, and upper gastrointestinal series showed a dilated proximal duodenum, with a complete obstruction of the descending duodenum. Intraoperative finding revealed encirclement of the duodenal second portion by pancreatic tissue. Duodenojejunostomy was performed. After the operation, he had developed two serious complications, neonatal septicemia by Enterobacter cloacae on postoperative day 12 and systemic candidiasis on postoperative day 19, and been managed with ventilatory support, antibiotics, and antifungal agents with recovery.
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