25 years' experience with lymphangiomas in children.

BACKGROUND/PURPOSE: The management of lymphangioma in children is challenging because complete resection is difficult to achieve in some cases, and recurrences are common. The authors reviewed their experience to assess the risk factors for recurrence and the role of nonoperative treatment. METHODS: A retrospective study over a period of 25 years was carried out. One hundred eighty-six patients with 191 lesions (five patients with de novo lesions in different sites) were treated. There were 98 boys and 88 girls. The average age at diagnosis was 3.3 years (range, fetal life to 17 years) and the average size 8 cm in diameter. Histocytological confirmation was obtained in all patients. The involved sites were head and neck, 89 patients (48%); trunk and extremities, 78 patients (42%); internal or visceral locations (eg, abdominal and thorax), 19 patients (10%). The treatment consisted of macroscopically complete excision in 145 patients (150 lesions, of which five were recurrences in different sites), partial excision in 10 patients, aspiration in five patients, laser excision in 10 patients, biopsy only in four patients, drainage and biopsy in two patients, and injection of sclerosing agents in 10 patients. RESULTS: There were 54 recurrences; 44 underwent excision (five of them more than once), and five regressed spontaneously on follow-up. Five other recurrences were stable and not progressing. Recurrences, (defined as clinically obvious disease), were found to be 100% after aspiration, 100% after injection, 40% after incomplete excision, 40% after laser excision, and 17% after macroscopically complete excision. The recurrence rate in the last group was the highest in the head (33%), the least in the internal locations (0%), and intermediate for the cervical location (13%). There were no significant differences, in terms of outcome, between those who had their surgery immediately at the time of diagnosis (n = 101) and those who had delayed surgery (n = 85). CONCLUSIONS:There were fewer recurrences after macroscopically complete excision. Aspiration and injection had the highest recurrence rate. Risk factors for recurrence included location, size, and complexity of lesions. A period of observation may be useful for infants to facilitate complete excision. In the present series, spontaneous regression was infrequent and was seen more often with recurrent lesions.     

Mesenteric lymphangiomas of the small intestine in children]

The authors have made an analysis of 15 cases with lymphangiomas of the small intestine mesentery in children aged from 3 days up to 12 years. Attention is called to problems in diagnosing this pathology. The optimum variant of examination is presented which allow the correct diagnosis to be made. All the children were operated upon. The method was chosen depending on localization of the cyst and on the patient's age. Good results of treatment were obtained.     

OK-432 therapy for unresectable lymphangiomas in children

To avoid surgery-related complications, intralesional OK-432 therapy was used in 23 patients with unresectable lymphangiomas (1986 through 1989). Total or near total shrinkage of the lesions, without serious complication, was noted in 10, marked shrinkage in 8, slight shrinkage in 3, and no response was noted in the remaining 2. Recurrence of the lesions has not been observed during follow-up (4 to 35 months). The side effects of OK-432 therapy were a fever for 2 to 3 days and a local inflammatory reaction lasting 3 to 5 days. The local inflammation did not cause any damage to the overlying skin and did not lead to scar formation. The results suggest that intralesional OK-432 therapy is effective for most unresectable lymphangiomas.     

Vacuum-assisted closure in the treatment of extensive lymphangiomas in children

BackgroundThe management of lymphangiomas in children is a complex problem with frequent recurrence and infection. Vacuum-assisted closure (VAC) devices have been shown to accelerate the healing of open wounds. We hypothesized that VAC therapy might decrease complications after resection of lymphangiomas.MethodsA retrospective review was performed on 13 children (August 2005 to April 2010) who were patients undergoing lymphangioma resection with postoperative VAC therapy. Patient demographics, size and location of the lymphangioma, VAC duration and number of changes, hospital stay, complications, need for further surgery, and length of follow-up were recorded.ResultsThirteen children (mean age, 8 years; mean weight, 34 kg) underwent 15 operations for lymphangiomas followed by postoperative VAC therapy. Locations included the head and neck, thorax and abdomen, and lower extremity. The mean VAC duration was 19 days, and they underwent a mean of 2.6 VAC changes. Six children had operative closure of the wound at a mean of 15 days postoperative. The remaining patients underwent closure by secondary intention. There were no recurrences. Complications included VAC device malfunctions requiring intervention and wound infections. Mean follow-up was 289 days.ConclusionPostoperative VAC therapy for the treatment of lymphangiomas can be an effective adjunct to surgical treatment by decreasing risks of recurrence and infection.     

Abdominal lymphangiomas in children: interest of the laparoscopic approach

Background Lymphangiomas are rare benign lesions of the lymphatic system. The most common symptoms are abdominal tumor or “acute abdomen” in children. The treatment of choice is complete surgical resection, but the recurrence rate with incomplete resection is high, and laparotomy exposes the patient to adhesions. The authors report their experience with the lymphangioma laparoscopic approach. Methods This retrospective study examined 15 consecutive operations for lymphangiomas in children, ages 5 months to 14 years, treated during the 5-year period from 1999 to 2004. Results Six patients were treated using the primary laparotomy approach, and nine patients underwent the laparoscopic procedure, six successfully. Three conversions were necessary (1 case requiring partial colectomy, 1 retroperitoneal case with adherence on the aorta and vena cava, 1 case with partial volvulus). Morbidity included two cases of acute occlusion caused by adhesions after laparotomy. There was no recurrence of lymphangioma during a mean follow-up period of 35 months. Conclusion The laparoscopy procedure could be used successfully for abdominal lymphangioma, even in an emergency. When the laparoscopic resection is impossible, laparotomy or sclerotherapy can be discussed.     

Complications of implant surgery in children.

Rapid recognition and appropriate therapy will prevent or remediate most of the complications occurring in children after cataract aspiration and IOL implantation. Some of the complications of pediatric IOL implantations are related to the increased scleral pliability and decreased rigidity which predispose scleral collapse, vitreous loss, flat anterior chamber and corneal endothelial damage. A second group of complications is associated with the enhanced inflammatory and fibrotic responses peculiar to a child's eye. This group includes operative striate keratitis and iridocyclitis, late IOL precipitates, secondary and postpseudophakos membranes, iris erosion and synechiae formation, and IOL tilt and displacement. Although the intraocular lens is a possible means of visual rehabilitation for children with traumatic cataracts or unilateral infantile cataracts, its long-term risk/benefit ratio must still stand the test of time.     

Complications of acute sinusitis in children.

OBJECTIVE: To review the demographic, microbiologic, and outcome data for children with complications of acute sinusitis. STUDY DESIGN AND SETTING: Retrospective review of children admitted with complications of acute sinusitis from January 1995 to July 2002 to a tertiary care children's hospital. RESULTS: One hundred four patients were reviewed with the following complications: orbital cellulitis (51), orbital abscesses (44), epidural empyemas (7), subdural empyemas (6), intracerebral abscesses (2), meningitis (2), cavernous sinus thrombosis (1), and Pott's puffy tumors (3). Sixty-six percent were males (P < 0.001), and 64.4% presented from November to March (P < 0.001). Patients with isolated orbital complications were younger than patients with intracranial complications (mean, 6.5 versus 12.3 years), had a shorter stay (mean, 4.2 versus 16.6 days), and had shorter duration of symptoms (mean, 5.4 versus 14.3 days; all P < 0.0001). Complete resolution was documented for 54/55 patients with restricted ocular motility, 7/8 with visual loss, 3/3 patients with a nonreactive pupil, 7/7 with neurological deficits, and 2/4 with seizures. The most common organism isolated was Streptococcus milleri (11/36 patients with surgical cultures). No mortalities occurred, and persistent morbidity occurred in 4 patients (3.8%). CONCLUSIONS: Despite significant deficits on presentation, permanent morbidity was low. Streptococcus milleri is a common pathogen with complications of sinusitis in children.     

Changes in the neural elements of lymphangiomas in children of various ages

The results of neurohistologic investigation of lymphangiomas, removed in 68 children, are presented. At the early age in children, the changes in neural fibers of lymphangiomas correspond to reaction of irritation. With increasing of the age, the pronouncement of the destructive processes in the neural fibers increases, leading to hypo- and anervia of the tissues of lymphangiomas.     

Treatment of lymphangiomas in children: an update of Picibanil (OK-432) sclerotherapy.

Picibanil (OK-432) is a sclerosing agent derived from a low-virulence strain of Streptococcus pyogenes that induces regression of macrocystic lymphangiomas. This report describes a prospective, nonrandomized trial to evaluate the efficacy of Picibanil in the treatment of 13 affected children ranging in age from 1 to 94 months. On average, 4.1 fluoroscopically guided intracystic injections were performed per child, with an average total dose of 0.56 mg of Picibanil. As judged by physical examination and radiographic studies, 5 children (42%) showed a complete or substantial response, and 2 children (16%) showed an intermediate response. No response was seen in 5 children (42%), 2 of whom had massive craniofacial lymphangioma. Factors that contribute to failure with Picibanil sclerotherapy are the presence of a significant microcystic component to the lesion, massive craniofacial involvement, and previous surgical resection. Macrocystic lymphangiomas of the infratemporal fossa or cervical area have the best response to therapy.     

Cystic lymphangiomas of the cauda equina.

BACKGROUND CONTEXT: Lymphangiomas, benign hamartomatous lesions of the lymph tissue, result from a failure of lymph channels to communicate with the venous system or normal lymph channels. Previously, two reports described lymphangiomas arising in the peripheral nerve (ulnar nerve and posterior tibial nerve). PURPOSE: To report a rare case of cystic lymphangiomas of the cauda equina not previously reported. STUDY DESIGN: Case report. PATIENT SAMPLE: A 56-year-old Japanese man. METHODS: Not applicable. RESULT: The patient recovered from most of his symptoms after tumor extirpation. He had no symptoms at a 5.8-year follow-up. CONCLUSIONS: We believe this is the first case report of lymphangiomas originating from the cauda equina, which was effectively treated by surgery.     

Abdominal lymphangiomas in adults

Abdominal lymphangiomas are rare benign cystic tumors that can become locally invasive and often require resection. They arise in all ages and have a variable presentation. We performed a retrospective review of a single institution surgical experience with this lesion in adults. The pathology prospective database was reviewed to identify patients with surgically resected abdominal lymphangiomas from January 1986 to May 2004. Retrospective review and follow-up was performed for each patient. The six patients with abdominal lymphangiomas ranged in age from 38 to 66 years. They presented with a variety of signs and symptoms. All underwent CT scan that demonstrated a cystic lesion, but in only one third was the diagnosis made preoperatively. Tumors were located in the retroperitoneum, small bowel mesentery, liver, and pancreas. Five of the six tumors were completely resected. Two of the six required resection of adjacent or involved organs. Follow-up ranged between 6 months and 18 years. All had symptomatic relief after resection, and no patient showed evidence of recurrence in this time period. Abdominal lymphangiomas are rare. The correct diagnosis often remains elusive until tissue is obtained. The treatment of choice is complete surgical resection. When completely resected, these lesions seem not to recur, and the overall prognosis is excellent.     

Complications of percutaneous insertion of Hickman catheters in children.

BACKGROUND/PURPOSE: The aim of this study was a retrospective evaluation of insertion and management complications of percutaneous Hickman catheter lines in pediatric patients to investigate whether the complication rate is acceptable in comparison with other insertion methods or other age groups. METHODS: Over a period of 22 months a total of 27 Hickman catheters were inserted in 22 pediatric patients (20 oncological, 2 nononcological; age 6 weeks to 17.5 years). RESULTS: Twenty-three of 36 insertion attempts (63.9%) were successful at first attempt. In another 4 patients, catheters were placed after repeated attempts. In an additional 4 patients, catheters were inserted by surgeons after percutaneous insertion failed. As immediate complications, 1 pneumothorax and 1 malposition were seen. Late complications included 1 to 29 (median, 8) days of fever in 15 patients, corresponding to 53 of 1,000 catheter days. Fourteen patients showed 21 positive blood cultures, including 11 cases of Staphylococcus epidermides, which might be related to the catheter. Antibiotics were given for a total of 1 to 130 (median, 35) days, that is 205 of 1,000 catheter days. No catheter was removed because of infectious complications. The total life span of the Hickman catheters was 1 to 371 (median, 163) days, the patients were in the hospital from 1 to 351 (median, 102) days because of their underlying disease. At the end of the study period, 8 of 27 (29.6%) catheters remained functioning in situ; 9 (33.3%) had been selectively removed. Two patients died with the catheter (7.4%) functioning well. Another 2 patients showed catheter thrombosis. Six catheters (22.2%) in 5 patients showed inadvertent dislodgement. CONCLUSION: Percutaneous Hickman catheter insertion in pediatric patients is effective; however, complication rate is relevant, but not higher than percutaneous insertion of subclavian vein or Hickman catheters in adults.     

Osteotomy for femoral anteversion. Complications in 95 children

Totally, 95 children with increased femoral anteversion had derotational substrochanteric osteotomy with plate fixation. The mean anteversion angle was reduced from 48 degrees to 4 degrees, internal hip rotation from 81 degrees to 42 degrees, and external rotation increased from 9 degrees to 48 degrees. All the osteotomies healed, but serious complications occurred in 13 of the 95 patients. Because of the risk for complications and the trend towards spontaneous regression of femoral anteversion with age, we recommend a much more conservative attitude with regard to operative treatment.     

Abdominal cystic lymphangiomas in children. Clinical, diagnostic and therapeutic aspects: apropos of 21 cases]

Twenty-one abdominal cystic lymphangiomas were observed in paediatric patients during a 15-year period, in 11 boys and 10 girls. Diagnosis was prenatal in 2 cases; the mean age of the other 19 children was 4.7 years (range: 3 months-8 years). Tumours were intraperitoneal in 16 cases and retroperitoneal in 5 cases. Symptoms were variable: abdominal pain in 15 cases, palpable tumour in 6 cases (excluding the two cases of prenatal diagnosis). Complications included obstruction in 7 cases (including 3 by volvulus), infection in 6 cases, and intracystic haemorrhage in 3 cases. Abdominal ultrasonography correctly established the diagnosis in all children. Surgical treatment included 20 complete resections and one incomplete resection, including 6 with bowel resections. With a follow-up ranging from 6 months to 10 years, one recurrence occurred and was successfully reoperated. Intraabdominal cystic lymphangioma in childhood is a rare tumour with a variable presentation. An accurate diagnosis can be established by abdominal ultrasound. Complete resection should be performed whenever possible.