Roberta E. Sonnino

Pediatric abdominal lymphangiomas: A plea for early recognition

Abdominal lymphangiomas are usually classified together with mesenteric cysts. However, they differ by location, histology, and potential for recurrence, and should be considered a separate clinical entity. Thirteen children, aged 2 weeks to 11 years (mean, 5.8 years), with abdominal lymphangiomas were identified over the past 16 years at this institution. Of these, 12 were symptomatic. Abdominal pain (11), vomiting (8), increased abdominal girth (8), and nausea (6) predominated. Other presentations were less frequent. Symptoms were present for an average of 2 months (7 less than 1 week) before correct diagnosis. An abdominal mass was palpable in 10 cases. Intestinal gangrene secondary to volvulus was present in 2. Although multiple imaging modalities were used ultrasonography (8/8) and computed tomography (CT; 4/4) proved most expedient and reliable. In 2 cases, the lymphangioma could not be completely resected. There was 1 recurrence. Although intraabdominal cystic lesions are described in the literature as relatively symptom-free, our experience suggests otherwise. In this series, abdominal pain and an abdominal mass were common. Catastrophic complications can occur and excision is facilitated by earlier diagnosis and the benefit of smaller size. Ultrasound and CT can accurately diagnose the lesion and should be used liberally in children with intermittent or ill-defined abdominal pain, leading to prompt recognition and definitive treatment.

Splenic cysts: aspiration, sclerosis, or resection.

Percutaneous aspiration and tetracycline sclerosis is a safe but temporary therapy of large splenic cysts in children. Between 1985 and 1987, three girls with splenic cysts were seen. Their ages ranged from 5 to 14 years, and the cysts were from 8 to 16 cm in diameter. Despite their large size, all were asymptomatic and were discovered upon physical examination or ultrasound for unrelated conditions. All cysts were avascular by scan and had irregular crenated or smooth walls by ultrasound. Further investigation excluded infectious or parasitic causes. Each cyst was aspirated for diagnosis, and a pigtail catheter was inserted for drainage and sclerotherapy. All needle aspirations resulted in cyst collapse, but in one patient the pigtail catheter insertion was unsuccessful, and in the other two cases, multiple attempts of tetracycline sclerosis failed to obliterate the cysts. There were no other complications. Surgery for the recurrent splenic cysts was performed 3 months to 2 years following the percutaneous procedures. The two patients operated on with 3 months of aspiration underwent successful partial splenectomy and have normal splenic function by ultrasound scan, and absence of RBCs. The third patient had progression of the cystic disease throughout the spleen, and required splenectomy. Pathology confirmed multiseptate congenital mesothelial cysts in the first two patients and massive lymphangiomatosis in the third. In all three cases, percutaneous therapy was safe but did not result in long-term control. In one patient, the cystic disease progressed following sclerotherapy and may have influenced the need for complete splenectomy. Prior manipulation did not adversely affect the dissection and mobilization of the spleens.(ABSTRACT TRUNCATED AT 250 WORDS)

Appendicitis in children: Current therapeutic recommendations

Wound infection is the most common source of morbidity in appendicitis. Most recent pediatric series use protocols of preoperative antibiotics with aerobic and anaerobic coverage, intraoperative lavage, no peritoneal or wound drains, and continuation of antibiotics postoperatively with complicated appendicitis. There still remains controversy concerning skin closure and the duration of antibiotic therapy. We report the results of a prospective protocol followed over 2 years with 420 children. The protocol was designed to determine whether the skin could be closed primarily in all patients undergoing appendectomy. Preoperatively all patients received triple antibiotics (ampicillin, gentamicin, and clindamycin) that were continued postoperatively for two doses if there was a normal appendix or simple acute appendicitis, for at least 3 days with gangrenous appendicitis, and at least 5 days with perforated appendicitis. Antibiotics were continued if the patient remained febrile or had a white count greater than 10,000. No drains were used and the skin was closed primarily. The overall infectious complication rate was 1.0% (4/420). Among those with a normal appendix or simple acute appendicitis there were no infectious complications. Among those with gangrenous or perforated appendicitis there were 1.7% wound infections (2/117) and 1.7% intraabdominal abscesses (2/117). Duration of hospitalization was 2.1 days (range, 1 to 5 days) after simple acute appendicitis and 6.9 days (range, 3 to 40 days) after gangrenous or perforated appendicitis. These results set new standards in terms of wound management, infectious complications, and length of hospital stay.

Plasma intestinal fatty acid binding protein in neonates with necrotizing entercolitis: A pilot study

BACKGROUND/PURPOSE Intestinal fatty acid-binding protein (IFABP) is found within cells at the tip of the intestinal villi, an area commonly injured when necrotizing enterocolitis (NEC) occurs. This study was undertaken to determine if measuring IFABP concentrations in the bloodstream early in the course of NEC would differentiate patients by severity before clinical findings made it clear who had stage 3 NEC and who had milder stages. METHODS Three plasma samples from newborn infants evaluated for NEC were obtained at symptom onset and after 8 and 24 hours. IFABP concentration was measured by radioimmunoassay. Infants were classified by the final and most severe stage of NEC, and IFABP levels were compared between groups at each sampling. RESULTS IFABP was detectable in blood samples from all 7 infants with stage 3 NEC compared with 3 of 24 with stages 1 or 2 NEC. Elevated plasma IFABP concentrations were detectable before clinical staging could be made in 5 of the 7 subjects with stage 3 NEC. CONCLUSION IFABP may be a specific marker for early identification of severe NEC.

Unusual patterns of congenital neck masses in children

Congenital neck masses--thyroglossal duct cysts (TDC) and branchial cleft cysts (BCC)--are frequently encountered in any pediatric surgical practice. While their diagnosis is usually straightforward, unusual or combined presentations may occur. We report eleven cases of unusual patterns of congenital neck masses in children. Two patients underwent resection of a BCC with a sinus tract extending through the carotid bifurcation. Both patients subsequently presented with a new mass near the previous scar, which was thought to be a recurrence but was found at surgery to have TDCs with sinus tracts extending through the hyoid bone. In one patient, a lateral neck mass (presumed to be a BCC) was found at surgery to be a TDC. In all three cases the diagnoses were confirmed histologically. Eight patients presented with a solitary thyroid nodule. Six of these had intrathyroid branchial cleft remnants, and two had intrathyroid TDCs. The diagnosis became apparent at operation in six patients, while in two it was made by the pathologist after hemithyroidectomy. Ages at presentation ranged from 16 months to 14 years. The embryology of these neck structures is closely related. It should not come as a surprise that errors in their development may occur, at times paralleling the occurrence of intrathyroid location of parathyroid glands. The possibility of an embryologic rest in the neck should therefore be kept in mind with all clinically evident neck masses. TDCs and BCCs may coexist in the same patient. The histologic differentiation may be difficult in the presence of inflammation, but differences in structure are often characteristic.(ABSTRACT TRUNCATED AT 250 WORDS)

Gracilis muscle transposition for anal incontinence in children : long-term follow-up

We report a series of 7 patients, aged 6.5 to 19 years (mean, 12.9), who have been treated for uncontrollable fecal incontinence since 1976: 5 had imperforate anus and multiple subsequent operative procedures, 1 had a low myelomeningocele with bi-sphincteric incontinence, and 1 had a traumatic destruction of the sphincter apparatus. A modified Pickrell procedure was performed, with the gracilis muscle transposed subcutaneously, without constructing a pulley through the median raphe as originally described. All patients were evaluated by anorectal manometry preoperatively and post-operatively. They were followed-up for a period of 0.5 to 12.5 years (mean, 4.4). All patients were continent at follow-up, with a normal defecation pattern and no enemas required. None of the patients had evidence of fibrosis of the muscle or anal canal, and tension in the transposed muscle was maintained. Voluntary contractions remain efficient in all cases. Age was thought to be an important factor: personal motivation and compliance with physiotherapy, essential for a good outcome, is unlikely to be present in the younger child. We conclude that the gracilis sling procedure is an excellent long-term alternative for total fecal incontinence when time and other therapeutic measures have failed.

Technical Aspects of Intestinal Transplantation in the Rat

The microsurgical technique for rat intestinal transplantation used in our laboratory is described. A 25-cm segment of jejunum is harvested from the donor on a pedicle of superior mesenteric artery (SMA) and superior mesenteric vein (SMV). These vessels are then anastomosed end-to-side with 10-0 nylon to the infrarenal aorta and inferior vena cava of the recipient. The bowel ends are placed in isoperistaltic continuity with the host intestine. The procedure was performed in 423 animals with an 86 percent success rate and has been used to investigate all aspects of small bowel transplantation. The technique is described in detail with schematic drawings and intraoperative photographs, with the goal of making the procedure more understandable and accessible to other investigators wishing to study vascularized intestinal transplantation in the rat.

Rat jejunal absorptive function after intestinal transplantation : effects of extrinsic denervation

Jejunal absorptive function was evaluated following small intestinal transplantation to determine the effects of extrinsic denervation. In particular, water and sodium absorption were measured following transplantation (denervated) and compared to a control group (Thiry-Vella). Water flow was initially secretory two days after transplantation (−41±27 μl/min/g dry tissue weight) but became absorptive at day 8 (25±25). Water flow in the Thiry-Vella group was not significantly different (P>0.05) from the transplant group at days 8 (17±7) and 10 (47±42). Sodium flows were also initially secretory in the transplant group and became absorptive. This study refutes previous claims that small bowel transplants have a deficiency of water absorption due to extrinsic denervation of the bowel and suggests normal absorptive function of water, electrolytes, and protein.

Monitoring of intestinal transplant rejection

Our results show that maltase, sucrase, and lactase activity are present at a normal level in nonrejecting small bowel transplants after an initial postoperative decline. This confirms that the disaccharide absorbing capacity of these grafts is intact. In allogeneic bowel, however, the levels of maltase and sucrase decline as histologic rejection occurs. These results suggest that serial maltase, sucrase, and possibly lactase levels in allogeneic intestinal transplants may serve as a useful adjunct in the monitoring of small bowel transplant rejection.

Hereditary sacrococcygeal teratomas

Hereditary presacral teratoma and its associated anomalies have been described in six kindreds. We report on a similar family where the father presents with a presumed meningocele and his two children with sacrococcygeal teratomas, associated with anterior meningocele in one.