Cystic lymphangioma of Retzius space manifested as acute abdomen.

The authors describe a rare pediatric case of cystic lymphangioma arising from the Retzius space. A 9-year-old boy underwent an appendectomy in a nearby hospital after a sudden onset of severe hypogastralgia. When laparotomy revealed a retroperitoneal mass, he was referred to our hospital. After diagnosis of a multicystic mass in the Retzius space, extirpation of the cystic lesion was performed. Histological evaluation of the resected specimens revealed cystic lymphangioma. The patient has been free of symptoms for 6 years since the operation.

A case of ischemic jejunal stricture after surgical reduction of intussusception.

A 15-year-old girl presented with small bowel obstruction due to ischemic jejunal stricture which developed three weeks after successful surgical reduction of an intussusception with a Peutz-Jeghers-type polyp as a lead point. The reduced jejunum had no macroscopic injury, and the stricture caused complete obstruction requiring jejunal resection.

Benign transient non-organic ileus of neonates.

There are a substantial number of neonates who present with Hirschsprung's disease-like symptoms, but respond very well to conservative therapy. However, once Hirschsprung's disease is ruled out, little attention is paid to these infants, because of the lack of necessity for surgical treatment and their excellent prognosis. The purpose of this study was to elucidate the clinical features of functional ileus of neonates, which we named benign transient non-organic ileus of neonates (BTNIN). Out of 61 neonates referred to our institution with suspected neonatal Hirschsprung's disease (NH), 10 were diagnosed as having NH and 51 as having BTNIN. All the cases of BTNIN showed marked abdominal distension, and 12 showed explosive defecation on digital examination at the first visit. Plain X-ray demonstrated marked whole intestinal dilatation in 12 cases including cases with niveau formation and segmental dilatation. These findings were indistinguishable from those of NH. However, all had a normal anorectal reflex, and rectal suction biopsy revealed normal acetylcholinesterase activity and submucosal ganglion cells. All the cases of BTNIN were treated with periodic glycerin enemas until daily spontaneous defecation was established, which took 2 to 14 months, with an average of 5.0 +/- 2.9 months. None of them showed residual symptoms during the follow-up period.

The preparation of the chronic hyper-endotoxemia experimental animal model by means of a drug delivery system.

A drug delivery system (DDS) consisting of lipopolysaccharide (LPS) as a drug and 2-hydroxyethyl methacrylate (HEMA)-diethylene glycol dimethacrylate (2G) or -polyethylene glycol dimethacrylate (4G, 9G) copolymer was prepared, and used for the efficient preparation of an experimental animal model of chronic hyper-endotoxemia. The release profiles of LPS in the in-vitro test were greatly influenced by the composition of HEMA-2G, 4G, 9G in the copolymer. It was found that LPS release from the DDS continued gradually and constantly throughout 2 weeks. In the in-vivo experiment with rats, the DDS maintained a high blood concentration level of LPS for 3 days. These results strongly suggest the possibility of convenient and reproducible preparation of a chronic hyper-endotoxemia animal model.

Application of a drug delivery system in a novel rat model of chronic hyperendotoxemia.

There has not been an ideal reproducible small-animal model of chronic hyperendotoxemia to date. Our drug delivery system (DDS) is a new technology that can deliver a drug conveniently to a target organ at an optional rate. 2-Hydroxyethyl methacrylate (HEMA) was used as a carrier of lipopolysaccharide (LPS), and diethylene glycol and polyethylene glycol dimethacrylates (2G, 4G, 9G) were used as cross-linking agents. A mixed solution of HEMA and di(poly)ethylene glycol dimethacrylate was charged into a glass tube with or without LPS and polymerized by ultraviolet irradiation. This polymer was cut into DDS tablets of the same size with or without LPS. A mixture with HEMA:4G = 1:3 was the most suitable composition to release a constant concentration of LPS. We also developed a novel rat model of chronic hyperendotoxemia. Four DDS tablets, each containing 15 mg LPS, were implanted into the abdominal cavity of rats in the LPS group. The control group was implanted with four DDS tablets without LPS. Plasma levels of LPS in the study group were maintained at more than 2,000 pg/ml for 72 h after implantation. Weight gain was lower and body temperature was higher in the LPS group than in the control group. Plasma levels of inter leukin (IL)-6 in the LPS group were higher than in the control group only during the initial 12 h after implantation of DDS tablets. The white blood cell count at 24 h and platelet counts at 24, 48, and 72 h in the LPS group were lower than those in the control group. These results indicate that chronic hyperendotoxemia was maintained for 72 h by continuous release of LPS from the DDS. Moreover, the intensity of endotoxemia could be varied by varying the number of DDS tablets. It is concluded that our new rat model using LPS-DDS will be applicable and useful as a model of chronic hyperendotoxemia.

Clinicopathologic relationship of hypoganglionosis.

BACKGROUND/PURPOSE: Congenital motor dysfunction of the intestine associated with a morphologically abnormal myenteric nervous plexus (MP) is known as Hirschsprung's disease allied disorder (HAD). However, the clinicopathologic features of HAD are not well understood, partially because a standardized method of histologic evaluation of MP has not been established. To elucidate the clinicopathologic relationship of HAD the authors reviewed 6 cases of HAD using a newly devised histologic evaluation method. METHODS: Flat-mounted frozen sections of the ileum were stained for S-100 protein by fluorescent immunohistochemistry. Quantitative evaluation of MP was performed by measuring the fluorescence-positive area (MP ratio), and the results were compared with those of age-matched normal controls. RESULTS: All of 6 patients required laparotomy within 1 month after birth and enterostomy between 23 days and 10 months. Three died of intractable enteritis by the age of 2.2 years and were totally dependent on parenteral nutrition (PN) throughout their lives. The other 3 have survived for 6 to 10 years but have required PN occasionally. MP ratio in controls was more than 0.34 at all ages, whereas that in HAD was significantly lower than that in controls according to the clinical severity. CONCLUSION: MP size measured on 2-dimensional demonstration is suggested to be an indicator of clinical severity of HAD. J Pediatr Surg 36:898-900.

Laparoscopic ovarian cystectomy using a single umbilical puncture method.

To establish a minimally invasive technique to perform ovarian cystectomy, the authors applied a single umbilical puncture method. A 2-month old female infant was admitted to the hospital because of an ovarian cyst that showed no spontaneous shrinkage after her birth. An umbilical semicircular incision was made to insert a 10-mm trocar, into which a 3-mm laparoscope was inserted. The ovarian cyst was grasped using forceps inserted through an operating channel of the scope, and the cyst was removed through the incision. After aspiration of the cyst, the free cyst wall was resected, leaving the intact ovarian tissue. The operation was performed without difficulty or complication. The postoperative course was uneventful. The wound was inconspicuous, and the result was cosmetically excellent. The case demonstrated the feasibility of the minimally invasive technique using a single umbilical puncture for ovarian cystectomy in an infant.

Surgical intervention for emphysematous pulmonary regions in a postoperative infant with congenital diaphragmatic hernia.

A postoperative infant with congenital diaphragmatic hernia (CDH) developed extrinsic obstruction of the trachea by the innominate artery that ensued from unequal expansion of the lungs followed by left mediastinal shift. Septation of the anterior mediastinum prevented unequal expansion of the lungs, and elongation of the innominate artery improved proximal airway obstruction. Prolonged artificial ventilation, however, resulted in the emphysematous bullae in the left lung. Lung volume reduction surgery (LVRS), at 3 years of age, ameliorated the respiratory distress and resulted in good weight gain. Surgical intervention, including LVRS, should be considered to improve respiratory disturbance caused by difference in compliance of the lungs in children.

Two cases of persistent hyperinsulinemic hypoglycemia that showed spontaneous regression and maturation of the Langerhans islets.

Near-total pancreatectomy has been recommended as the treatment for persistent hyperinsulinemic hypoglycemia (PHH) in infants. However, recently there has been a report described that one third of 95% pancreatectomy failed to prevent hypoglycemia and more than two thirds had diabetes ultimately. The authors experienced 2 cases of PHH, which raise a query about the extensive pancreatectomy. Case 1: A female patient who manifested PHH shortly after birth underwent less extensive pancreatectomy twice at age 2 months and 8 years. After each operation, her clinical symptoms regressed, and she became free from the disease eventually. Histologic findings showed nesidioblastosis in which the islets clearly matured. Case 2: A male infant with PHH had an absolute indication for pancreatectomy. However, after a meticulous control of the blood glucose level with parenteral nutrition followed by continuous enteral nutrition feeding combined with medication, he became free from the disease. The current cases show there exist cases of PHH in which the islets mature and symptoms regress spontaneously. Therefore, we conclude near-total pancreatectomy is not always the treatment of choice. As an alternate strategy, long-term controlled feeding and medication combined with or without less extensive pancreatectomy should be considered with the expectation of spontaneous regression.

Congenital ileal atresia presenting as a single cyst-like lesion on prenatal sonography.

A routine prenatal sonographic examination at 37 weeks' menstrual age revealed a large sonolucent lesion with peristaltic movement in the abdomen of a fetus. After birth, the female infant showed progressive abdominal distention, and radiography showed a bubble-like dilatation of the small intestine. Exploratory laparotomy revealed ileal atresia with nearby partial torsion of the dilated small bowel. The incomplete torsion may have functioned as a check valve, inducing segmental dilatation of the ileum without proximal dilatation.

Handlebar hernia: case report and review of pediatric cases.

The authors describe a rare case of handlebar hernia in a 9-year-old-boy. All layers of his abdominal wall were disrupted by a fall on a bicycle; however, his skin and intra-abdominal organs were completely intact. Computed tomography demonstrated subcutaneous intestinal loops protruding through the rent. Surgical repair was performed, and his postoperative course was uneventful.

Subcapsular hemorrhage of the liver in a very-low-birth-weight neonate: survival after decompression laparotomy.

Subcapsular hemorrhage of the liver in a very-low-birth-weight neonate was successfully treated by decompression laparotomy. This may be the second smallest survivor after surgery in the literature.

Three patients with bilateral breast tumors examined by technetium-99m-sestamibi scintimammography.

We presented three patients with bilateral breast tumors (one with breast cancers, one with breast cancer and fibroadenoma, and one with fibroadenomas) examined by 99mTc-hexakis 2-methoxy-isobutylisonitrile (MIBI) scintimammography. In all cases 99mTc-MIBI uptake was recognized only in the breast cancers. 99mTc-MIBI scintimammography was useful in evaluating bilateral breast tumors since preoperative diagnosis is valuable in determining correct surgical treatment.