Koonosuke Nakada

Digestive tract disorders associated with asplenia/polysplenia syndrome.

The authors reviewed experience gained over a 20-year period of asplenia or polysplenia syndrome, focusing on patients with associated digestive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia/polysplenia had associated DTDs. The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus in three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bowel obstruction and on one patient with biliary atresia. One patient with both malrotation and gastric volvulus, and another with only associated malrotation survived. Nine patients died, eight of cardiac insufficiency and one because of hepatic insufficiency. When infants are diagnosed with heterotaxia, they should be examined for other combined DTDs, because they may have a chance for survival if they undergo surgery when their condition is still stable.

Two cases of torsion of the gallbladder diagnosed preoperatively

BACKGROUND The authors experienced two cases of torsion of the gallbladder, both of which were correctly diagnosed preoperatively. METHODS Two boys, aged 4 and 5 years, were transferred to Yokohama City Seibu Hospital because of their acute abdominal disorders. Diagnostic imaging demonstrated acute inflammatory changes in the gallbladder with an abnormal arrangement of the gallbladder and common bile duct in both cases. Laparotomy findings showed torsion of the gallbladder by 180 degrees and 360 degrees, respectively, at the cystic ducts, resulting in gangrenous change. RESULTS Both children recovered uneventfully after cholecystectomy. The diagnostic imaging criteria are (1) collection of fluid between the gallbladder and the gallbladder fossa of the liver, (2) a horizontal rather than vertical arrangement of the long axis of the gallbladder, (3) the presence of a well-enhanced cystic duct located on the right side of the gallbladder, and (4) signs of inflammation including marked edema with thickening of the wall. CONCLUSIONS The authors report the clinical characteristics of this uncommon condition, and discuss the significance of accurate preoperative diagnosis of these acute surgical disorders.

Demonstration of apoptosis in neuroblastoma and its relationship to tumour regression.

The in vivo occurrence of apoptosis in neuroblastomas was investigated. Histologically, a number of tumour cells showed typical apoptotic changes, including cell shrinkage, condensed and fragmented nuclei, eosinophilic cytoplasm, and absence of the inflammatory response. These cells coincided closely with the so-called karyorrhectic cells. An electrophoretic DNA ladder, a functional hallmark of apoptosis, was demonstrated in four of six tumours, and DNA fragmentation was detected in situ by terminal deoxytransferase-mediated nick end-labelling in 26 of 35 tumour specimens (74%). The labelled cell counts ranged from 5 to 62 per 5000 tumour cells (mean±SD: 15.0±14.5). Immunoperoxidase staining revealed that an apoptosis-suppressing protein, bcl-2, was expressed abundantly in advanced-stage tumours, whereas it was absent from karyorrhectic-apoptotic cells. Several tumours with the potential for spontaneous regression were bcl-2-deficient. Immunostaining of the Fas receptor for apoptosis demonstrated that the tumour cells expressed this molecule on their cell surfaces. Our results provide evidence of apoptosis in neuroblastomas and suggest that bcl-2 and the Fas receptor may play a role in its regulatory mechanisms.

Apoptosis in favourable neuroblastomas is not dependent on Fas (CD95/APO-1) expression but on activated caspase 3 (CPP32).

The mechanisms of apoptosis in neuroblastomas have been investigated by examining the expression profiles of Fas, Fas ligand (FasL), and caspase 3 in 42 primary tumour tissues. Immunohistochemically, no or weak Fas expression was detected in 25 out of 29 neuroblastomas (86 per cent), whereas high levels of expression of FasL and pro‐caspase 3 were noted in 30 and 29 of 42 tumours, respectively (∼70 per cent). Overexpression of pro‐caspase 3, but not FasL, correlated significantly with a younger age and low tumour stage. Western blot analysis of ten neuroblastomas confirmed the lack of Fas expression and the presence of strong FasL expression in all samples and pro‐caspase 3 expression in five tumours, of which four belonged to the favourable type. These favourable tumours also showed vigorous Asp‐Glu‐Val‐Asp (DEVD) hydrolytic, or caspase 3‐like activities, while the unfavourable tumour lacked such activity. Moreover, immunostaining for the p17 subunit of the caspase 3 heterodimer showed that active caspase 3 was mainly localized in apoptotic tumour cells. Combined together, our results suggest that caspase 3, activated via a Fas‐independent pathway, may play important roles in apoptosis, suppression of growth, and, in some cases, regression of favourable neuroblastomas. Copyright

Unilateral pulmonary agenesis with tracheoesophageal fistula: A case report

The authors describe a neonate with unilateral pulmonary agenesis and esophageal atresia (Gross type C). Definitive repair for the atresia was completed during the early neonatal period, and the patient is alive and well more than 1 year after surgery. Most patients with pulmonary agenesis associated with esophageal atresia have not survived, mainly because of postoperative cardiorespiratory failure. Early protection and preservation of respiratory units is essential for the management of these patients.

Hemangioma of the upper arm associated with massive hemorrhage in a neonate

A rare case of an ulcerated hemangioma of the upper arm in a neonate is described herein. Resection of the huge hemangioma, which occupied the axilla and extended down the entire length of the upper arm, was necessary due to massive hemorrhage from the ulcer and progressive heart failure caused by the arteriovenous fistulae in the hemangioma. After complete resection of the hemangioma, extensive defects of the skin and subcutaneous tissue were replaced by a skin flap closure technique which involved considerable difficulty. However, the arm gradually recovered its size and function with good wound healing. The present case illustrates that rapid surgical intervention may be required to prevent clinical deterioration in such cases.

Doppler ultrasonographic evaluation of hepatic circulation in patients following kasai's operation for biliary atresia

The hepatic circulation of eight children who underwent Kasais operation for biliary atresia was serially evaluated by Doppler ultrasonography (US). A total of 36 examinations were performed to evaluate the maximal velocities (mvs) of the main portal vein (MPV), splenic vein (SV), and hepatic artery (HA), and to analyze the spectral waveform and the directions of flow. The mean mvs-MPV in four patients for whom adequate biliary diversion had been achieved and whose serum bilirubin had fallen to within the normal range (group A), was 19.8±7.5 cm/s. Their MPV waveforms were constant, with blood flowing toward the liver, while their mean mvs-SV was 12.1±5.8 cm/s. In two other patients with apparent hypersplenism, but whose serum bilirubin levels had fallen to nearly normal (group B), the mean mvs-MPV was 20.7±10.4 cm/s and the mvs-SV was 22.4±10.4 cm/s. In contrast, the mvs-MPV in the two remaining patients, whose serum bilirubin levels had either not fallen to within the normal range, or had fallen initially but increased due to recurrent cholangitis (group C), was 14.2±9.1 cm/s. In these patients, the waveforms were unstable, the MPV flows were occasionally hepatofugal, and their mean mvs-SV was 18.0±5.8 cm/s. The mvs-HA were markedly increased in the latter four patients, whose clinical condition had also deteriorated. These observations led to the conclusion that hepatic circulation evaluated by serial Doppler US provides important information about liver status in children who have undergone Kasais operation for biliary atresia.

Efficacy of exogenous fibronectin in wound healing in malnourished rats.

BACKGROUND/PURPOSE Fibronectin (FN) plays an important role in fibrin matrix formation during the wound healing process. The authors investigated whether exogenous FN increases the bursting strength (BS) of surgical wounds in malnourished rats. METHODS Ninety rats were grouped according to three nutritional conditions (n = 30 in each group). All animals underwent a transverse celiotomy after 3 weeks of feeding, and FN (2 mg/body/day) was given postoperatively to 15 rats in each of the three groups. Wound BS was measured on postoperative days (PODs) 3, 5, and 7 after removal of the sutures. RESULTS BS in normally nourished rats (group N; 142.6+/-23.4 mmHg) was significantly higher than that in protein malnourished rats (group PM, 110.2+/-11.2 mm Hg) and protein/ calorie (Cal)-malnourished rats (group PCM, 76.5+/-10.7 mm Hg) on POD 7 (P<.01). However, BS values for groups PM + FN (147.0+/-21.1 mmHg) and PCM + FN (115.1+/-28.9 mm Hg) were intensified significantly in comparison with groups PM and PCM (P<.01). Plasma FN levels in rats of the three FN nontreated groups were similarly decreased on POD 3 or 5, but returned to the preoperative level on POD 7, whereas those for the other three FN-treated groups increased after POD 3. CONCLUSION Intravenous administration of FN might strengthen the weakened wounds of malnourished animals.

Tracheal agenesis with bronchoesophageal fistulas: a case report

Although tracheal agenesis is presently considered to be a rare and fatal anomaly, an increasing number of institutions have been reporting such cases. Reported herein, is a clinical and necropsy report of a premature infant whose tracheal agenesis was associated with bronchoesophageal fistulas and an imperforate anus. Accidental esophageal intubation enabled a perineal anoplasty to be performed under general anesthesia and allowed him to survive for 14 days.

An infant with bilateral adrenal neuroblastoma found by mass-screening: Report of a case

A male infant with bilateral adrenal neuroblastoma found by mass-screening is herein reported. The patient presented with almost equal-sized tumors, each remaining confined to the respective adrenal gland without crossing the midline. No other tumorous lesion was evident. The two adrenal tumors had similar histologic features and appeared to have grown concurrently. Such simultaneous occurrence of primary adrenal neuroblastoma is unusual, and appears to reflect the multicentric origin of this tumor.