Recurrent mesenchymal hamartoma associated with 19q translocation. A call for more radical surgical resection.

Hepatic mesenchymal hamartoma is a rare benign tumour in children. It is often large and centrally located in the liver at diagnosis, making surgical resection difficult; thus non-radical resection has been proposed in the past as acceptable management. However, a literature survey and a case with recurrence associated with cytogenetic anomalies suggest that radical liver surgery (resection with a margin of normal liver parenchyma, as for malignant tumour) should be recommended for mesenchymal hamartoma.

Diagnosis and therapy of ultrashort Hirschsprung's disease.

BACKGROUND: Although ultrashort Hirschsprung's disease (UHD) was enzyme-histochemically characterised about 35 years ago, its existence is still often ignored. The aim of this study is to summarise the clinical diagnostic, incidence, gender ratio, morphological characteristics, and therapy over 15 years. METHODOLOGY: The reliable diagnosis of suspected UHD requires a minimal enema of contrast medium to exclude Hirschsprung's disease (HD). In UHD during pressing or crying no reflux of contrast medium is observed. Final proof of UHD is an enzyme-histochemical biopsy examination of distal rectal mucosa. The biopsies must demonstrate submucosa and be taken from the dentate line and 1 cm, 2 cm, 4 cm and 6 cm above the dentate line. The cryostat sections must be cut 15 microm thick; this thickness is reduced to 4.5 microm by the thawing, spreading and drying of the sections on microscope slides. A reliable diagnosis of UHD needs an enzyme-histochemical acetylcholinesterase reaction of native sections of rectal mucosa. RESULTS: UHD develops with first symptoms of chronic constipation in the second half of the first year of life. The chronic constipation proves to be therapy resistant. In HD constipation occurs in the first weeks of life or after weaning. In contrast to HD, no nerve fibres with increased AChE activity are observed in the lamina propria mucosa. Nets of nerve fibres with increased AChE activity can be found only in the muscularis mucosa and the musculus corrugator cutis ani (MCCA). The therapy of choice has proven to be a partial myectomy of the distal internal sphincter if dilatation of the internal sphincter was ineffective. UHD is either limited to the anal ring, or extends 3 - 4 cm into the distal rectum. Over the past 15 years, UHD had in our series an incidence of 13.4 % of all aganglionoses. The gender ratio of girls to boys was 1 : 2. CONCLUSION: UHD is reliably diagnosed by an AChE reaction in native biopsy sections from the anocutaneous transitional zone and, potentially, from 3 - 4 cm above the pectinate line. As UHD is always accompanied by aganglionosis of the distal internal sphincter, an increase in AChE activity is observed in the nerve fibres of the MCCA. The therapy of choice is a partial myectomy of the distal internal sphincter.

Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries.

AIM: In contrast to other countries, no collective study of Rehbein's procedure in German-speaking nations has been performed. Therefore, our intention was, analogously to Goto and Ikeda's (10) Japanese study in 1984, Kleinhaus's (13) study on Swenson's procedure in 1979, Bourdelat's (2) French-Canadian investigation into Duhamel's technique in 1997 and Martuciello's (11) and Teitelbaum's (16) follow-up in the year 2000, to perform a follow-up study of Rehbein's technique of deep anterior resection. METHODS: The data of 200 patients from 22 German-speaking centers in Switzerland, Austria and Germany were collected. These data were gathered by questionnaire and the children were followed up in the individual participating hospitals for at least 3.5 years after the procedure. The procedure was performed between 1993 and 1997, over a 5-year period. The questionnaire contained 74 items including anamnestic data, diagnostic postoperative treatment and reoperations. RESULTS: Concerning the incidence of anastomotic leaks and resolving anastomotic strictures there was no significant difference between the results in our series and those of the collective analyses made by Hofmann von Kap-herr (7), Holschneider (9) and Sherman (18). In 6.6 % of the 191 patients an anastomotic leak and in 9.9 % a rectal stricture, which had to be dilated, was observed. Concerning late complications, 22.8 % of the children suffered from constipation, 4.3 % from encopresis, 10.6 % from enterocolitis and only 0.5 % from enuresis. The frequency of constipation diminishes over the years. A comparison of the different large series in the literature clearly shows that the incidence of constipation is higher after Rehbein's procedure and the frequency of urinary incontinence and encopresis higher following Swenson's, Soave's and Duhamel's techniques. The incidence of enterocolitis is less after Rehbein's procedure than after Swenson's, Soave's and Duhamel's techniques. CONCLUSIONS: The different results in the literature are due to the individual experience of the author, the very different follow-up methods and the date of follow-up. Therefore, the different results are hard to compare with our study. Nevertheless, Rehbein's anterior resection still could be presented as an adequate and important method to treat Hirschsprung's disease.

Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension.

We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.

Atypical hemihepatectomy during primary CDH-repair in a neonate on ECMO.

We report the case of a newborn with a large left-sided congenital diaphragmatic hernia (CDH) who required extra corporeal membrane oxygenation (ECMO) for severe respiratory insufficiency. CDH repair had to be performed on bypass circulation. Intraoperatively, an atypical hemihepatectomy of the herniated lobe was conducted, because reposition of the liver led to a kinking of the vena cava and to a torsion of the right lobe, resulting in ischemia and compromised venous flow. The extraordinary anatomical indication and the potential danger of uncontrollable bleeding are discussed.

Patient controlled analgesia (PCA) in paediatric surgery: a prospective study following laparoscopic and open appendicectomy.

Patient controlled analgesia (PCA) has not yet gained universal acceptance for the management of postoperative pain in paediatric surgery. In a prospective study we evaluated feasibility and complications of PCA following 90 cases of laparoscopic or open appendicectomy. PCA proved to be a safe and feasible method with few complications (2% of medical complications, no abort of application, 17 technical checks in a total running time of 4125 h). Acceptance by patients was high and children of all age groups worked the system properly. Assessment of application protocols showed, that the consumption of analgesics was significantly reduced following laparoscopic appendicectomy (P < 0.05). PCA is a safe and feasible method for the management of postoperative pain in children and PCA recording provides an excellent insight into the consumptional behaviour of patients, enabling staff to evaluate postoperative pain for various procedures.

The pharmacological effect of sodium phosphate after absorption from the peritoneal cavity.

Hyperosmolar solutions are frequently used in clinical practice for enemas. In a review of the literature we found 43 published cases of severe complications connected with the use of hyperosmolar enemas. The absorption of sodium phosphate led to a raise of the temperature up to 41.1 degrees C, massive acidosis and electrolyte disturbances with hypocalcemia and hypernatremia. The clinical picture was dominated in most of the cases by somnolence or coma. In our experiment the absorption of sodium phosphate from the peritoneal cavity of rats led to similar clinical symptoms and--depending on the amount of sodium phosphate absorbed--to the death of the animals. Hypovolemia, hypernatremia, hypocalcemia and a direct toxic effect of phosphate on the kidneys is responsible for the complex mode of intoxication. Sodium phosphate solutions should not be used in patients with inflammatory bowel disease with a high risk of laceration of the mucosa or perforation of the bowel.

[Diagnosis of fractures of the occipital condyles]. / Diagnostik von Frakturen der Okzipitalkondylen.

The imaging diagnosis of occipital condyle fractures by CT and MRT is reported for cases of 4 patients. Following the conventional X-ray diagnosis of the skull and upper cervical spine, CT proved to be the primary diagnostic method after a skull and brain injury: for all 4 cases we succeeded in detecting the occipital condyle fracture and in determining its size and location by reconstructions (coronal, sagittal, 3-D). In the case of complex accompanying injuries like soft tissue hematomas (cerebral, in the spinal cord, and the soft tissue of the neck) or for the detection of brain stem contusion, MRT had significant advantages. MRT can offer a higher image quality in soft tissue, especially in the spinal cord and the brain.

Endoscopic appendectomy in childhood--technical aspects.

Since March 1992 we initially performed 87 laparoscopic appendectomies applying two cat-gut loops for ligation of the stump. Since we postoperatively found abscess formation twice, we modified the technique in the following aspects: After the introduction of the scope at the umbilicus, 2 working trocars with a diameter of a 5 mm are placed in the left lower quadrant of the abdomen, so that the surgeon can skeletonize the appendix bimanually until it can be dissected with an Endo-GIA. This procedure we carried out in 41 cases and did not see any postoperative complications yet. Thus we consider it an appropriate alternative to conventional surgery.

Current concepts in pediatric burn care: general management of severe burns.

Severe burns and scalds are still frequent. If the burn victim is a child, it should ideally be taken care of in a pediatric burn center, where both the burn and child-related needs are specifically met. The goals of burn care are to preserve life, to preserve function, to limit physical and psychological sequelae and to provide social reintegration. The system of burn care essentially consists of adequate initial resuscitation followed by early surgery aimed at rapid and definitive wound closure. Vigorous nutritional support as well as early rehabilitation and continuous psychosocial care are of paramount importance. The paper summarizes the essentials of pediatric burn care (burn surgery excluded).

Current concepts in pediatric burn care: surgery of severe burns.

Optimal surgical therapy of severe burns in children has been controversial for a long time. A review of the current literature shows that prompt surgical excision of necrotic tissue and immediate autografting have become the standard in most burn centers. The authors present a concept based on prompt excision of third degree burns and discuss the problems of intermediate cover arising in massive burns.

[Organ preservation by reconstruction of Wirsung's duct in complete pancreas rupture]. / Organerhaltung durch Rekonstruktion des Ductus Wirsungianus bei kompletter Pankreasruptur.

Blunt abdominal traumas with pancreatic injuries often cause partial or subtotal resection of the organ, especially when the pancreatic duct is damaged. A reconstructive method for anastomosis of the duct is reported in a case of a 9-year-old boy with complete pancreatic rupture in which the whole organ could be salvaged.

Chlorinated hydrocarbons in fat tissue: analyses of residues in healthy children, tumor patients, and malformed children.

The content of chlorinated hydrocarbons (CHC) was determined by means of capillary column gas chromatography in samples of fat tissue from 183 healthy children, 46 children with malignant tumors and 33 children with benign tumors or congenital malformations. The highest concentrations were found for total polychlorobiphenyls (PCB) (mean = 1.614 ppm), followed by the DDT group (mean = 0.556 ppm, HCB (mean = 0.097 ppm), the HCH isomers (mean = 0.083 ppm), dieldrin (mean = 0.020 ppm) and total heptachlor (mean = 0.010 ppm). Neonates displayed high concentrations in the adipose tissue before the first uptake of food. In the first six months of life, the concentrations of total PCB, the individual PCB components as well as DDT and HCB decreased significantly. In the second year of life, they rose again to the initial values and then remained relatively constant during the rest of childhood. The regional differences with regard to total CHC residues were slight, so that the CHC exposure cannot be reduced by a change of domicile within West Germany (FRG). Children with congenital malformations or benign or malignant tumors do not display raised concentrations of CHC.

[Long segment esophageal atresia and possibilities of esophageal replacement]. / Die Problematik der langstreckigen Osophagusatresie und Möglichkeiten des Osophagusersatzes.

In up to 20% of newborns with esophageal atresia a primary anastomosis of the esophagus is not possible because of the long distance between the two ends. In some cases a circular myotomy can elongate the upper pouch to allow a primary anastomosis. If primary anastomosis is not possible bougienage of the upper and lower pouch may stimulate growth of the esophagus and allow direct anastomosis after several weeks. In all other cases esophageal replacement is necessary. Colonic replacement of the esophagus must be weighed against replacement by jejunum, gastric interposition and the gastric tube. Each technique for esophageal replacement involves characteristic complications, and these are discussed in detail. For about 80% of the patients esophageal replacement allows a normal life with normal oral uptake of nutrition.

[Behavior of pO2 on rabbit small intestine serosa, measured with the Clark electrode in different models of intestinal ischemia]. / Verhalten des pO2 auf der Serosa des Kaninchendünndarms, gemessen mit der Clark-Elektrode bei verschiedenen Modellen intestinaler Ischämie.

The pO2 on the surface of the small intestine serosa was measured on an isolated small intestine loop of the rabbit after synchronistic arterial and venous ligature of the mesenterium, after synchronistic arterial starvation and venous ligature, and after torsion of the mesenterium. The measurements of the pO2 were carried out by means of a Clark-electrode. Different periods for the decrease of the pO2 to zero Torr were shown in the different models of intestinal ischemia. Only the difference between the synchronistic arterial and venous ligature and torsion was statistically significant.

[Use of fibrin glue in prevention and therapy of intra-abdominal adhesions]. / Die Anwendung des Fibrinklebers zur Prophylaxe und Therapie intraabdomineller Adhäsionen.

In prevention of the recurrent intestinal obstruction we performed at the Department of Pediatric Surgery of the University of Mannheim a sutureless plication of the small bowel with fibrin glue only over the last 7-year period. The postoperative course was uncomplicated in all patients. The clinical and experimental experiences suggest that the high concentrated human fibrinogen is able to start healing of the lesions of the serosa to prevent intraabdominal adhesions prospectively. Furthermore the time saving and easy procedure is to be stressed. First of all the high risk of tissue necrosis or intestinal perforation due to ischemia by sutures and stitches like in the traditional technique of plication is not present.

[Incidence of accessory adrenal cortex tissue of the inguinal region in the child--results of a prospective study]. / Häufigkeit akzessorischen Nebennierenrindengewebes in der Inguinalregion beim Kind--Ergebnisse einer prospektiven Untersuchung.

Aberrant adrenal cortical tissue is often found in close contact to the urogenital system. In a prospective study covering 150 operations for inguinal hernia, and 110 operations for undescended testes, we looked for aberrant adrenal cortical tissue. In 5.2% of the inguinal hernias and 10.9% of the undescended testes, aberrant adrenal cortical tissue was found. The frequency was higher than reported in the literature. The aberrant adrenal cortical tissue is of clinical importance only in the very rare cases of development of malignant tumours in this tissue.

[Abnormalities of the common bile duct and Vater's papilla in various forms of duodenal atresia]. / Fehlbildungen des Ductus choledochus und der Papilla Vateri bei den verschiedenen Formen der Duodenalatresie.

Associated anomalies are often found in newborn with duodenal obstruction. Anomalies of the biliary tract are of special interest. Malformations of the terminal common bile duct and the papilla of Vater are the most common ones. The recognition of the possible anatomical variations is most important to avoid inadvertent injuries in the correction of duodenal obstruction.

[Anesthesia for congenital diaphragmatic hernia]. / Anaesthesie bei angeborener Zwerchfellhernie.

Hypoplasia of the lungs is the cause of the high mortality of newborns with diaphragmatic hernia. Survival depends mainly on the development of the contralateral lung. Eighty percent of diaphragmatic hernias are postolateral hernias of the left side. The most serious postoperative complication is a relapse into fetal circulation with increased pulmonary vascular resistance and right-to-left shunting (Fig. 2). The clinical signs of diaphragmatic hernia are cyanosis and tachypnea. Intermittent suction via a nasogastric tube and early intubation without mask ventilation should be performed. The inspiratory pressure should not exceed 25 cm H2O to minimize the risk of pneumothorax. Survival of the baby is unlikely if the initial blood gas analysis shows pH less than 7.10, pO2 less than 50 mmHg, and pCO2 greater than 65 mmHg. Hypothermia should be strictly avoided because it leads to increased oxygen consumption. Intraoperative monitoring should include a precordial stethoscope, ECG, blood pressure, and rectal temperature. Anesthesia is maintained with fentanyl 0.02-0.03 mg/kg body wt. and pancuronium 0.08-0.1 mg/kg. One dose of atropine (0.02 mg/kg) is administered before fentanyl. Intraoperative ventilation is performed by hand or by use of a Siemens Servo ventilator. Thirty newborns were anesthetized for repair of a congenital diaphragmatic hernia with no intraoperative complication and an overall mortality of 27%.