Rectal atresia, choanal atresia and congenital heart disease: a rare association.

Rectal atresia is an extremely rare anorectal malformation. A unique case of rectal atresia presenting as an imperforate membrane associated with congenital cardiac malformations and unilateral choanal atresia in a newborn is reported. Ultrasound examination in the newborn infant located the presence of the rectal membrane which was surgically treated successfully.

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.

Omphalocele: clinical review and surgical experience using dura patch grafts.

Over a period of 15 years we surgically treated 50 cases of omphaloceles. The pathology occurred more frequently in boys (n = 30) than in girls (n = 20). The mean birth weight was 2995 g and mean gestational age was 38 weeks. Four infants (8%) were delivered vaginally and the rest (92%) by cesarian section. Eleven infants (22%) underwent primary closure, but in 20 infants (40%) with larger defects a primary closure of the skin was possible; however, a single solvent-dried dura graft implant was employed for the fascia enlargement. The remaining 19 infants (38%) had extremely large defects, and optimal closure of the defect required a two layered graft implantation. Twenty-five infants (50%) had associated anomalies, the majority being congenital cardiac anomalies. Five patients (10%) required secondary laparotomies due to bowel associated complications. Four patients (8%) experienced non-bowel-associated complications. The average postoperative mechanical ventilation required was for a period of 3.2 days and the average hospital stay was 45.7 days. The overall mortality rate was 8% (n = 4) and was largely due to severe congenital heart anomalies. Solvent-dried dura was successfully employed in the management of the larger defects with no major complications; only one patient (2%) had a local abscess around the area of the implant and was managed conservatively. Our experience favors the employment of solvent-dried dura graft implants for the repair of large omphaloceles. The solvent-dried dura grafts are biomaterials that promote rapid scar formation and integration with the adjacent skin tissue and do not produce any foreign body reactions at the site of implantation.

Laparoscopic appendectomy in children with Enterobius vermicularis.

As surgeons gain more experience with laparoscopy in children, it is becoming apparent that one of the best applications of the technique is for appendicitis. The advantages of laparoscopic appendectomy include a better cosmetic result, particularly in young female patients, a shorter recovery time, and an early return to normal activities. In the differential diagnosis of appendicitis, the association of Enterobius vermicularis (pinworms) inflammation in the pediatric age group is not uncommon. The endo-loop technique for laparoscopic appendectomy is the standard technique used in our department. In three patients with E. vermicularis undergoing appendix removal using this technique, pinworms were found to be set free into the abdomen after dividing the appendix between the loop ligatures. Bipolar coagulating forceps were used carefully to thermally desiccate the worms, which were then removed using a blunt grasper. E. vermicularis being released into the abdomen in children undergoing laparoscopic appendectomy using the endo-loop technique has not been reported. Careful evaluation of the appendix stump and simple but careful thermal desiccation and removal of pinworms, if present, must be considered when using the endo-loop technique.

Application criteria for infrared ear thermometers in pediatric surgery.

Fever is an important and sensitive indicator of infectious diseases in children. For the past decades, measurement of body temperature in routine medical practice was limited to oral, rectal and axillary sites. In infants and children, however, temperature measurements are further limited to the rectal and axillary sites due to technical and clinical considerations. In the field of pediatric surgery, pathological and surgical conditions of the ano-rectal area often further restrict the application of conventional rectal temperature measurements. The application of tympanic temperature measurements in such pediatric surgical pathologies provides a reliable alternative to conventional rectal temperature measurements. The pediatric surgical clinico-pathological states where ear temperature measurements are the only mode of accurate temperature determination have been identified. Tympanic thermometry is well tolerated by children and predicts temperature with relative accuracy within a few seconds.

Endochondral ossification of costal cartilage is arrested after chondrocytes have reached hypertrophic stage of late differentiation.

Late cartilage differentiation during endochondral bone formation is a multistep process. Chondrocytes transit through a differentiation cascade under the direction of environmental signals that either stimulate or repress progression from one step to the next. In human costal cartilage, chondrocytes reach very advanced stages of late differentiation and express collagen X. However, remodeling of the tissue into bone is strongly repressed. The second hypertrophy marker, alkaline phosphatase, is not expressed before puberty. Upon sexual maturity, both alkaline phosphatase and collagen X activity levels are increased and slow ossification takes place. Thus, the expression of the two hypertrophy markers is widely separated in time in costal cartilage. Progression of endochondral ossification in this tissue beyond the stage of hypertrophic cartilage appears to be associated with the expression of alkaline phosphatase activity. Costal chondrocytes in culture are stimulated by parathyroid hormone in a PTH/PTHrP receptor-mediated manner to express the fully differentiated hypertrophic phenotype. In addition, the hormone stimulates hypertrophic development even more powerfully through its carboxyterminal domain, presumably by interaction with receptors distinct from PTH/PTHrP receptors. Therefore, PTH can support late cartilage differentiation at very advanced stages, whereas the same signal negatively controls the process at earlier stages.

Vascularized three-dimensional skeletal muscle tissue-engineering.

Medical science continues to battle against the loss or failure of organs or tissues. Since, skeletal muscle loss lead not only to the functional compromise of the affected site, but also a structural deformation; tissue engineering of skeletal muscle attempts to provide solutions to replace loss of tissue contour and function. In our study, myoblasts seeded onto polyglycolic acid (PGA) meshes were used to engineer skeletal muscle tissue in vivo. The cell-polymer constructs harvested after a period of 6-weeks were well vascularized three-dimensional structures with the ability to generate neo-muscle-like tissue. This is the first time that the ability of myoblasts to survive in vivo in the absence of mature skeletal muscle tissue was demonstrated. The successful ability to transplant myoblasts using biodegradable polymer strands without using the traditional transplantation buffer mediums as carriers was also employed for the first time.

Non-retractile foreskin: a single center 13-year experience.

BACKGROUND: Non-retractile foreskin comprise a significant number of referrals by pediatricians and general practitioners to pediatric surgical centers. In attempts to find alternatives to widely practised circumcision, various procedures have been developed over the years in order to relieve the non-fibrotic narrowing of the foreskin. PATIENTS AND METHODS: In a 13-year period from 1984-1997, we treated 2554 patients with non-retractile foreskin at our center. Dorsal relieving incision was the technique of choice and was performed in 2177 patients, circumcision in 73 patients and preputial adhesiolysis was sufficient to retract the foreskin in 284 patients. RESULTS: Satisfactory esthetic results, an extremely low rate of postoperative complications (1.8%) with a recurrence rate of only 0.8% (18 patients) was observed in our series with dorsal relieving incision. CONCLUSIONS: Dorsal relieving incision operation was found to be a less invasive, safe and effective procedure for non-retractile foreskin not affected by extensive secondary scarring. This technique has fewer complications in comparison to circumcision and can be performed as an ambulatory procedure.

Skeletal muscle tissue engineering using isolated myoblasts on synthetic biodegradable polymers: preliminary studies.

Skeletal muscle is responsible for the control of voluntary movement and the maintenance of structural contours of the body. Muscle loss or deficiency is encountered in various pathological states, and attempts to correct them have been employed with limited success. The aim of the present study was to tissue engineer three-dimensional vascularized skeletal muscle using isolated myoblasts attached to synthetic biodegradable polymer for tissue replacement in the enhancement of muscle regeneration. Myoblasts derived from neonatal rats (3-5-day-old), Fisher CDF-F344, were seeded onto polyglycolic acid meshes and implanted into the omentum of syngeneic adult Fisher CDF-F344 rats. Rats were sacrificed on day 30 and day 45 after the transplantation, and the cell-polymer constructs were harvested for morphological analysis. Histological analysis of the constructs were performed by hematoxylin and eosin, and immunohistochemical staining was positive for alpha sarcomeric actin and desmin skeletal muscle marker. Viable myoblasts organized between strands of degrading polymer mesh formed the new tissue, and vascularization of the entire construct was observed. Organization of neomuscle strands surrounded by vascularized tissue composed of degrading polymer and fusing myoblasts demonstrated the ability of myoblast constructs to survive, reorganize and regenerate tissue-like structures. Since myoblast transplantation to date has been limited to the cellular level of replacement, myoblast-polyglycolic acid constructs may be useful in defining the application of tissue engineering for future skeletal muscle transplantations.

Surgical correction of pectus excavatum: the Münster experience.

OBJECTIVES: Pectus excavatum is the most common congenital hereditary chest-wall deformity. This study analyses a single-center experience of pectus excavatum-thoracic wall reconstruction using a uniform technique of internal stabilization employing stainless steel struts. METHODS: From June 1984 to December 1997, we performed correction operations on 777 patients with pectus excavatum. The condition occurred more frequently in boys (621 patients) than girls (156 patients). Surgical repair was performed using a standard method of double bilateral chondrotomy parasternally and at points of transition to normal ribs. This was followed by detorsion of the sternum, retrosternal mobilization and correction of the inverted ribs. The anteriorly displaced sternum was stabilized by one trans-sternal and two bilateral parasternal metal struts. RESULTS: The corrections were completed with successful repair in 765 patients (98.5%) with a low complication rate of 6.7%. The follow-up period ranged from 4 weeks to 12 years, mean 6.4 years. Major recurrences were observed in 12 patients (1.5%) and mild recurrence were observed in 35 patients (4.5%). CONCLUSION: Significant reduction in postoperative cardiorespiratory disorders, low lethality, improvement of subjective complaints, satisfactory long-term results and improvement in psychological problems indicate the need to offer this method of surgical correction to low-risk children.

Octylcyanoacrylate tissue adhesive in the repair of pediatric extremity lacerations.

Lacerations comprise a significant number of emergency department referrals for pediatric patients. Management of lacerations with sutures involves the use of needles and the injection of local anesthetic and represents a unique challenge in the wound management of an already distressed and frightened child. Octylcyanoacrylate, a new-generation, medical-grade tissue adhesive, has been found to be an effective alternative to replace skin sutures on virtually all facial lacerations and has been employed in low-skin tension wound management. Its use, however, has generally been avoided in the management of high-skin tension lacerations. Over the last 10 months, 32 children with high-skin tension (hand, feet, and over joints) lacerations were managed at our center by octylcyanoacrylate tissue adhesives. Skin closures and splints were applied to restrict movement of the affected area to overcome the limitation of adhesive application. Octylcyanoacrylate adhesive applied with optimal immobilization was found to be an effective method of skin closure in high-skin tension lacerations. Advantages of tissue adhesives for incision and laceration include quick application, excellent cosmetic results, patient preference, and cost effectiveness.

Thermography of Clostridium perfringens infection in childhood.

Gas gangrene is not a frequently encountered toxic wound infection in childhood. We present a case of postoperative Clostridium perfringens infection with proximal forearm myonecrosis. In order to reveal the full extent of tissue viability in the right upper extremity, infrared thermography was performed. Although dyschromia was evident in the proximal forearm, thermographs revealed viable tissue only up to the supracondylar region. Angiography, which provided valuable clues to the patency of the vascular supply, and subsequent intraoperative findings confirmed the extent of tissue perfusion as revealed by infrared thermography.

Surgical repair of pectus carinatum.

Pectus carinatum represents a variety of protrusion deformities of the anterior chest wall. Although various non-operative methods of treatment have been employed, surgery has been widely accepted as the only effective method for the correction of pectus carinatum. We evaluate our 14 year single center experience of pectus carinatum correction on 111 patients using a uniform technique of internal stabilization employing stainless steel struts. Operative correction required double bilateral chondrotomy parasternally and at points of transition to normal ribs, followed by detorsion of the sternum, retrosternal mobilization and correction of the everted sternum as well as of the everted and inverted ribs. The mobilized sternum after incomplete wedge osteotomy was finally stabilized by one transternal and two bilateral parasternal metal struts. The corrections were completed with successful repair in 109 patients (98.2%). Major recurrences in 2 patients (1.8%) were corrected while mild recurrence were observed in 3 patients (2.7%).

[Electron microscopy assessment of the effectiveness of intraoperative laser in reoperation of juvenile bone cysts]. / Elektronenmikroskopischer Nachweis der Effektivität intraoperativer Laseranwendung bei Rezidivoperationen juveniler Knochenzysten.

The application of the ND-YAG-Laser in juvenile bone cysts prevents recurrencies. That can be put down to the fact that the laser has a deep action which destroys the organells of the cystic tissue up to 2-4 mm irreversibly. By this the whole cystic tissue is being destroyed and recurrencies are being prevented.

[Significance of prenatal diagnosis in interdisciplinary treatment of sacrococcygeal teratoma]. / Bedeutung der pränatalen Diagnostik in der interdisziplinären Behandlung sakrococcygealer Teratome.

The perinatal treatment of 23 infants with sacrococcygeal teratomas was recorded prospectively from 1990 to 1997, during which period 14 children (teratomas 6.9-18 cm) could be followed throughout the whole pregnancy at our own perinatal department. There were three prenatal deaths (21 h-27 week of gestation) and two peripartal deaths (1 hydrops and 1 ruptured teratoma). Prenatal Doppler sonography allows appropriate selection of high-risk fetuses. The vital prognostic sign was developing fetal hydrops with associated umbilical vein pulsations, increased aortal flow and an increasing pulsatility index in der venous duct.

[Laparoscopic staging in abdominal tumors in children: an alternative to staging laparotomy]. / Laparoscopia diagnóstica en tumores abdominales infantiles: una alternativa a la laparotomía.

INTRODUCTION: In abdominal tumors in childhood, staging laparotomy is usually an essential diagnostic tool to obtain information about the histology and the extension of the tumor, when less invasive methods do not reach to clarify the process. PATIENTS: In fourteen children between 3 months and 17 years a laparoscopical procedure was performed. MATERIAL AND METHOD: Only in patients without a clear diagnosis after noninvasive exploration were submitted to an explorative laparoscopy in order to define the histology, the extension of the illness or to locate it. The laparoscopical instrumentarium used was from Dufner, adapted to children. RESULTS: The patients tolerated well this procedure, the posoperative period was short (mean 2.07 days), no complications after the operation are reported. Only one case of hemorrhage lead to a conversion after biopsy of a neuroblastoma. In all cases the diagnosis and staging was achieved. CONCLUSIONS: When non invasive diagnostic methods are not conclusive an explorative laparoscopy should be performed because it offers a lot of advantages over a laparotomy.

[Congenital sternal cleft. Diagnosis and treatment]. / Esternón bífido congénito. Diagnóstico y tratamiento.

The first known case of sternal cleft was described by Torres in 1740. Since then, many publications have appeared concerning sternal cleft, which have led to introduce a classification and some therapeutical procedures. This disease is a developmental anomaly of the sternum that usually shows vascular and cardiac malformations as well as diaphragmatic, abdominal wall, pericard and middle line organs anomalies. Two cases of sternal cleft are presented who have been treated in the neonatal period with different associated anomalies. Diagnostic procedures and therapeutical aspects are discussed.