[Osteomyelitis in children]. / Osteomyelitis im Kindesalter.

Acute hematogenous osteomyelitis is a typical childhood illness, occurring predominantly in boys. The main symptom is inflammation but poor general condition also occurs occasionally. Elevation of CRP and sedimentation rate as well as leucocytosis are the most important laboratory findings. Conventional x-ray is mandatory, however scintigram and MRI are now specific in the early stage. Ultrasound can show abscess formation in the soft tissue. Treatment involves the immediate use of specifically targeted, intravenous antibiotics. Surgical intervention stands in second place. Immobilisation is only useful in the initial treatment of pain. Strict diagnostic and therapeutic management prevents complications, which would need reconstructive bone and soft tissue reconstruction, and is the key to a good primary prognosis.

Ileoileal intussusception caused by a Ewing sarcoma tumour. An unusual case report.

Intussusception typically occurs in childhood, presenting with a well-known medical history and clinical symptoms. Pathologically, a "leading point" may be attributed to lymphadenomatosis, polyps, or a tumour. In older patients and adolescents, the diagnosis can be complicated due to the lower incidence and variable subacute symptoms. We report on an 18-year-old patient with increasing abdominal discomfort over several weeks. External diagnostics showed no pathological signs or were misinterpreted as a malfunction of intestinal motility. The patient experienced increasing colics, recurrent vomiting, dehydration and weight loss. Finally he was transferred to our paediatric surgical department and laparotomy had to be performed for the clinical and radiological signs of an ileus. An ileoilealic intussusception was found, caused by a small bowel tumour, which almost completely obstructed the intestinal lumen. It was resected and bowel continuity was re-established. Histopathology revealed a very rare, highly malignant mesenchymal Ewing sarcoma, infiltrating the complete bowel wall. After the postoperative course, the patient was transferred to our oncological department for chemotherapy. In older children or young adults, intestinal malignancies are extremely rare. Nevertheless, if these patients suffer from unspecific complaints of chronic intestinal obstruction, a tumour must be ruled out. A Ewing sarcoma may be responsible for an intussusception.

Intrauterine repair of gastroschisis in fetal rabbits.

OBJECTIVE: Infants with gastroschisis (GS) still face severe morbidity. Prenatal closure may prevent gastrointestinal organ damage, but intrauterine GS repair (GSR) has not been established yet. METHODS: In New Zealand White rabbits we developed and compared GS versus GSR: creation of GS was achieved by hysterotomy, right-sided laparotomy of the fetus and pressure on the abdominal wall to provoke evisceration. GSR was accomplished by careful reposition of eviscerated organs and a running suture of the fetal abdominal wall. For study purposes, 18 animals were divided equally into 3 groups: GS, GS with GSR after 2 h, and unmanipulated controls (C). Vitality was assessed by echocardiography. After 5 h all animals were sacrificed. RESULTS: GSR inflicted no increased mortality, because all fetuses survived GS or GS with GSR. All fetuses with GS demonstrated significant evisceration of abdominal organs. In contrast, the abdominal wall of the fetuses from GSR was intact. CONCLUSION: The present animal model demonstrated the technical feasibility and success of an intrauterine repair of GS for the first time. However, further long-term studies (leaving GS and GSR in utero for several days) will be necessary to compare survival rates and intestinal injury, motility or absorption. The clinical application of GSR in utero remains a vision so far.

Does the transplantation process modify the immunogenicity of fetal adrenal grafts in rat?

The concept that fetal tissue transplants enjoy an immunologic privilege grounds on the primary immaturity of major histocompatibility complex (MHC) expression. However, experiences in human organ transplantation reveal that the immunogenicity of any graft could be modified by external factors such as ischemia. Consequently, the question arises, whether the process of transplantation modifies the immunogenicity of fetal grafts. In a syngeneic rat model (Lewis), fetal adrenal glands were transplanted into the greater omentum of adult hosts. After harvesting the grafts sequentially, the immunogenicity was evaluated by analyzing the expression and distribution of the MHC classes I and II and were compared with untreated organs of equivalent age. The untreated fetal adrenal gland depicted little immunogenicity. However, compared with age-matched untreated control organs, at 2 wk after transplantation, the grafts demonstrated an increased expression of MHC I and II, upregulated throughout the entire adrenal cortex. No signs of MHC-mediated rejection were found. The upregulation of MHC persisted until the eighth week after transplantation. At 3 months after transplantation the expression of MHC I and II returned to the normal pattern of untreated controls. As this study used a purely syngeneic model, the immunologic changes observed could not be induced by a graft vs. host incompatibility, instead they were caused by experimental factors. The expressions of MHC class I and II was increased at 2 wk, but these proteins did not induce a T-cell mediated rejection or cellular infiltration. In conclusion, these findings question the concept of an immunologic privilege of fetal tissue transplants. Instead, experimental factors may modify the tissue's primary immaturity of its MHC. Further investigations must evaluate, whether the increase in MHC expression will have an impact on the rejection of fetal adrenal grafts in allogeneic hosts.

Laparoscopic resection of a congenital liver cyst and simultaneous closure of a diaphragmatic defect in a 5-month-old infant.

Congenital liver cysts are an unusual finding and present a difficult diagnostic challenge. In a 5-month-old infant, routine echocardiography detected a cystic lesion (3 cm in diameter) just below the diaphragm. All conventional diagnostics, including magnetic resonance imaging (MRI), failed to clarify its etiology definitively. Moreover, a phrenic irregularity was suspected at the site of Morgagni. Finally, laparoscopy (5-mm scope and instruments) confirmed the diagnosis of a solitary liver cyst and a diaphragmatic defect. Using a Harmonic scalpel, the cyst was resected and the diaphragmatic defect was closed with interrupted nonabsorbable sutures. The patient's postoperative course was uneventful. Pathological analysis showed a true liver cyst with mesothelial lining and hepatic residues. Retrospective speculation suggests a common embryologic origin for both malformations. In conclusion, laparoscopy allowed final diagnosis and simultaneous treatment of an infant with the rare combination of a liver cyst and a diaphragmatic defect. To our knowledge, this case represents the first endosurgical experience of such a simultaneous procedure.

Organ-specific maturation of the major histocompatibility antigens in rats.

The essential role of major histocompatibility complex (MHC) class I and II in the process of rejection has been documented, and some studies suggest that fetal transplants could enjoy an organ-specific immunologic privilege. However, little is known as to when these antigens develop in fetal organs and which tissues mainly present them. This study investigated the dynamics of immunogenicity in the developing transplant organs of rats. The study focused on the classic transplant organs including lung, heart, liver, pancreas, intestine, and kidney. Fetal organs (14th and 20th day of gestation), organs at the 3rd, 7th, 10th, 14th and 28th days postpartum (pp), 2 and 3 months pp, and adult organs were taken and snap-frozen in liquid nitrogen. MHC expression was analyzed applying the APAAP technique on serial cryosections by two well-defined monoclonal antibodies (mAb) generated against rat MHC class I (Ox 18) and class II (Ox 6). Immunoreactivities were compared to those of different monoclonal markers against endothelial cells (HIS52, CD 31), histiocytes (ED 1, ED 2), dendritic cells (Ox-62), granulocytes (HIS48), B-cells (RLN-9D3), T-cells (Ox-52), CD 4 (Ox-35), CD 8 (Ox-8a), natural killer cells (10/78), and CD 45 (Ox-1, leukocyte common antigen). A non specific mAb (MR 12/53) served as a negative control. In all stages of organ maturation, MHC I expression was found predominantly on immunocompetent cells, endothelial cells, and certain parenchymal cells, whereas MHC II was almost entirely restricted to dendritic cells. In organ development, the onset of MHC I expression and the number of MHC II-positive cells varied in a time-dependent manner. However, between the 2nd and 3rd month pp the expression pattern was comparable to adult organs. The study indicates that each organ carries a variable immunologic burden, that matures heterogeneously. Consequently, the variable content of MHC I/II in organ maturation needs to be considered for any transplantation model.

[Perioperative pain therapy. Deficiencies in children and adolescents]. / Perioperative Schmerztherapie Im Kindes- und Jugendalter besteht erheblicher Handlungsbedarf.

Present studies reveal that postoperative pain management for children is still insufficient in Germany. This situation is not only caused by a lack of knowledge, but, mainly, by a deficiency of standardized protocols in the hospitals and the lack of administrative responsibility. This article describes a specific concept for postoperative pain management in children. It basically focuses on three main topics: prevention of pain, the algorithm for analgesic treatment and the evaluation of the efficiency of the treatment. The surgeon plays a central role in pain prevention. By intraoperative blocking of the peripheral nerves, he can achieve effective analgesia. Postoperatively, he prescribes a basic medication according to the procedure used and the surgical trauma. Its effectiveness must be measured routinely thereafter and, depending on the score, an individual pain medication should be added. Pain measurement in children requires the application of specific scales for each age group. The evaluation of this concept has revealed therapeutic success, broad acceptance and efficiency in ward use. However a long-term benefit can only be achieved when postoperative pain management is one of the essential duties of the surgeon and it is constantly monitored within a department.

Testosterone-producing adrenocortical neoplasm in a 6-year-old boy.

Adrenal tumours that predominantly secrete testosterone are virtually unknown in prepubertal male patients. We present the case of a 6-year-old boy with premature sexual development and markedly elevated serum testosterone, but normal urinary steroid levels. Diagnostic imaging demonstrated a spherical tumour of the left adrenal gland. Surgical excision led to normalisation of testosterone levels, and postoperative serial low hormone measurements ruled out tumour recurrence.Although extremely rare, this case illustrates that testosterone-producing adrenal adenomas may be encountered in boys without urinary steroid elevation. Surgical excision promises a definite cure. Testosterone is a useful tumour marker in these patients until the beginning of puberty.

Transient pseudohypoaldosteronism secondary to posterior urethral valves--a case report and review of the literature.

In transient pseudohypoaldosteronism (TPHA), renal tubular resistance to aldosterone is thought to be secondary to renal disease. We report a case of TPHA caused by posterior urethral valves associated with urinary tract infection and review 62 cases previously reported. The infant presented with unspecific signs of vomiting and dehydration, so that pyloric stenosis was first suspected. Laboratory data and retroperitoneal sonography led to the diagnosis TPHA. This case illustrates that urine culture and renal ultrasonography should be performed in any infant with electrolyte disturbances to exclude infection or obstructive uropathy.

A computerized vector manometry and MRI study in children following posterior sagittal anorectoplasty.

The study was designed to evaluate computerized eight-channel vector manometry (8CVM) and pelvic floor magnetic resonance imaging (MRI) as methods to assess the anal sphincter following posterior sagittal anorectoplasty (PSARP) for anorectal malformations, in particular the functional capacity of the sphincter structures in correlation with postoperative MRI findings. Seventeen children had been operated upon for a rectovesical, -urethral, or -vaginal fistula including one female cloacal malformation; 4 had a secondary PSARP. Mean follow-up was 5.57 years. Continence was evaluated with a modified Kelly score. A CVM technique with an eight-channel perfusion catheter was used. In addition to software-supported data, the manometric parameters included a score-system assessing three different pressure zones of the anal canal qualitatively on the three-dimensional image of the anal sphincter profile. The same procedure was performed on sagittal, oblique axial, and oblique coronal MRI. Furthermore, the thickness of the sphincter muscle was assessed at the level of the maximal mean segmental pressure. All children had decreased absolute vector-volumetry values at rest and on squeezing. Correlation with the clinical score was poor. Correlation of the manometric score with the clinical course was similar to the correlation of MRI score with clinical course (R = 0.425; P = 0.1). Thirteen children demonstrated normal or increased sphincter length; 5 of these had a decreased high-pressure zone (HPZ). The position of the anorectum in the sphincter muscles could be evaluated by the vector-volumetry image as anatomic in 11 cases, nearly correctly positioned in 4, and ectopic in 1 child. MRI detected 2 cases of malposition, 10 anatomic, and 4 nearly-anatomic findings. Correlation of the manometric score with the MRI score and the thickness of the sphincter muscle at the HPZ was significantly high (R = 0.801; P < 0.0001). 8CVM is thus highly sufficient in illustrating the function of the sphincter musculature seen on pelvic floor MRI. Both methods only moderately reflect clinical follow-up, since continence depends on more than sphincter ability.

Multiple intestinal stenoses and peripheral gangrene: a combination of two rare surgical complications in a child with Kawasaki disease.

Kawasaki disease (KD) often presents with a challenging variety of clinical symptoms. Severe gastrointestinal complications are rare and mainly appear as pseudo-obstruction. However, the authors report the unique case of a 4-month-old girl with KD suffering from a mechanical ileus. The optimal timing of surgery presented a dilemma, because she received lytic treatment for gangrenes of both her hands and feet and additionally had large coronary artery aneurysms. Finally laparotomy had to be performed while the patient was on an anticoagulant medication, and it showed a 30-cm-long jejunal segment with multiple filiforme stenoses, requiring resection and anastomosis. The postoperative course was uneventful regarding the abdominal situation; however, the left hand and left foot remained gangrenous and had to be amputated. In patients with KD, not only pseudo-obstruction, but irreversible intestinal obliteration has to be encountered. This combination of intestinal stenosis and acral gangrene has not been described before. J Pediatr Surg 36:651-653.

[Circumcision--criticism of the routine]. / Die Zirkumzision--Kritik an der Routine.

Circumcision is one of the most frequent operative procedures done in males. About 120 circumcisions are performed every 5 minutes over the world [14]. Three different reasons lead to circumcision: 1) Medical reasons in present of a pathologic phimosis. 2) Circumcisions done due to religious, social or cultural rea-reasons. 3) Finally in many countries circumcision is performed as "routine-circumcision" in the newborn period. While in the United States the number of routine-circumcisions decreases (about 60% of all male newborns) South-Korea has a rate near to 100%. Even with no religious or cultural background in Germany circumcision often is performed without scrutinizing medical indication. Circumcision is regarded as an procedure with no complications and no disadvantage for the patient. In general circumcision has no medical benefit neither in decreasing the incidence of urinary tract infections nor of sexual transmitted diseases nor of neoplasias. Medical indication for circumcision is given in present of pathologic phimosis in 4% of all males. Postoperative complications range up to 2% and "circumcision is the amputation of the prepuce from the rest of the penis, resulting in permanent alteration of the anatomy, histology and function of the penis...". There are many reports about having discomfort and disadvantages after circumcision as well to the males as to the sexual partners. This challenge the legality of neonatal involuntary circumcision because legality is based on saving the children's best interests.

Primitive neuroectodermal tumor of the pancreas. An extremely rare tumor. Case report and review of the literature.

Peripheral primitive neuroectodermal tumor (PPNET) is a malignant neoplasm of the peripheral nervous system and soft tissues. Representing the fourth case published we herein report a PPNET arising in the pancreas of a six year old girl. She presented with severe anemia due to ulcerative tumor growth and hemorrhage into the duodenum. From the first biopsy pancreatoblastoma was considered as histological diagnosis. Therefore pancreato-duodenectomy was successfully performed. Immunohistochemically, the tumor cells were positive for cytokeratines and several neuronal markers. Due to focal membranous staining for MIC-2 gene product and rosettes in one lymph node metastasis the diagnosis had to be altered into PPNET. This was confirmed by cytogenetic analysis. We conclude that the interpretation of histologic sample excisions from pediatric pancreatic neoplasms may be difficult and that PPNET should be included in the differential diagnosis.

Organ-specific distribution of major histocompatibility antigens in rats.

The present study systematically investigated the expression and distribution of the major histocompatibility complex (MHC) classes I and II in the rat. About 150 native tissue probes from eight adult Lewis rats were taken, representative for most organs, tissues, and the vascular system. MHC expression was analyzed by two monoclonal antibodies (mAb) generated against the non-polymorphic determinants of rat MHC class I (Ox-18) and class II (Ox-6). Immunoreactivities were compared to those of different endothelial (HIS52, TLD-3A12, Ox-43, REHA-1 antigen), histiocytic (ED1, ED2), B-cell (RLN-9D3), and T-cell (MRC Ox-52) markers. A nonspecific mAb (MR12/53) served as a negative control. Pretested concentrations on various tissues and the alkaline phosphatase-anti-alkaline phosphatase technique allowed semiquantitative evaluation of serial cryostat tissue sections. MHC class I expression was detected on most immunocompetent cells. Endothelial cells were stained heterogeneously along the vascular system and the organ-specific microcirculation. Furthermore, some organs showed staining of parenchymal cells. MHC class II was found on all immunocompetent cells positive for the B-cell marker and about 15% of cells positive for the histiocytic markers. Besides the well-known expression of MHC class II in the outer zone of the renal proximal tubule, further organ-specific cell forms were found positive. In conclusion, the present study outlines tissue-specific distribution of MHC I/ II and implies that each organ carries a variable immunologic burden that needs to be considered for any transplantation model.

Proliferation, zonal maturation, and steroid production of fetal adrenal transplants in adrenalectomized rats.

The present study investigated the histologic maturation, proliferative capacity, and steroid production of fetal adrenal transplants (Tx) in adrenalectomized rats. A pair of fetal adrenal glands (18-20 days of gestation) was transplanted into the omentum of syngeneic Lewis rats (n = 45). Four weeks later, in 5 animals the grafts were excised for morphologic evaluation. Proliferation was investigated by immunohistochemical staining for KI-67 protein and quantified by the proliferation index (PI = positive cells/100 counts). All other hosts (Tx; n = 40) underwent bilateral adrenalectomy (AE) to induce Addisonian crisis. Postoperatively, survival and concentrations of potassium, sodium, aldosterone, and corticosterone were recorded for 6 months. These data were compared to controls (C=only AE; n = 30) and a sham group (S; n = 10). At the end of the study period all surviving hosts were killed for histologic examination of grafts. At 4 weeks post-Tx the adrenal grafts demonstrated a distinct zona glomerulosa and frequent proliferation with a PI of 0.084, comparable to normal control (0.092). Following AE survival was significantly prolonged in Tx (86% vs 12% of C, P < 0.05). Control animals developed severe hyponatremia and hyperkalemia, whereas in Tx only transient signs of Addisonian crisis were recorded. Levels of aldosterone dropped within 7 days in the Tx and C groups, but returned to normal for Tx within 8 weeks. Corticosterone levels of Tx animals fell to 25% within week, but steadily increased to 70% by the end of the study. At 6 months, grafts revealed a mature adrenocortical structure with little proliferative activity, which was comparable to controls. In a syngeneic rat model fetal adrenal transplants thus mature and proliferate to provide sufficient steroid production for adrenalectomized hosts.

A computerised vector manometry study of the so-called ectopic anus.

There are several totally different definitions of the so-called ectopic anus. To determine whether computerized eight-channel manometry helps to define the entity of the ectopic anus, ten patients (nine females) were operated upon for an ectopic anus by the anal transposition technique (perineal pull-through procedure). Besides the software-supported manometric data, the qualitative imaging analysis was of interest. We calculated the factor by which the pressure of the three ventrally-located segments was lower than the mean segmental pressure at this part of the anal canal. The results were compared to standard age-related values established in a former study in 100 children. Besides pre- and postoperative manometry, barium roentgenograms were obtained. The anal-canal length at rest in the ectopic anus was significantly (P < 0.0001) longer. The segmental asymmetry index in the lower anal canal (LAC) was increased, but not significantly. The pressure in the ventral segments of the LAC was significantly (P < 0.0001) decreased and was less than one-half of the mean segmental pressure in this zone. We found a significant correlation between the degree of anterior displacement and the factor by which the ventrally-located pressure values were decreased. Postoperatively, this factor increased significantly. From a functional point of view, the definition of the ectopic anus includes a deficient high-pressure zone ventrally in the LAC. The LAC seems to run out or nearly out of the ventral sphincteric issue to end ectopically on the perineal surface.

Cryopreservation and transplantation of fetal adrenal glands in adrenalectomized rats.

In a syngeneic rat model, the present study investigated the cryobiology of fetal adrenal glands and compared the endocrinological function of cryopreserved versus fresh fetal adrenal transplants in the adrenalectomized hosts. Ultrastructural studies showed no significant tissue damage by the cryopreserving technique: cellular membranes were intact, the mitochondria showed discrete swelling and vacuoles were found in the endoplasmatic reticulum. Following transplantation, maturation and bilateral adrenalectomy in the host, assessment of endocrinological parameters demonstrated that survival was prolonged and Addison crisis could be prevented in both transplant groups with no significant difference between fresh versus cryopreserved grafts. To our knowledge this study presents the first morphological and endocrinological data about the successful transplantation of cryopreserved fetal adrenal glands in rats. In conclusion, cryopreservation is tolerated well by the tissue and long-term banking may therefore contribute to the feasibility and benefit of fetal adrenal transplantation. However, further investigations will be necessary to evaluate fetal adrenal transplantation in an allogeneic and xenogeneic setting.