First-trimester diagnosis of fetal hepatic cyst.

Fetal intra-abdominal cysts seen on antenatal sonography pose a diagnostic problem as they may have many etiological origins. We present a case of a hepatic cyst measuring 11 x 7 x 7 mm that was diagnosed at 13 weeks' gestation by transvaginal sonography. The cyst increased in proportion with the growth of the fetus. Ultrasound-guided needle aspiration of the cyst at 22 weeks' gestation helped to clearly identify the formerly displaced gall bladder and demonstrated the intrahepatic location of the cyst. The aspirated fluid was identified as bile. After aspiration the fluid reaccumulated rapidly. Shortly prior to delivery the cyst measured 75 x 44 x 46 mm. At 39 weeks of gestation a female infant was delivered by forceps (3610 g; Apgar 9/10/10 at 1, 5 and 10 min, respectively). Increasing cyst size and concomitant feeding problems prompted surgery on the 14th day postpartum. A large hepatic cyst was partially excised and marsupialized, confirming the prenatal diagnosis. The postoperative course was complicated by cholangitis, septicemia and recurrence of the cyst. Therefore Roux-en-Y hepatojejunostomy was performed in the second month of life. The postoperative period was uneventful and the child was doing well at the time of writing.

Intussusception in preterm infants: case report and literature review.

UNLABELLED: Intussusception is an extremely rare disorder in preterm infants. An infant of 29 weeks gestational age with this condition is reported and a retrospective analysis of 17 previously reported cases presented. In the reviewed preterm infants, risk factors for intussusception seemed to be multifactorial. Clinical features included severe abdominal distension (17/17), gastric aspirates (13/17), commonly bilious, bloody stools (10/17) and rarely a palpable abdominal mass (5/17). Diagnostic features were signs of small bowel obstruction on the abdominal radiographs. Signs and symptoms were similar to those seen in necrotizing enterocolitis, therefore difficulties in establishing a correct diagnosis led to an average delay of 7 days between the onset of symptoms and abdominal surgery, increasing the risk of developing a compromised bowel. CONCLUSION: The differential diagnosis of an intussusception should be considered in preterm infants with acute abdominal distension and tenderness.

Morphologic alterations of the enteric nervous system and deficiency of non-adrenergic non-cholinergic inhibitory innervation in neonatal necrotizing enterocolitis.

Although damage to intramural nervous tissue should be expected in neonatal necrotizing enterocolitis (NNEC), as the pathology of NNEC is defined by substantial destruction of the bowel wall, only a few studies have considered its implication. Thus, the aim of the study has been to examine morphological alterations of the enteric nervous system (ENS) in intestinal segments affected by NNEC. Immunohistochemical methods allowed the demonstration of both neuronal and glial elements and the assessment of an altered localization of non-adrenergic non-cholinergic (NANC) inhibitory mediators within the intramural plexuses. Intestinal segments from patients with NNEC (n = 8) and control subjects (n = 3) were obtained and submitted to immunohistochemical examination incubating with antibodies against protein gene product (PGP) 9.5, protein S-100, vasoactive intestinal polypeptide (VIP) and nitric oxide synthase (NOS). The most severe damage of nervous tissue was found within the plexus mucosus and plexus submucosus internus. The ganglionated plexuses showed a loss ot both glial and nerve cells with various stages of cell deterioration and the formation of central lesions within the myenteric ganglia. The observed neuropathologic changes correspond to the group of acquired segmental hypoganglionosis. Specimens from patients with NNEC were also characterized by an absence of immunoreactive VIP and NOS in the plexus submucosus and within the circular muscle layer. The deficiency in NANC inhibitory innervation may contribute to the formation of functional obstructions following acute NNEC. Furthermore, it is likely that the neuropathological lesions induced in early stages of NNEC may result in dysfunctional intestinal motility facilitating intraluminal bacterial overgrowth and translocation, and therefore, possibly promote the self-perpetuating pathophysiologic cycle culminating in progressive NNEC. As an additional finding, two patients with NNEC showed typical features of intestinal neuronal dysplasia (IND). The association of NNEC and IND is reviewed in the literature and possible causalties are discussed.

Alterations of the enteric nervous system in neonatal necrotizing enterocolitis revealed by whole-mount immunohistochemistry.

Pathology reports on neonatal necrotizing enterocolitis (NNEC) rarely consider its effects on the enteric nervous system (ENS). Thus, the aim of this study has been to perform a two-dimensional assessment of neuropathologic lesions within the three ganglionated plexuses of the intestinal wall by means of whole-mount immunohistochemistry. Resected segments of ileum and colon affected by acute NNEC were submitted to immunohistochemical procedures using antibodies against neuronal (protein gene product 9.5) and glial (protein S-100, glial fibrillary acidic protein) proteins. Examination of the myenteric plexus and external submucosal plexus revealed a noticeable reduction in glial cells concomitant with the gradual deterioration of nerve cells, both findings predominating in the antimesenteric intestinal circumference, where ischemic lesions tend to appear first. The most severe damage of nervous tissue was observed in the plexus submucosus internus dependent on the depth of mucosal injury. The destroyed ganglia appeared like "empty baskets" (residual tangles) and housed deteriorated nerve and glial cells. Taking the anatomy of the intestinal vascular blood supply into consideration, the characteristic topography of neuropathologic lesions gives further support to an ischemic event within the cascade of different pathogenetic factors culminating in NNEC. Moreover, the demonstrated alterations of the ENS and their potential adverse effects on intestinal motility and neuroimmunologic interactions may contribute to the complex pathogenesis of NNEC, which remains a field of further investigation.

[Peripheral neuroectodermal tumor of the thoracic wall. Experiences with surgical treatment]. / Der Periphere Neuroektodermale Tumor der Thoraxwand. Erfahrungen mit der operativen Behandlung.

The peripheral neuroectodermal tumor (PNET) is seen nowadays as a more specified form of Ewing's Sarcoma with a worse prognosis. We report our experiences of three children (10-14 years old) with PNET of the right thoracic wall, all treated surgically within 1 year. Complete local resection-the main aspect of PNET therapie-of the initially voluminous tumors was not difficult due to chemotherapy and radiotherapy for 5 months prior to surgery.

Expansion of mucosal surface by intestinal patching in experimental cases of short bowel.

Young female beagles underwent either 85% resection of the small intestine; simultaneous 85% resection and patching of incised ideal remnant with colon serosa or with muscularis propria from transposed colon; or muscularis propria patching of ileum without resection of the small intestine. Patch areas were created in antiperistaltic position of participating bowel loops. After 20 weeks 50% of serosal patch and 34% of muscularis propria patch were overgrown by neomucosa. After 40 weeks 75% of the serosal patch was covered by neomucosa. Serosal patch area at necropsy 20 weeks after 85% resection of small intestine was 60% of the initial size; muscularis propria patch showed 44% of its primary area, but was only 12% of the initial surface area without bowel resection. Increases in resorptive surface consisted of neomucosa and an impressive adaptive elongation of short bowel, that was intensified by the simultaneous growth of the neomucosa.

Histopathological features of neuronal intestinal dysplasia of the plexus submucosus in whole mounts revealed by immunohistochemistry for PGP 9.5.

Neuronal intestinal dysplasia (NID) is wellknown, but its definition is a topic of debate. The histopathological diagnosis of NID is based on traditional enzyme-histochemical methods such as the acetylcholinesterase and dehydrogenase reaction on native cryosections. In this study, we have investigated the enteric nervous system in whole mount preparations of resected intestinal segments affected by NID of the plexus submucosus (type B). The plexuses of the tunica mucosa and tunica submucosa were visualized by immunohistochemical methods using a polyclonal antibody to protein gene produce 9.5 (PGP 9.5). PGP 9.5 is a novel general cytoplasmatic marker specific for the nervous system. The morphology of the plexuses is revealed in full, making possible changes easily discernible. Known pathological findings of the NID can be identified and judged more precisely with this method. Numerous enlarged nerve trunks run within the tunica submucosa and tunica mucosa. Hyperplastic ganglia with an unusually high nerve cell number in the tunica submucosa can be demonstrated as well as heterotopic nerve cells in the tunica mucosa.

Immunohistochemistry of markers of the enteric nervous system in whole-mount preparations of the human colon.

In this study we tested the immunohistochemical reactions of various markers for the enteric nervous system in whole-mount preparations of the human colon. For that purpose we used polyclonal antibodies against the neuronal markers--protein gene product 9.5 (PGP), neuron-specific enolase (NSE), neurofilament protein 200 (NFP), microtubule-associated proteins (MAPs); and the glial markers--S-100 protein and glial fibrillary acidic protein (GFAP) for the immunoperoxidase reaction. Whole-mount preparations are more suitable for histopathological evaluation and interpretation than sections, because the enteric nervous system consists of three-dimensional plexuses lying within the layers of the intestinal wall. Sections show only a part of the plexuses, neurons and glial cells. On the other hand, whole-mount preparations reveal the morphology of the plexuses as a whole. Among the neuronal and glial markers used, S-100 protein, the neurofilament protein, and the protein gene product 9.5 (PGP) produced the best results. Furthermore, this developing method provides new possibilities for the histopathological analysis of defects in the enteric nervous system, such as neuronal intestinal dysplasia (NID).

[GFAP immunoreactivity in newly formed ileal mucosa in a serosal patch in dog colon]. / GFAP-Immunreaktivität in der Neomukosa des Ileums auf einem Serosa Patch des Colons beim Hund.

A Short bowel was induced in 8 beagle bitches by resecting 85% of the small intestine. Following resection, a tangential fixation was performed in antiperistaltic direction between the ileum and the proximal colon. A 15 cm segment of the remaining ileum was incised opposite to its mesenterial attachment and sewn onto the serosa of the neighboring segment of colon (Serosa patching). After 20 weeks of observation, segments of the ileum, the colon and the anastomosed intestinal segment were removed. The neomucosa covering the patch surface was investigated using immunohistochemical methods in order to demonstrate the presence of nerve fibers. The immunofluorescence reaction using anti-GFAP (glial fibrillary acidic protein) clearly demonstrated immunoreactivity in the formed neomucosa and in the underlying granulation tissue. The immunoreactive fibers were comparable with fibers demonstrated in the original mucosa. This finding is an indication that the neomucosa of a serosa patch may be functionally intact because the presence of nerves suggests that mucosal functions, e.g. resorption and secretion, are under nervous regulation.

[Acyl coenzyme A dehydrogenase deficiency of medium-chain fatty acids in a 9-year-old boy with adymia. A rare mitochondrial cytopathy which may be more common than previously assumed]. / Acyl-Coenzym A-Dehydrogenase-Defekt mittelkettiger Fettsäuren bei einem 9jährigen Jungen mit Adynamie. Eine seltene mitochondriale Zytopathie, die vermutlich häufiger vorkommt, als bisher angenommen.

The medium-chain acyl-CoA dehydrogenase (MCAD) deficiency of mitochondrial beta oxidation has been identified in a nine-year old boy with a very bland course and easy fatigue as the main symptom. Repeated low frequency stimulation test and EMG for excluding a myasthenia gravis, and screening for urinary organic acid excretion were helpful for the diagnosis. The EMG test at the m. trapezius by stimulation of the n. accessorius showed an extreme decrease of muscle power down to 49%. After i.v. injection of Edrophonium the loss of power of 20% was still significant, so that we could exclude a myasthenia gravis, but we had found signs of a generalised defect in cell chemistry. The diagnosis could be confirmed by a positive 3-phenylpropionic acid-test and moleculargenetic proof of the Adenine to Guanine mutation at position 985 in the MCAD cDNA (G985) with the polymerase chain reaction. The incidence of this organic aciduria is probably 1:60,000 in Germany, but with more attention to this disease and diagnosis of cases with bland courses the incidence will be higher. The MCAD-defect should be considered in the differential diagnosis of patients with Reye syndrome-like encephalopathies, non-ketotic hypoglycaemia or sudden unexpected deaths in infancy.

[Pacemakers of the upper urinary tract using power spectral analysis of spontaneous activity]. / Untersuchung der Schrittmacher der oberen Harnwege mit Hilfe von Leistungsdichte-Spektren der Spontanaktivität.

Spontaneous activity of isolated smooth muscle strips of the upper urinary tract has been analysed by time series analysis. Frequency components of spontaneous contractions of the human urinary tract have been determined quantitatively by means of power spectral analysis. This method allows to characterise exact frequency components of rhythmicity in various parts of the urinary tract and accordingly helps to localise the site of various pacemakers. The results indicate tentatively the existence of two different pacemakers located in the wall of calix and pelvis. Furthermore, it is assumed that these pacemakers complement each other both cooperatively and by additive functioning, as though in shifts (reverberation), to stimulate urine transport through the pyeloureter.

[Arthroscopy of the traumatized knee joint in childhood]. / Arthroskopie des traumatisierten Kniegelenkes im Kindesalter.

63 children underwent diagnostic arthroscopy of the knee carried out under general anaesthesia, 46 of them presenting an acute haemarthrosis, 17 because of recurrent pain of effusion that developed under remobilisation stress two or more weeks after the accident. Arthroscopy provides--in addition to radiographs and stability test--complete evaluation of internal derangement of the knee. Clinical diagnosis based on examination and radiographs alone was confirmed by arthroscopy in only 25% in our series. Several arthrotomies would have been performed following "safe" clinical diagnosis, but could be avoided by use of arthroscopy; in other cases, diagnosis could be corrected, thus influencing preoperative planning. Arthroscopy improves diagnostic accuracy and eliminates a "wait-and-watch" policy of treatment. For several chondral and meniscal lesions, arthroscopic operation is the therapy of choice.

[Comparison of Blount's sling and Kirschner wire fixation in the treatment of a dislocated supracondylar humeral fracture in childhood]. / Vergleich von Blountscher Schlinge und Kirschner-Drahtfixation zur Behandlung der dislozierten suprakondylären Humerusfraktur im Kindesalter.

From 1979 to 1982 we were treating 63 cases of displaced supracondylar fractures of the humerus. Patients underwent either closed reduction and fixation by collar and cuff immobilization (32 cases), or fixation by means of Kirschner wires following closed or (more often) open reduction (31 cases). Follow-up controls after an average time of three years clearly showed the more favourable results of the conservative method.

Bridging a gap in oesophageal atresia using Rehbein's technique: dilatation of a thread canal.

During the last 12 years, operations were performed on 144 infants with oesophageal atresia. In 18 of them who were suffering from an atresia with long gap between segments, perlon threads were laid surgically. Continuity of the oesophagus was attempted using Rehbein's technique. The newborn had type II and IIIb atresia according to Vogts' classification. 11 of these 18 infants survived the third week of life. The fibroepithelial canal that had developed along the perlon thread, was dilated in steps in 6. The clinical course and results are reported.

[Omphalocele and gastroschisis: problems in intensive medical treatment]. / Omphalozele und Gastroschisis: Probleme der intensivmedizinischen Behandlung.

Aetiologically and pathognomically, omphalocele and gastroschisis are different forms of occlusive disorders in the anterior abdominal wall of newborns. Their prognosis has clearly improved in recent years, owing to progress in surgical and conservative treatment, primarily on account of improved intensive medical care of the children concerned. Courses and survival rates of newborns with either malformation have proved to depend strongly on the initial condition of the patients. The authors' own studies, in agreement with most of the literature, have shown that the incidence of severe syndromes of malformation has been comparatively high among newborns with omphalocele, whereas children with gastroschisis are usually small-for-date newborns with low birth weight. This has been a frequent limitation to successful treatment. Major importance, in terms of intensive medical treatment, should be attributed to postoperative completely parenteral feeding, throughout the early postsurgical phase during which the patient is particularly endangered from recurrent disorders of passage, as well as to artificial respiration due to respiratory constraint on account of elevated diaphragm and disproportionality between the abdominal cavity and retroposed viscera. Early administration of amino acids, carbohydrates, fat, trace elements, immunoglobulins, and vitamins, with doses being systematically increased by a prespecified pattern, has proved to be necessary to counteract catabolism accompanied by somewhat severe disorders in wound healing, subacute obstruction, and massive attacks of septic infections. This is possible only through central venous catheterization. Resulting thrombotic or septic complications may be mitigated by partial heparinization of the children.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomotic leak and recurrent fistula following operation of oesophageal atresia.

Experiences from 14 disruptions of oesophageal sutures and with 8 cases of recurrent fistula from 1973 to 1982 are described. Healing of an early oesophageal insufficiency could be achieved by conservative means in 3 newborns. Conservative therapy seems to be possible if life-threatening clinical conditions are reduced effectively. 6 oesophago-pulmonary and recurrent oesophago-tracheal fistulas were closed operatively, two cases closed spontaneously.

Two-stage operations of small bowel in infancy and childhood.

Out of 813 laparotomies performed for surgical problems of small bowel in infants and children, resections were necessary in 327 patients. A primary enterostomy was done in 75 cases with subsequent anastomosis. The different surgical problems, the operative findings, the various procedures adopted and the results thereof are discussed here. The greatest danger was seen to arise from the ischaemia of the bowel and from peritonitis. In doubtful conditions of small bowel two-stage operation seems advisable.

[Necrotizing enterocolitis of the newborn infant]. / Die nekrotisierende Enterocolitis des Neugeborenen.

The experience at the Hospital for Pediatric Surgery in Dortmund with 47 cases of necrotizing enterocolitis in 16 years are reviewed. 43 patients had been transferred from pediatric hospitals for immediate operation. 7 newborns had developed gastric perforation, 25 previously intestinal perforation. 4 patients had undergone laparotomy for other indication before. The chance to survive the necrotizing process is smaller in case of inflammation of the small bowel than of the large bowel. The current knowledge about necrotizing enterocolitis is summarized, including modern aspects about the indication to operate.

[Atresia and obliteration of the small bowel in cases of necrotizing enterocolitis (author's transl)]. / Atresia und Obliteration am Dünndarm bei Enterocolitis necroticans.

As a result of necrotizing enterocolitis, one patient developed multiple obliterations of jejunum and ileum, partly over long stretches of bowel. In a second case of a neonate with enterocolitis necroticans, an ileum atresia formed in the second and third weeks of life, completely interrupting the continuity of the intestine. In the literature, we could only find one report comparable with each case.