Inflammatory pseudotumor (IPT)-surgical cure of an inflammatory syndrome.

UNLABELLED: We report on four female adolescents, who presented with inflammatory symptoms. Extensive diagnostic workup revealed tumors on different locations. After surgical removal, clinical and laboratory signs of inflammation disappeared rapidly. On histology, the tumors showed a mixture of inflammatory cells characteristic of inflammatory pseudotumors in three of the patients. CONCLUSION: In patients with unclear inflammatory symptoms, inflammatory pseudotumor should be added to the differential diagnosis. WHAT IS KNOWN: • The inflammatory pseudotumor (IPT) is a mostly benign myofibroblastic tumor of the soft tissue and causes inflammatory symptoms. What is new: • IPTs have may wider than hitherto defined histologic features. Removal of IPT is curative.

Fibroadenoma of the breast in an 11-year-old girl.

Fibroadenoma is the main cause of unilateral breast mass in teenagers and adolescents. 4% of these are a special form described as giant or juvenile fibroadenoma. For primary diagnosis, ultrasound is the method of choice. The MRI allows exact evaluation of size and location. The fibroadenoma must be distinguished from the phylloid tumour, which can be malignant. The latter occurs in patients of all ages, but peaks between the ages 40 and 50 years. Only 2% of all primary malignant breast lesions are found in women aged under 25. Metastases of other primary tumours must be excluded, especially with a history of prior malignancies. When planning the surgical excision, the final cosmetic result is important. Although the main reason of an asymmetrical breast enlargement of young girls is a benign mass, an early surgical excision is efficient with regard to the best possible cosmetic outcome.

Lymphangioma colli--a new classification contributing to prognosis.

The aim of the study was to introduce a classification of lymphangioma colli, which allows us to predict the expected morbidity and prognosis with surgical treatment. We defined 4 types, dependent on the volume of the lymphangioma as assessed by prenatal ultrasound or clinically: type I included tumors which had no or only a minimal effect on the contour of the neck; type II lymphangiomas were smaller than a line drawn at the lateral border of the head; type III tumors exceeded this line; in type IV the lymphangioma extended beyond the midline of the body. We report on the pre- and postoperative findings in 36 cases operated on between 1988 and 2000, mean follow-up was 4.2 years. 25 % of the cases were classified as type I, 36 % as type II, 22 % as type III, and 17 % as type IV. Cesarean section was carried out for 79 % of type III and IV lymphangiomas following prenatal diagnosis (71 %) and for 14 % of type I and II. MRT was indicated in 38 % of type II compared to 86 % in type III and IV lymphangiomas. Endoscopy was necessary in 25 % of type III and in 66 % of type IV lymphangiomas. In 2 cases (type I and IV), imaging revealed an intrathoracic portion. In 64 % of type I and II cases, resection was complete compared to 21 % in infants with more extended tumors. In all type IV cases, at least one further operation was required, likewise in 25 % of type III lymphangiomas. Postoperative effusion and infection occurred significantly more often in group IV than in groups I to III. 5 nerval lesions remained in children with operated type IV lymphangioma and 2 in type III cases. A visible lack of facial symmetry was evident at the follow-up examination in 83 % of type IV cases and moderate asymmetry was found in 19 % of all type I and II cases. Tracheostomy and gastrostomy were required only in type IV. The introduced classification of lymphangioma colli enables predictive statements to be made concerning the morbidity and prognosis with surgical therapy. The prognosis in type I is excellent; in type II, a cosmetically significant facial asymmetry remains in only 15 %. In type III lymphangiomas postoperative complications occurred in 38 % of cases, the necessity for reoperation with definitive lesions occurred in only 25 %. Morbidity gets significantly higher and the prognosis is worse if the lymphangioma exceeds the midline of the body.

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.

[Lymphangioma colli--what are the prognostic factors? Experiences with 36 cases]. / Lymphangioma colli--Welche Prognosefaktoren gibt es? Erfahrungen mit 36 Fällen.

AIM: A new classification of the Lymphangioma colli (LC) should allow to inform about prognosis at the time of diagnosis or prenatal sonography. METHODS: Pre- and postoperative parameters in 36 operated children (1988-2000, Ø follow up 4.3 years) were evaluated dependent on the volume of the LC as defined. Type I: the tumor does not alter the normal shape of the neck; type II: the LC reaches a line placed at the lateral border of the head; type III: the LC does not respect this line; type IV: the LC exceeds the midline of the body. RESULTS: According to delivery, diagnostics, resection, reintervention, postoperative complications, nerve injury, hospital stay, aesthetics, tracheo- and gastrostoma predominantly significant differences were found. CONCLUSIONS: The proposed classification permits to advise the parents about the expected prognosis: Type I: it is excellent; type II: some asymmetry remains in 15%; type III: postoperative complications occur in up to 38%; type IV: morbidity increases and prognosis decreases significantly.

[Differential therapy of hemangiomas--when cryotherapy, laser therapy or operation?]. / Differentialtherapie von Hämangiomen--Wann Kryo, Laser oder Operation?

Hemangiomas are the most common soft tissue tumours in childhood, which arise after birth, grow for several months and regress spontaneously. Serious problems can occur with closure of the eye resulting in amblyopia or with psychologica problems due to disfiguring of the face. Therapeutic possibilities are cryo therapy, laser therapy and surgical removal. Cryotherapy can be used for small and flat hemangiomas. Bigger hemangiomas are treated with the Nd-YAG laser. Its effect is an acceleration of involution. Typical residues of hemangiomas must be secondarily corrected surgically. Indications for primary surgical therapy are hemangiomas of the eye lid and in the scalp.

[Systemic therapy of hemangioma]. / Systemische Therapie von Hämangiomen.

For life-threatening hemangiomas where surgical or laser techniques are not feasible, systemic therapy with cortisone or interferon represents the therapy of choice. With response rates of 80-90%, both drugs are equally effective. Both treatments, however, might be associated with severe side-effects. Among these the risk of interferon-induced neurotoxicity has to be given special mention. Thus, the indication for systemic therapy of hemangiomas has to be made carefully, weighing up risk and benefit.

Brain hemangiomas of infancy. Sonographic detection and follow-up.

Vascular birthmarks of the skin can be associated with hemangiomas or arteriovenous malformations of different organs. Computed tomography and magnetic resonance imaging (MRI) combined with angiography are the diagnostic tools used in children, adolescents, and adults to depict vascular malformation or bleeding. In infants, ultrasound examination through the anterior fontanelle is possible. This report describes 2 mature infants with brain hemangiomas primarily detected by real-time sonography: In both the brain lesions were associated with cutaneous and other vascular birthmarks. MRI was used to verify the brain hemangiomas. Subsequent follow-up evaluations during therapy were easily performed by ultrasonography.

[Isopropanol and acetone level in serum after preoperative surface disinfection with antiseptics containing isopropanol]. / Isopropanol- und Acetonspiegel im Serum nach präoperativer Flächendesinfektion mit isopropanolhaltigen Antiseptika.

After preoperative skin disinfection in pediatric surgery, serum levels of isopropanol up to 12.2 mg/l (MW 5.0 mg/l +/- 3.37, n = 26) were found. They result from a rapid and prolonged but uncharacteristic percutaneous resorption of the isopropanol-containing disinfectant. In about 50% of the cases, serum levels of acetone showed an increase up to 82 mg/l already before skin disinfection, presumably caused by preoperative starvation. After skin disinfection, raised acetone levels were found in 19 of 26 cases. As increased isopropanol and acetone levels are discussed as alcoholism markers, a falsification of congener analysis after skin disinfection, e.g. in cases of adult victims of accidents, has to be taken into consideration. Endogenous serum levels of methanol (0.87 mg/l +/- 0.49), ethanol (0.32 mg/l +/- 0.09), acetaldehyde (0.31 mg/l +/- 0.10) and others remained unaffected. Some uncharacteristic elevations of propanol-1 levels are caused by contaminated rubber caps.

[Experiences with completely implantable central venous catheter systems]. / Erfahrungen mit vollständig implantierbaren zentralvenösen Kathetersystemen.

Over a 30 month-period (1.7.85-7.1.88) 44 totally implantable catheter-systems were used in 41 patients under oncological treatment. Indication was in 40 cases pending, or already started chemotherapy; in one case the indication were multiple transfusions because of aplastic anemia. We used three similar systems of different manufacturers. Our experiences with implantation and care of these systems as well as minor complications are demonstrated.

[Intraperitoneal fetal small intestine transplantation in allogeneic rat strains]. / Intraperitoneale fetale Dünndarmtransplantation bei allogenen Rattenstämmen.

We demonstrated the possibility of a successful transplant of foetal rat small intestine into the peritoneal cavity of adult rats. Several combinations of rat strains with defined genetic differences were chosen. On the whole, in more than 66 percent of the performed transplantations a successful adequate growth of the transplants was noted. Oral application of Cyclosporin A (20 mg/kg/d) as immunosuppressive to prevent allograft rejection was necessary only in the combination across major genetic barriers. The preoperative insertion of a 7-0 non-absorbable thread into the lumen of the transplant helped to develop a tubular segment of small intestine which can be anastomosed to the intestinal continuity of the host in a second stage operation.

[Separation of an asymmetric xipho-omphalo-ischiopagus tripus]. / Trennung eines asymmetrischen Xipho-omphalo-ischiopagus tripus.

A case of asymmetrical thoraco-omphalo-ischiopagus tripus will be reported. Anal atresia, ruptured omphalocele with lesion of the small intestine made an artificial anus necessary. Recurrent adhesion ileus made three further operations necessary. After precise preliminary examinations we separated these twins at the age of 21/2 years. After 7 and 9 months both children had to be reoperated because of fistula of the small intestine. Twin R. died at the age of 3 years and 8 months in consequence of a candida sepsis. Twin P. now lives with his parents.

[Surgical separation of symmetrical conjoined ischiopagus twins]. / Zur Trennung eines symmetrischen Ischiopagenpaares.

This is a case report on symmetrical ischiopagus tetrapus. We made exact preoperative examinations of the topographic anatomical relationships before performing the separation at the age of ten months. Both twins survived the operation without complication. A second procedure to correct the misformed pelvices of both children was successfully performed. The twins have been released from the hospital. One child, who can walk, lives with his parents. The second child has a perinatal brain defect, and as such is cared for in a nursing home.

[Microsurgical autotransplantation of cryptorchism]. / Mikrochirurgische Autotransplantation eines Bauchhodens.

An intra-abdominally located testicle was brought into the scrotum as a free transplant in a 13 year-old boy by microsurgery as a second surgical intervention. This procedure is regarded as an alternative compared to the high ligature of the spermatic vessels (Fowler), a two staged-orchiopexy and/or eventually orchidectomy.

Mitochondrial cardiomyopathy with involvement of skeletal muscles.

In this report we describe an idiopathic hypertrophic cardiomyopathy in a 21 month old infant girl; who died shortly after a small surgical intervention for cardiovascular failure. Fine structural investigation disclosed an extreme increase of often abnormally structured and enlarged mitochondria and a great loss of myofibrils in the heart muscle cells. Furthermore, mitochondrial hyperplasia was observed focally in all skeletal muscles investigated. The pathogenesis of this mitochondriopathy in heart and skeletal muscle is unknown. It might be a consequence of a functional mitochondrial defect with compensatory hyperplasia of mitochondria. Differential diagnosis of this very rare infantile cardiomyopathy from the myopathies of storage diseases, typical hypertrophic cardiomyopathy and carnitine defiency is discussed, as is the distinction from the oncocytic or so-called histiocytic transformation of heart muscle cells. Methological hints for diagnostic procedures are given.

[Mitochondrial cardiomyopathy with a high degree of heart muscle hypertrophy]. / Mitochondriale Cardiomyopathie mit hochgradiger Herzmuskelhypertrophie.

Because of cardiomyopathy with concomitant cardiac hypertrophy, a 21 month-old girl died 7 days following operative closure of a cleft palate. The cardiomyopathy was due to an excessive increase of enlarged and abnormally structured mitochondria. Similar mitochondrial change were found in the skeletal muscles (Mitochondrial Myopathy).