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.

Fetal adrenal transplantation: success of a laparoscopic technique in rats.

BACKGROUND/PURPOSE: The present study investigates a new laparoscopic technique for fetal adrenal transplantation in rats. RESULTS: The procedure was successful in 9 of 10 cases (one hole in the omentum) with no postoperative complications. On examination 4 weeks postoperatively, all but one graft showed macroscopic integrity, vascular supply, and histological maturation to normal zonal differentiation. When bilateral adrenalectomy was performed in the recipient to assess endocrine competence of the fetal adrenal grafts, survival was prolonged and Addison crisis was prevented in the animals that underwent transplantation. Levels of aldosterone dropped within the first week after adrenalectomy, but recovered steadily. Analysis of corticosterone demonstrated that levels fell to 25% of sham operated rats in the first week, but then steadily climbed to 70%. CONCLUSIONS: To the authors' knowledge this report presents the first study for laparoscopic transplantation of fetal tissues. Laparoscopic transplantation of fetal adrenal glands seemed feasible and successful in rats. The fetal adrenal transplants matured and served for a prolonged survival.

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.

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.

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.

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.

Indication for using extracorporeal membrane oxygenation in congenital diaphragmatic hernias and pulmonary hypoplasia.

Despite the apparent surgical simplicity of the anatomic defect, congenital diaphragmatic hernia continues to be a critical problem in neonatal surgery, so that survival is still uncertain. Therefore, we must realize that the barriers to survival are pulmonary parenchymal and vascular hypoplasia as well as the complex syndrome of persisting fetal circulation. However, new treatment methods, such as extracorporeal membrane oxygenation (ECMO), although controversial, may improve survival. We believe that no infant should be excluded from diaphragmatic repair or consideration for ECMO-support before accurate predictive parameters have been developed that take both pulmonary hypoplasia and pulmonary hypertension into account. ECMO additionally enables us to postpone the operation until stabilization of the newborn (Late Operation Protocol). Apart from this, we can probably improve the long-term results after ECMO by reconstructing the common carotid artery.

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.

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.

[Surgery of atresia of the gastrointestinal tract]. / Chirurgie der Atresien des Gastrointestinaltraktes.

The most common sites of atresia in the gastrointestinal tract are the oesophagus, the duodenum and the anorectum. Surgical intervention is always necessary. Because of the rapid progress in surgical techniques and peri- and postoperative intensive care management, lethality has been minimized, even when treating very small premature infants. In the meantime, the surgical intervention itself is no longer the great risk it was; associated congenital malformations such as complex cardial defects are now much greater risks.

[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.

[Operative pediatric gynecology. Pediatric surgical aspects in congenital and acquired changes in the female genitals]. / Operative Kindergynäkologie. Kinderchirurgische Gesichtspunkte bei angeborenen und erworbenen Veränderungen des weiblichen Genitale.

Surgical problems of congenital malformations of the female genital tract, especially in combination with anorectal or urological anomalies, the correction of clitoral hypertrophy due to endocrinological disorders (AGS) and inflammatory and tumorous alterations of the ovary in childhood are discussed.

[Acute abdominal pain in childhood]. / Akuter Bauchschmerz im Kindesalter.

According to the difficulties in communication with babies and very young children there often are problems in the early diagnosis of abdominal pain or symptoms. This is demonstrated in the example of appendicitis in children and meconium peritonitis in the newborns. The possibilities of pain measuring in newborn or preterm babies are shortly discussed.

[Guidelines in pediatric surgery]. / Leitlinien in der Kinderchirurgie.

Guidelines are intended to give more structure and transparency to medical procedures and to reduce unnecessary medical costs. The aims of guidelines and the claims made upon them are defined and their usefulness is discussed, with a short review of special problems in pediatric surgery.