Iniencephaly and long-term survival: a rare case report

Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence. This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.     

Partially de-epithelialised and buried V-Y advancement flap for reconstruction of sacrococcygeal and ischial defects.

Defects in the sacrococcygeal and ischial soft tissues can be treated with gluteus maximus and posterior thigh V-Y advancement flaps. However, late complications include recurrence and dehiscence of the suture line. Increasing the amount of the soft tissues over the bony prominences and multilayered closure may have an advantage for long-term durability. We modified the V-Y advancement technique by de-epithelialising the medial parts of the flap and burying them under the opposing edge of the wound or the flap. Sixteen patients with various defects of the sacrococcygeal and ischial soft tissues were operated on using this technique. All the flaps healed well with no partial or complete loss of the flap. Three patients developed complications. The main advantage of our technique is the use of healthy tissues to obliterate the dead spaces under the edges of the wound or the opposing flap. In this way, not only the defect in the skin but the defect in the subcutaneous tissue, with its iceberg tip at the surface, is treated effectively. To have an additional layer of tissue between the bone and the superficial tissues provides an extra cushion of soft tissue and avoids putting the suture line directly over the bony prominences. We used this modification safely for both unilateral and bilateral flaps. It could also be used successfully in other parts of the body.     

Reversed anterior interosseous flap.

Reversed flaps from the forearm have been firmly established for hand coverage. Each has its own advantages and disadvantages. The reversed anterior interosseous flap is one option with special advantages in hand reconstruction. From January 2002 to July 2003 we used this flap in five consecutive male patients aged between 36 and 59. The defects were located on the first web space (2), on the dorsal side of the thumb (1), on the first metacarpal bone (1), and on the volar side of the wrist (1). Flap size was between 6 x 3 cm and 11 x 7 cm. All flaps healed without any problem and no complication was observed during the postoperative period. The major advantage of this flap is the preservation of the main arteries of the upper limb. Other advantages are as follows: good texture and colour for hand reconstruction, satisfactory rotation arc, and availability of composite and fascial flaps. Besides its advantages, the major disadvantages are unsightly donor area scar and the need for meticulous technique. As a conclusion, we found this flap very useful in hand reconstruction for coverage of small and moderate sized soft tissue defects of the hand.     

A case of juvenile chronic myeloid leukemia with XX/XXX mosaicism.

Cytogenetic abnormalities are rarely found in patients with juvenile chronic myelogenous leukemia (JCML). In patients with chromosomal abnormalities, chromosomes 7 and 8 are usually involved. A case of JCML with 47 XXX and a 46 XX karyotype is described and the literature is reviewed. To our knowledge, this is the first case ever to have been reported.     

Cerebellar abscess and syringomyelia due to isoniazid-resistant Mycobacterium tuberculosis.

A 19-year-old immunocompetent man was admitted to hospital with diplopia, nausea, vomiting and change in mental status. The patient had a history of tuberculous meningitis that was diagnosed at another hospital 6 months before the present admission, and at that time anti-tuberculosis treatment was initiated using a first-line drug combination. A computed tomography (CT) scan of the brain revealed non-communicating hydrocephalus. A ventriculo-peritoneal shunt was inserted surgically. Two months later, the patient was hospitalized again for fever, dysphagia and left hemiparesis. At that time, his cranial CT findings were within normal limits; however, magnetic resonance imaging (MRI) revealed an irregular multilocular peripheral contrast-enhancing lesion in the posterior fossa. The abscess was surgically drained. The presence of acid-fast bacilli in the abscess material was demonstrated by Ziehl-Neelsen staining. Mycobacterium tuberculosis grew on Lowenstein-Jensen culture medium, and the strain was found to be resistant to isoniazid. One month after the operation, the patient became quadriparetic. Cervical MRI revealed a cervico-thoracic syringomyelitic cavity, after which a syringoperitoneal shunt was placed. Treatment with four drugs was continued for 10 months, and then treatment with three drugs for a total period of 18 months. The patient recovered, with residual quadriparesis. Even though very rare, isoniazid-resistant M. tuberculosis may be the causative agent of progressive tuberculosis.     

A reverse-flow composite flap in the rat.

Reverse-flow flaps are currently particularly used for the reconstruction of defects of the distal part of the extremities. Despite their common usage there have been many reports of postoperative complications, especially resulting in partial or total flap necrosis. There is insufficient knowledge of flap haemodynamics, physiology and wound healing properties in reverse-flow flaps. Development of the proper experimental models is needed to investigate these issues. The purpose of this study was to describe a new reverse-flow flap model in the rat. A total of 20 adult Wistar rats weighing 200-250 g were used in this experiment. In five rats, the vascular anatomy of the auricle of the rat was determined by anatomic dissection and microangiography. In the experimental group (N=5), 1x1 cm reverse-flow composite flaps were harvested as a semi-island shape, based on the distal course of the medial branch of the anterior auricular artery. In the control group, consisting of five rats, the flap was designed and raised based on the proximal course of the medial auricular artery, again in a semi-island shape. In the remaining five animals, a square-shaped composite tissue of the whole layer of the auricle, 1x1 cm in size, was harvested dividing all the bases circumferentially. The composite tissue was replaced in situ. While the former was considered a conventional antegrade-flow flap subgroup, the latter was designated as a graft subgroup. All flaps were replaced in situ. The survival of the flap was evaluated on postoperative day 7 by direct observation and microangiography. The skin island of all the reverse-flow flaps and conventional antegrade-flow flaps survived completely giving a success rate of 100%, whereas all grafts in the control group underwent complete necrosis. Microangiographic studies revealed the vascularity of the reverse-flow and antegrade-flow flaps, identifying the course of the auricular arteries. In conclusion, with its evident advantages of easy to design and harvesting, reliable survival pattern and consistent vascular structure, our new flap model will provide a means for future studies on flap haemodynamics, physiology in reverse-flow flaps.     

Evaluation of flow hemodynamics by color-Doppler following two different brachial arterial repair techniques.

OBJECTIVES: We compared the clinical and hemodynamic results following surgical repair of traumatic brachial artery injury using two different techniques micro- and macrovascular repair. MATERIALS AND METHODS: This was a retrospective study of 27 patients who had sustained penetrating, clean cut injuries of the brachial artery. Macrovascular techniques and a saphenous vein graft was used in 13 patients, while 14 patients were treated by primary microsurgical technique. Postoperatively, patients were followed for a mean of 26 months. All patients had color Doppler examination of the brachial artery, digital artery pressures and transcutaneous oxygen saturation determined. RESULTS: Clinical results based on distal pulses, Allens test and digital pressures were similar in the two groups. Color Doppler showed 8/13 anastomotic stenoses in macrovascular vein grafted repairs and 2/14 in microvascular repairs (p<0.05). The ratio of flow velocity proximal compared distal to the injury was significantly decreased in patients who had macrovascular repairs. CONCLUSION: Using ratio between proximal and distal site of anastomosis maximal peak systolic velocity as a objective color Doppler parameter, we were able to demonstrate differences in the hemodynamic status following macrovascular repair with vein grafts and microvascular primary repair. The results emphasize the importance of using a standard repair technique for similar injuries rather than the preference of the surgeon.     

Brucella peritonitis and leucocytoclastic vasculitis due to Brucella melitensis.

Brucellosis is a multisystemic disease that rarely leads to a fatal outcome. While reticuloendothelial system organs are mostly affected, peritonitis and posthepatitic cirrhosis are also complications of brucellosis, though they are very rare. Brucella spp. can also trigger immunological reactions. We report a case of brucellosis with peritonitis, renal failure and leucocytoclastic vasculitis caused by Brucella melitensis, which led to a fatal outcome. Brucellosis should be considered in the differential diagnosis of vasculitic diseases, especially in endemic areas.     

Immunosuppression by a peptide from the gelatin binding domain of human fibronectin

Circulating immunosuppressive peptides are found in conjunction with elevated protease activity in injured patients' serum. Previous work suggests that fibronectin may be a source of these peptides. Elastase-generated fibronectin degradation products were chromatographically separated and assessed for immunosuppressive capacity in the neutrophil chemotaxis and mixed lymphocyte reaction bioassays. The suppressive fibronectin degradation products fraction 22 inhibited chemotaxis by 53% (P less than 0.01) and mixed lymphocyte reaction by 41% (P less than 0.001). Incubation of the suppressive fraction 22 with gelatin, neuraminidase, or anti-SAP (suppressor active peptide) antibody reverses the chemotaxis inhibition to control values. These results indicate that an elastase-generated fragment of fibronectin, which potently inhibits neutrophil and lymphocyte activity, is located within the sialated gelatin binding portion of the molecule. Immunosuppression reversal by anti-SAP antibody, and the requirement for sialic acid, suggests similarity between fraction 22 and SAP, although other explanations are plausible. This peptide fragment from fibronectin may impact on the clinical immunosuppression seen in patients following severe trauma.     

A mature cystic teratoma in pineal region mimicking parietal encephalocele in a newborn

Objective Teratoma is the most frequently encountered intracranial tumor at birth and constitutes 18–20% of all germ cell tumors. They are usually located in pineal and suprasellar regions. The authors aim to report an extremely unusual presentation, location, and appearance of a teratoma in a newborn. Case report A soft tissue swelling in the vertex was detected in a 1-month-old girl. Neurological examination was normal. A big, cystic–solid lesion beginning from pineal region and extending to the scalp was detected in magnetic resonance imaging. It is interesting to note that cerebral venous angiography showed that the superior sagittal sinus (SSS) was divided into three branches at the level of the lesion and they joined together distally. The tumor was excised totally. Histopathological examination revealed the diagnosis of a mature cystic teratoma. Conclusion A mature cystic teratoma mimicking parietal encephalocele is extremely rare. Germ cell tumors should be kept in mind in the differential diagnosis of all midline lesions with unusual radiographic appearance. Cerebral venous angiography or MR angiography must be performed for the diagnosis and the surgical planning in lesions located near SSS.