Mermaid syndrome associated with VACTERL-H syndrome.

Mermaid syndrome or sirenomelia is an extremely rare congenital malformation with an incidence between 1.5 and 4.2 per 1,000,000 pregnancies. Association of mermaid syndrome with VACTERL-H syndrome is extremely rare, with only two cases reported so far in the literature. We present a new case of type I sirenomelia associated with VACTERL-H syndrome and review the relevant literature. A 15-year-old female patient was admitted to the Department of Pathological Pregnancy at St George University Hospital, Plovdiv with progredient abortion during her first pregnancy. She had low socioeconomic status, negative history of concomitant diseases and addictions. The patient avoided prophylactic intake of folic acid during her pregnancy. Prenatal ultrasound found a malformative fetus. Consequently, magnetic resonance imaging was performed which established the presence of hydrocephalus and defects in the lower part of the spine. These pathological findings indicated interruption of pregnancy at 20 weeks of gestation. The fetopathological examination found sirenomelia type I associated with myelomeningocele, hydrocephalus, anal imperforation, single umbilical artery, bilateral renal and ureteric agenesis, bladder agenesis, tracheo-esophageal fistula, agenesis of external genitals, monkey fold of the left palm of the hand, also known as VACTERL-H syndrome.Our case demonstrates that mermaid syndrome and VACTERL-H syndrome represent different manifestations of a single pathological process that results in disorders of the blastogenesis at different stages during embryonic development.

Operative treatment of postpulmonectomy empyema.

OBJECTIVE: To analyse retrospectively 10 patients with postpulmonectomy empyema. METHODS: Ten patients (9 men, 1 woman, aged 48 to 69 years) with postpulmonectomy empyema treated over a 12 year period (1991--2002) were analysed. Nine of the patients had pulmonectomy for lung carcinoma and one--for pulmonary abscess. All postpulmonectomy empyemas were managed in two stages--an open-window thoracostomy for curing the infective process in the empyema cavity and then closure of the bronchopleural fistulas and obliteration of the cavity. Video-assisted thoracoscopy (VATS) was successfully used by the authors for sterilization and debridement of the cavity, and finally to insert two thoracic drains for the cavity lavage. The obliteration of the empyema cavity was achieved by a vascular muscle flap transposition from the thoracic muscles. The latissimus dorsi muscle flap was used in 6 patients. The pectoralis major muscle flap was used in 4 patients. Additionally, a thoracoplasty was performed in the patients with pectoralis major muscle flap transposition. RESULTS: Eight patients (80%) were discharged from hospital without complications, and two patients died (20%). CONCLUSIONS: On the basis of the study results and the literature review the authors conclude that the applied therapy yields good results in the treatment of postpulmonectomy empyema.

Perforation of the esophagus.

For the period 1993 through 2001 a retrospective clinical analysis of 22 patients with perforation of the esophagus was conducted. All patients received treatment at the Clinic of Thoracic and Abdominal Surgery, St. George University Hospital, Plovdiv. Perforation of the esophagus had several causes: instrumentation (upper endoscopy and dilatation) in 12 patients (54.5%), foreign bodies in 5 patients (22.78%) and trauma in 3 (13.64%). Spontaneous rupture was observed in 2 cases (9.10%). Surgical treatment was used in 20 patients (90.90%), 2 patients (9.10%) received conservative treatment. In 12 patients surgery was performed within 48 hours following esophageal rupture, 8 patients received surgical treatment after 48 hours. Surgical procedure included: esophageal repair using soft tissue (pleural or diaphragm) vascular flaps. Of all patients 9 fatalities were registered--3 patients died within the first 48 hours following surgery, 5 died after 48 hours. Death occurred in one case among conservatively treated patients. On the basis of the accumulated evidence it is concluded that early diagnosis, prompt surgical treatment and prevention of postoperative complications result in improved treatment outcome.