Ultraschall Med 2005; 26 - OP220
DOI: 10.1055/s-2005-917428

ROLE OF ULTRASOUND DIAGNOSIS IN CLINICAL CASES OF HEREDITARY ANGIONEUROTIC OEDEMA (HANO)

G Harmat 1, K Fáy 2, B Visy 2, L Varga 2, H Farkas 2
  • 1Ultrasound and Intervention, The Madarász Str. Children's Hospital, Budapest
  • 2Dep. Radiology and Allergology and Angioedema Outpatient Clinic, Kúvölgyi Clinical Center, Semmelweis University, Budapest, Hungary

Purpose: Ascites can result from diverse causes, most often in the abdomen and the pelvic region. However, hereditary angioneurotic oedema (HANO) is seldom mentioned as a cause of ascites. Angioneurotic oedema can be fatal, if the oedema is located in the larynx, but acut abdominal attacks can also be a real diagnostic problem.The exact diagnosis for proving theillness is the use of specific laboratory methods, as the disease is related to C1 inhibitor deficieny, but the most potent and suitable tool for helping its differential diagnosis is ultrasound.

Methods and Materials: In Hungary, a HANO center was set up in 1987, and since then, numerous occurrences of this illness have been diagnosed by laboratory methods. During each abdominal attack, ultrasound is successfully used. The apparatus applied is a Hitachi 451, Hitachi EUB 41 or Aloka SSD-1700 system with transducers of 3.5 or 5 MHz frequency, or a linear 7.5 MHz transducer, depending on the organ to be examined. The presence of free peritoneal or retroperitoneal fluid can be detected.

Results: After the diagnosis, patients were treated successfully by administering C1-INH concentrate. 24 hours after this treatment, repeated examination of the organ showed that the volume of the oedema decreased significantly, and in 48 hours it fully disappeared.

Conclusions: Since HANO is an uncommon illness, its differential diagnosis is very important in its early stage. In case of a laryngheal oedema, the patient can suffocate. Even now, the mortality rate of acute oedematous attacks makes 25-30%. Thus, if a patient shows skin symptoms, or acute pain, nausea, vomiting or profuse diarrhea of unrecognized reason, he/she should be immediately hospitalized and diagnosed. Early recognition of the acute abdominal attack is of utmost importance, because incorrect or delayed diagnosis often leads to unnecessery surgical intervention. During the correct treatment we can follow the regression of the free peritoneal fluid. In undiagnosed patients, ultrasound examination can be a differential diagnostic means for recognizing HANO in abdominal organs, and conversely, a search for HANO is warranted when the typical sonographic feature is ascertained in a patient with abdominal symptoms.