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Biomedical subjects

E H Strübbe

Publications and source records attributed to E H Strübbe.

8 recordsLinked to original sources
PubMed1995

Stapedial ankylosis in the Mayer-Rokitansky-Küster-Hauser syndrome.

We describe a 21-year-old woman with congenital unilateral conductive hearing loss and an atypical form of the Mayer-Rokitansky-Küster-Hauser syndrome. To our knowledge, this is the first reported case of this syndrome in which surgery for congenital stapedial ankylosis was successful. Besides aplasia of the vagina and uterus, the patient also had various other anomalies, such as the Klippel-Feil syndrome, Sprengel's deformity, and congenital stapedial ankylosis. Congenital hearing loss is an associated characteristic of the Mayer-Rokitansky-Küster-Hauser syndrome (10% to 20% of cases), particularly in the atypical form. Against the background of the favorable results of surgery for isolated unilateral congenital stapedial ankylosis and other unilateral congenital anomalies of the middle ear that have been described in the literature and the significant advantages of bilateral hearing, we used stapedectomy to successfully treat this case of Mayer-Rokitansky-Küster-Hauser syndrome with unilateral congenital stapedial ankylosis.

Abnormalities, Multiple↗
PubMed1994

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome without and with associated features: two separate entities?

A multidisciplinary study was conducted on a total of 100 women with congenital absence of vagina and uterus, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. It was possible to analyse whether the MRKH syndrome can be considered as a single clinical entity or whether two or more syndromes lie behind the title 'the MRKH syndrome'. Complete gynaecological and laparoscopic data were available on all of the patients. The patients were divided into two groups on the basis of the laparoscopic data: a typical and an atypical form of the MRKH syndrome. We performed various diagnostic investigations to establish whether there were any associated congenital anomalies. These tests included general physical examination, radiographs of the vertebral column, the upper extremities and intravenous urography (IVU), and general otorhinolaryngological and ossicular chain examinations. Associated anomalies were most common in the group with the atypical form of the MRKH syndrome. These findings suggest that there might be two different syndromes in this patient group, namely an isolated form of congenital agenesis of the vagina and uterus and a more generalized condition, in which agenesis of the vagina and uterus is a major and perhaps even obligatory characteristic. The term MRKH syndrome should no longer be used for the atypical group. A suggestion has been made to call this type the GRES [genital (G), renal (R), ear (E), skeletal (S)] syndrome.

Abnormalities, Multiple↗
PubMed1994

Hearing loss and the Mayer-Rokitansky-Küster-Hauser syndrome.

The hearing of 51 female patients with the Mayer-Rokitansky-Küster-Hauser syndrome was examined using otoscopy and standard audiometry. A unilateral or bilateral hearing loss of more than 15 dB Fletcher index was found in 13 of 51 (25.5%). Four of these 13 patients had a hearing loss of less than 20 dB in the worst ear. The remainder had a hearing loss of at least 30 dB in the worst ear. Five of the 13 patients had pure conductive hearing loss; in four of these five, a congenital origin was accepted. Two of the 13 had mixed hearing loss that was a residual symptom from previous otitis media; six had sensorineural hearing loss. A congenital cause was found in one of these six, based on the fact that she had been deaf and dumb since birth. In one other patient, noise-related deafness was likely (i.e., an acquired cause). In the other four cases in this group, the cause was unknown. The results of this study show that hearing loss is a characteristic associated with the Mayer-Rokitansky-Küster-Hauser syndrome.

Adult↗
PubMed1993

Mayer-Rokitansky-Küster-Hauser syndrome: distinction between two forms based on excretory urographic, sonographic, and laparoscopic findings.

OBJECTIVE: The purpose of this study was to discriminate typical (type A) from atypical (type B) Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (congenital absence of vagina and uterus) and determine their association with renal anomalies and ovarian disease. MATERIALS AND METHODS: The excretory urographic, sonographic, and laparoscopic findings in 91 patients with MRKH syndrome were compared retrospectively. Symmetric muscular buds and fallopian tubes were diagnostic of type A, and asymmetric muscular buds or abnormally developed fallopian tubes were diagnostic of type B. RESULTS: On the basis of laparoscopic findings, type A was diagnosed in 40 patients (44%) and type B was diagnosed in 51 patients (56%). Renal anomalies were found in 34 (37%) of the 91 patients, all of whom had type B syndrome. Renal agenesis and a pelvic kidney were the most common findings in the upper part of the urinary tract. Ovarian abnormalities were seen in 14 patients (15%), all of whom had type B syndrome. Sonography did not allow discrimination between types A and B in patients with normal kidneys (17/51 = 33%), but it provided important information in patients with associated cyclic abdominal pain, in cases of diagnostic dilemma, and in patients with associated renal anomalies. CONCLUSION: Discrimination between type A and type B of MRKH syndrome is important because associated renal and ovarian abnormalities occur only in type B. Laparoscopy is still needed to discriminate between these two forms. Sonography is useful for diagnosing cyclic abdominal pain and associated renal anomalies.

Abnormalities, Multiple↗
PubMed1992

Spinal abnormalities and the atypical form of the Mayer-Rokitansky-Küster-Hauser syndrome.

In 96 patients with congenital absence of the uterus and upper vagina, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, it proved possible to distinguish between the typical and the atypical form using laparoscopy. The typical form was characterized by symmetrical nonfunctioning muscular buds (the Müllerian duct remnants) and normal fallopian tubes, and the atypical form by aplasia of one or both buds, one bud smaller than the contralateral one, with or without dysplasia of one or both fallopian tubes. The atypical form was found in 52 patients (54.2%). Radiographs of the spine showed that congenital spinal abnormalities, especially the Klippel-Feil (KF) syndrome, were seen in 14 of the 52 patients with the atypical form only. Renal agenesis or ectopia together with the MRKH and KF syndromes, known as the MURCS association (MU: Müllerian duct aplasia; R: renal agenesis/ectopia; CS: cervical somite dysplasia), was diagnosed in 10/52 patients in the atypical group. From our results we conclude that additional cervical spine films in patients with the MRKH syndrome are indicated only in the atypical form the syndrome. In those cases where the MRKH syndrome is associated with the KF syndrome, the MURCS association should be considered.

Adolescent↗
PubMed1987

Evaluation of radiographic abnormalities of the hand in patients with the Mayer-Rokitansky-Küster-Hauser syndrome.

Radiographs of the hand in a group of 40 patients with the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome were studied. Most of the patients had the following abnormalities: brachymesophalangy of digits 2-5 (22/39 patients), small distal phalanx of digit 1 (22/39 patients), long proximal phalanx of digits 3-4 (19/39 patients), and long metacarpals of digits 1-4 (20/39 patients). In addition, three patients had distinct radial dysplasia and abnormalities of the carpals.

Abnormalities, Multiple↗
PubMedDate not supplied

Degenerative joint disease in female ballet dancers.

The relationship between long-term ballet dancing and eventual arthrosis of the hip, ankle, subtalar, and first metatarsophalangeal joint was examined in 19 former professional female dancers, aged 50 to 70 years. The dancers were compared with pair-matched controls. All 38 women underwent medical history taking, clinical examination, and roentgenography of the joints studied. The roentgenographs were independently judged by two investigators and grouped according to a modified classification of Hermodsson. We found a statistically significant increase in roentgenologic arthrosis of the ankle, subtalar, and first metatarsophalangeal joints in the ballet group compared with the control group. There was no significant difference regarding degenerative changes of the hip joint. However, subjects in the dance group who had evidence of degenerative changes on roentgenographs had no clinical complaints. There was a statistically significant increase in hallux valgus deformity in the ballet group (P < 0.05). The dancers also showed a statistically significant increase in flexion, external rotation, and abduction of the hip joint, dorsal flexion of the first metatarsophalangeal joint, and inversion and eversion of subtalar joint. But the control group had statistically significant increased plantar flexion of the first metatarsophalangeal joint. The most important cause of the statistically significant increase of arthrosis of the ankle and first metatarsophalangeal joints must be explained by repetitive microtrauma.

Aged↗