British Journal of Obstetrics and Gynaecology August 1992, Vol. 99, pp. 689-696
C A S E REPORTS
Atypical female intersex P. C. S E N G U P T A Projessor & Head Department of Obstetrics & Gynaecology Ramakrishna Mission Seva Pratishthan Calcutta 700 026, India
Case report The patient described in this report was an adult married woman who first attended the Ramakrishna Mission Seva Pratishthan Hospital, Calcutta, India on 25 March, 1988. She was 23 years of age and had been married for 5 years. Micturition and menstruation occurred through the anus, as did sexual intercourse. Urine also dribbled occasionally through a phallic urethra. She was continent of faeces. She was a phenotypic female with well-developed secondary sex characters.
The labia majora were normal but the pudendal cleft was obliterated, the perineal body absent and there were marked clitoromegaly with a meatal opening; part of the labia minora projected into the anorectal wall with concomitant shifting of the urethra and vagina into the rectum. The phallus was more anterior and more cephalad than normal and lacked the caudal curvature usual in women (Figs. 1 & 2). Compression of the distended shaft of the phallus caused small quantities of urine to spurt out. There was no history of in utero exposure to androgenic agents. The haematological and endocrine investigations and excretory pyelogram were normal. A buccal smear was chromatin positive and repeated karyotypes were 46 XX. Ultrasonography and laparoscopy showed that she had a bicornuate uterus and tuba1 blockage at the fimbrial ends. Examination under anaesthesia, sigmoidoscopy and vaginoscopy revealed the vagina and, caudal to it the urethra, opening into the
Correspondence:P. C. Sen Gupta, ‘Jharna’, 21 Syed Amir Ali Avenue, Calcutta 700 017, India.
Fig. 1. 46 XX, phenotype female; well developed secondary sex characters and a large phallus.
Fig 2. External genitalia with well developed labia majora, fused pudendal cleft, retro-placed labial vestibule encroaching the anal canal, and the large phallus with meatal opening on the glans.
689
690
CASE REPORTS
anterior rectal wall about 5 cm cephalic to the anus; the cervix and sagittal septum in the upper third of the vagina could be palpated through the anus (Fig. 3). A fine polythene tube was introduced with difficulty through the phallic meatus and a cystoscope through the rectal urethra. Cystoscopy showed normal ureteric orifices and two separate urethral openings; retrograde catheterization detected no anomalies. Contrast injected through the phallus and rectal urethra on separate occasions confirmed phallic megalourethra with outlet obstruction. At abdomino-perineal reconstructive surgery on 24 June, 1988, the bicornuate abnormality of the uterus was confirmed and there were bilateral peritubal adhesions with fimbrial occlusion; biopsies of the gonads showed normal ovarian histology. The vagina, which was adherent to the anterior rectal wall, was separated around the rectovaginal communication. The dissection was extended in the para-rectal space down to the pelvic floor and the puborectalis muscle and the anorectual ring were identified following the principle of Goligher’s operation (Goligher 1958, 1975) for rectal prolapse (Fig. 4). A perineal procedure followed: liberal para-anal incisions opened the para-rectal space: the pubococcygeus and the puborectalis muscles were identified. The vaginal and urethral openings were located through the stretched anal orifice, separated and dissected by incising the rectal mucosa around the upper (posterior) lip of the vagina. The urethra, vagina and the associated vestibule, and labia minora were displaced enmasse Corwards and downwards and
anchored in near to the normal position by opening the pudendal cleft. The rectal mucosa was repaired longitudinally and the anus reconstituted with tightening of the sphincters. The puborectalis muscles were sutured together in front of the rectum, and the pubococcygeus and laterally displaced perineal muscles were used to construct the perineal body (Fig. 5). The phallus was excised and the accessory urethra ablated (Figs. 6, 7). The post-operative recovery was uneventful. She achieved normal menstruation and micturition through the re-directed genitourinary passages. A hysteroplasty and bilateral tuboplasty were performed at the second laparotomy on 17 September, 1988. She conceived 15 months after the last operation and remained in hospital throughout her gestation. A healthy female baby (birthweight 2.5 kg) was born by elective caesarean section on 25 July, 1990. The genital organs, ovaries and the bladder were assessed again during the casarean section; a weak area in the ventral wall of the bladder where the phallic urethra opened was incised and repaired. A 4 cm urethral length was confirmed later by a micturating cystogram and cystourethrogram (Fig. 8). The excised phallus measured 5x2 cm and had a median skin raphe on the caudal surface; the glans was well developed and had a meatal opening: cross-sections showed a single fibro-elastic cylinder traversed by the megalourethra.
Discussion Idiopathic female intersex (Park e t a / . 1972) involving a spectrum of urogenital anomaly, with or without cloaca1 malformation, is rare (Johnson et a/.1972; Klugo et a / . 1974; Kounami et al. 1986; Turnock & Rickwood 1987). Most are diagnosed at birth or during infancy. Park et al. (1 972), further to the observations of Blum (1904), classified female intersex into five groups: (i) Congenital adrenal hyperplasia; (ii) maternal androgenic influence, either exogenous or endogenous; (iii) idiopathic masculinization without somatic anomalies; (iv) special group with serious somatic anomalies and (v) familial group. The present patient is unusual and belongs to group (iii). A
Fig. 3. Schematic diagram of multiple genito-urinary defects before operation. Abbreviations: BU = Bicornuate uterus; M Ur = Megalourethra (phallic); P = Phallus; R = Rectum; TB = Tuba1 blockage (bilateral); Ur (E) = Ectopic urethra; UB = Urinary bladder; VS = Vaginal septum.
Fig. 4. Planning of abdomino-perineal operation. UB = Urinary bladder; R = Rectovaginal septum.
CASE REPORTS
Fig. 5. Schematic presentation of reconstructive surgery before excision of phallus. ( I ) Repair of anterior rectal wall at the site of ectopic opening of vagina and urethra; (2) Anterior approximation of puborectalis muscle; (3) Reconstruction of perineal body.
few similar cases have been reported by Grubenmann (1912), Howard & Hinman (I95 I), Bonney et al. (1975) and Thiry et al. (1979); the last two groups of authors claimed theirs to be the 4th and 5th instance in literature but Bellinger & Duckett (1982) and Kounami et al. (1986) recently analysed 19 cases including their own. The clinical features varied widely. The ectopic openings of the vagina and urethra inside the rectum, with accessory phallic urethra as in our patient, have not been described before. This has been confinned by many through personal communication (The Royal Society of Medicine, UK; Royal College of Obstetricians & Gynaecologists, UK; Prof S. S. Ratnam, Singapore; Prof John E. Skandalakis, Georgia, USA; Sir John Stallworthy, UK; Prof Peter L. Williams, UK; Prof Carl Wood, Australia).
Fig. 6. Correction of external genital malformations with ablation of phallic urethra and excision of the phallus.
691
The simple idiopathic group comprises a range of urogenital anomalies and degrees of phallic differentiation (Kounami et al. 1986). A functional double urethra has been recorded in a few cases (Grubenmann 1912; Jones & Scott 197 1; Bonney et al. 1975; Belis & Hrabovsky 1979; Hurwitz & Fitzpatrick 1982), but ‘Y’-type duplication is more frequent (Perloff et a/. 1953; Jones & Scott 1971; Bellinger & Duckett 1982). The phallic urethra was stenotic in most cases with a terminal stricture (Hurwitz & Fitzpatrick 1982). The rectal urethra functioned normally in our patient so that there was no obstructive uropath y. In the normal woman, the ventral corpus spongiosum does not differentiate, resulting in an absence of a phallic urethra and causing a ventral curvature. The clitoral prominence in the present case may be accounted for by phallic urethralization, as suggested by Bellinger & Duckett (1982), which prevents the development of a normal female ventral chordee. The terminal stricture of the phallic urethra, as observed by many (Bonney etal. 1975; Belis & Hrabovsky 1979; Hurwitz & Fitzpatrick 1982), is the result of separate development of this part from an ectodermal groove on the undersurface of the corona glandis (Bellinger & Duckett 1982; Marberger 1975). The megalourethra is the result of abnormal development of the phallus, but there is controversy about the fibro-elastic cylinders (Belis & Hrabovsky 1979; Huben et al. 1981; Kounami et al. 1986). Surgical treatment of the phallic urethra is not often
Fig. 7. A close view of the external genitalia, after reconstructive surgery.
692
C A S E REPORTS
cult to ascertain. Possibilities include a local disturbance of the inductive factor (Howard & Hinman, 1951), resulting from an incomplete union between the Miillerian ducts and the urogenital sinus; this might be responsible for the uninhibited development of an accessory phallic urethra. The urethralization of the female phallus is distinguishable from masculinization in adreno-genital syndrome by the presence of both phallic and vulva1 urethral orifices. Turnock & Rickwood (1987) suggested that this may be due to failure of differentiation of the peri-cloaca1 mesoderm, because of late arrival and incomplete fusion of the Miillerian ducts. Our patient had unique multiple atypical genito-urinary deformities and surgical correction was followed by a successful pregnancy. Such a case has not been described before.
Acknowledgments The author is grateful to staff, Department of Obstetrics & Gynaecology, R.K.M.S.P. Hospital for their assistance; to Dr Asim Kr Dutta, Professor of Anatomy, University College of Medicine, Calcutta for his guidance; the secretarial staff for their support and the Secretary of the hospital for kind permission to use the hospital data. He is thankful to the numerous scientists for personal communication and especially to the Librarians of the Royal College of Obstetricians & Gynaecologists and the Royal Society of Medicine, UK, for computer search and reprints.
References
Fig. 8. Post-operative cysto-urethrogram showing the 4 cm length of the urethra relocated from the rectum.
necessary as little urine usually passes through it (Bellinger & Duckett 1982). Diagnosis is usually at birth or in early childhood because of the ambiguous external genitalia, but there may also be obstructive uropathy, urinary tract infection and frequent dribbling of urine. Supra-pubic cystostomy and cutaneous nephrostomy have been necessary when there was severe urinary tract obstruction (Grubenmann 1912; Belis & Hrabovsky 1979). The treatment is usually directed towards relieving urinary obstruction and correcting the masculinized external genitalia. Clitoridectomy has now been replaced by clitoroplasty relocating normal caudal curvature to reduce the phallic prominence and to preserve normal clitoral sensation. The excess shaft skin has been used to reconstruct the labia minora (Bellinger & Duckett 1982). The internal genitalia rarely require surgical correction, but reduction vaginoplasty and vaginal septotomy (Hurwitz & Fitzpatrick 1982) are recorded. Laparotomy is not necessary for the assessment of gender (Bellinger & Duckett 1982). Uteroplasty and tuboplasty were essential in the present patient as such a major degree of uterine deformity might have not been compatible with term pregnancy. Clitoridectomy was obligatory as clitoroplasty was technically difficult because of the high anatomical location of the phallus. The embryological pathogenesis in the present case is diffi-
Belis J. A. & Hrabovsky E. E. (1979) Idiopathic female intersex with clitoromegaly and urethral duplication. J Urol 122, 805-808. Bellinger M. F. & Duckett J. W. (1982) Accessory phallic urethra in the female patient. J Urol 127, 1159-1164. Blum V. (1904) Die Hypospadie der weiblichen Harnrohre. Monatshericht Urology 9, 522. Bonney W. W., Young H. H., 11, Levin D. & Goodwin W. E. (1975) Complete duplication of the urethra with vaginal stenosis. J Urol 113, 132-137. Goligher J. C. (1958) The treatment of complete prolapse of the rectum by the Roscoe Graham operation. Brit J Surg 45, 343. Goligher J. C. (ed.) (1975) Surgery ofthe Anus, Rectum and Colon. 3rd ed., Baillikre Tindall, London, pp. 292-340. Grubenmann I. (19 12) Eine sagittale Verdoppelung der weiblichen Hamrohre. Frankjurt Ztschr Path 10, 101-128. HowardF. S. &HinmanF., Jr. (1951)Femalepseudohennaphroditism with supplementary phallic urethra: report of two cases. J Urol65, 439-452. Huben R. P., Devine P. C., Harkins G. A. & Stecker J. F., Jr. (1981) Clitoromegaly and megalourethra in idiopathic female intersex. J UI-01126,849-85 1. Hunvitz R. S. & Fitzpatrick T. J. (1982) Vaginal urethra, clitoral hypertrophy and accessory phallic urethra: a rare syndrome of female pseudohennaphroditism. J Urol 127, 1165-1168. Johnson R. J., Palken M., Derrick W. & Bill A. H. (1972) The embryology of high anorectal and associated genito-urinary anomalies in the female. Surg Gynecol Ohstet 135, 759-762. Jones H. W., Jr. & Scott W. W. (1971) Hermaphroditism, Genital Anomalies and Related Endocrine Disorders, 2nd ed., Williams & Wilkins Co., Baltimore, p.275. Klugo R. C., Fisher J. H. & Retik A. B. (1974) Management of urogenital anomalies in cloaca1 dysgenesis. J Urol 112, 832-835. Kounami T., Takeuchi H., Takayama H. & Tomoyoshi T. (1986)
C A S E REPORTS
Vaginal and phallic urethra with prominent clitoris in female pseudohermaphroditism.J Urol 136,915’)18. Marberger M. J., Jr. (1975) Accessory phallic urethra in female pseudohermaphrodite. Urology 5,505-507. Park I. J., Johanson A., Jones H. W. & Blizzard R. (1972) Special female hermaphrodite associated with multiple disorders. Obst
693
Thiry A. J., Wincqz P. J. & Schulman C. C. (1979) Female pseudohermaphroditism with supplementary phallic urethra. Eur Urol 5, 285-286.
Tumock R. R. & Rickwood A. M. M. (1987) Urethralisation of the female phallus: a rare form of intersex. Brit J Urol59(5),481-482.
Gynecol39, 100-105.
Perloff W. H., Conger K. B. & Levy L. M. (1953) Female pseudohermaphroditism: a description of two unusual cases. .I Clin Endocrinol 13. 783-790.
Received 11 September 1990 Resubmitted 5 November 1991 Accepted 19 January 1992
British Journal of Obstetrics and Gynaecology August 1992, Vol. 99, pp. 693-695
Intermittent abdominal decompression: an option for prevention of intrauterine growth retardation S. S H I M O N O V I T Z S. YAGEL D. Z A C U T A. B E N - C H E T R I T D. H O C H N E R C E L N I K I E R M. R O N Department of Obstetrics and Gynecology Hadassah University Hospital Mount Scopus, Jerusalem, Israel
Case reports Patient 1 A 26-year-old healthy woman was admitted in the 12th week of her sixth pregnancy because of her bad obstetric history. All her previous pregnancies had been complicated by hypertension and severe intrauterine growth retardation; the first three pregnancies were terminated between 26 and 32 weeks gestation because of intrauterine fetal death accompanied by moderate to severe hypertension. In her fourth pregnancy caesarean section was performed at 3 1 weeks gestation because of moderate hypertension, a small fetus, oligohydramnios and a positive oxytocin challenge test. The infant, who weighed 700 g at birth, died 4 days later from cardiorespiratory failure. The fifth pregnancy was terminated at 28 weeks gestation, again because of moderate hypertension and the death in-utero of a very small fetus. She was treated in this pregnancy by bed rest, hydralazine, methyldopa, low dose aspirin and prednisolone. No underlying cause was found for the recurrent hyperCorrespondence: S. Shimonovitz MD, Department of Obstetrics & Gynecology,Hadassah Mount Scopus, Jerusalem 91240 POB 24035, Israel.
tension, poor fetal growth and intrauterine death. On admission in her sixth pregnancy her blood pressure was 130/85 mmHg. She was treated with intermittent abdominal decompression using a Heyns’ suit (Heyns 1963) with negative pressure of around 70 mmHg for 30 s every minute for 30 min twice a day. In addition she was treated by methyldopa. Her blood pressure during the pregnancy did not exceed 120/85 mmHg. Fetal growth was monitored by repeated ultrasonographic examinations and was normal. Repeated systolic/ diastolic (S/D) ratios were calculated from the umbilical artery flow velocity waveforms obtained by continuous Doppler ultrasound, values ranged between 3.3 and 3.7. Umbilical artery flow velocity waveforms were not recorded during treatment with decompression because the ultrasound transducer could not be used within the sealed unit. At 38 weeks gestation a healthy 3280 g male infant was born by caesarean section.
Patient 2 A 27-year-old healthy woman was admitted in the 15th week of her fourth pregnancy because of her bad obstetric history. She developed moderate hypertension during her first pregnancy which ended in intrauterine fetal death at 28 weeks. In her second pregnancy labour was induced at 37 weeks because of moderate hypertension and a small fetus. A 1900 g infant was born vaginally. No underlying cause for the recurrent hypertension was found. In her third pregnancy she was treated by bed rest, low dose aspirin (100 mg/day) and dipyridamole (225 mg/day). At 32 weeks gestation intrauterine death occurred. In her fourth pregnancy treatment with intermittent abdominal decompression, as described for patient 1, was initiated at 15 weeks. Her blood pressure was normal throughout the pregnancy and no drug treatment was given. Repeated S/D ratios, calculated during the treatment period from umbilical artery flow velocity waveforms, were below 3.5. At 39 weeks gestation a normal 4100 g male was born spontaneously. In her fifth pregnancy she used the decompression suit at home
C A S E REPORTS
Atypical female intersex P. C. S E N G U P T A Projessor & Head Department of Obstetrics & Gynaecology Ramakrishna Mission Seva Pratishthan Calcutta 700 026, India
Case report The patient described in this report was an adult married woman who first attended the Ramakrishna Mission Seva Pratishthan Hospital, Calcutta, India on 25 March, 1988. She was 23 years of age and had been married for 5 years. Micturition and menstruation occurred through the anus, as did sexual intercourse. Urine also dribbled occasionally through a phallic urethra. She was continent of faeces. She was a phenotypic female with well-developed secondary sex characters.
The labia majora were normal but the pudendal cleft was obliterated, the perineal body absent and there were marked clitoromegaly with a meatal opening; part of the labia minora projected into the anorectal wall with concomitant shifting of the urethra and vagina into the rectum. The phallus was more anterior and more cephalad than normal and lacked the caudal curvature usual in women (Figs. 1 & 2). Compression of the distended shaft of the phallus caused small quantities of urine to spurt out. There was no history of in utero exposure to androgenic agents. The haematological and endocrine investigations and excretory pyelogram were normal. A buccal smear was chromatin positive and repeated karyotypes were 46 XX. Ultrasonography and laparoscopy showed that she had a bicornuate uterus and tuba1 blockage at the fimbrial ends. Examination under anaesthesia, sigmoidoscopy and vaginoscopy revealed the vagina and, caudal to it the urethra, opening into the
Correspondence:P. C. Sen Gupta, ‘Jharna’, 21 Syed Amir Ali Avenue, Calcutta 700 017, India.
Fig. 1. 46 XX, phenotype female; well developed secondary sex characters and a large phallus.
Fig 2. External genitalia with well developed labia majora, fused pudendal cleft, retro-placed labial vestibule encroaching the anal canal, and the large phallus with meatal opening on the glans.
689
690
CASE REPORTS
anterior rectal wall about 5 cm cephalic to the anus; the cervix and sagittal septum in the upper third of the vagina could be palpated through the anus (Fig. 3). A fine polythene tube was introduced with difficulty through the phallic meatus and a cystoscope through the rectal urethra. Cystoscopy showed normal ureteric orifices and two separate urethral openings; retrograde catheterization detected no anomalies. Contrast injected through the phallus and rectal urethra on separate occasions confirmed phallic megalourethra with outlet obstruction. At abdomino-perineal reconstructive surgery on 24 June, 1988, the bicornuate abnormality of the uterus was confirmed and there were bilateral peritubal adhesions with fimbrial occlusion; biopsies of the gonads showed normal ovarian histology. The vagina, which was adherent to the anterior rectal wall, was separated around the rectovaginal communication. The dissection was extended in the para-rectal space down to the pelvic floor and the puborectalis muscle and the anorectual ring were identified following the principle of Goligher’s operation (Goligher 1958, 1975) for rectal prolapse (Fig. 4). A perineal procedure followed: liberal para-anal incisions opened the para-rectal space: the pubococcygeus and the puborectalis muscles were identified. The vaginal and urethral openings were located through the stretched anal orifice, separated and dissected by incising the rectal mucosa around the upper (posterior) lip of the vagina. The urethra, vagina and the associated vestibule, and labia minora were displaced enmasse Corwards and downwards and
anchored in near to the normal position by opening the pudendal cleft. The rectal mucosa was repaired longitudinally and the anus reconstituted with tightening of the sphincters. The puborectalis muscles were sutured together in front of the rectum, and the pubococcygeus and laterally displaced perineal muscles were used to construct the perineal body (Fig. 5). The phallus was excised and the accessory urethra ablated (Figs. 6, 7). The post-operative recovery was uneventful. She achieved normal menstruation and micturition through the re-directed genitourinary passages. A hysteroplasty and bilateral tuboplasty were performed at the second laparotomy on 17 September, 1988. She conceived 15 months after the last operation and remained in hospital throughout her gestation. A healthy female baby (birthweight 2.5 kg) was born by elective caesarean section on 25 July, 1990. The genital organs, ovaries and the bladder were assessed again during the casarean section; a weak area in the ventral wall of the bladder where the phallic urethra opened was incised and repaired. A 4 cm urethral length was confirmed later by a micturating cystogram and cystourethrogram (Fig. 8). The excised phallus measured 5x2 cm and had a median skin raphe on the caudal surface; the glans was well developed and had a meatal opening: cross-sections showed a single fibro-elastic cylinder traversed by the megalourethra.
Discussion Idiopathic female intersex (Park e t a / . 1972) involving a spectrum of urogenital anomaly, with or without cloaca1 malformation, is rare (Johnson et a/.1972; Klugo et a / . 1974; Kounami et al. 1986; Turnock & Rickwood 1987). Most are diagnosed at birth or during infancy. Park et al. (1 972), further to the observations of Blum (1904), classified female intersex into five groups: (i) Congenital adrenal hyperplasia; (ii) maternal androgenic influence, either exogenous or endogenous; (iii) idiopathic masculinization without somatic anomalies; (iv) special group with serious somatic anomalies and (v) familial group. The present patient is unusual and belongs to group (iii). A
Fig. 3. Schematic diagram of multiple genito-urinary defects before operation. Abbreviations: BU = Bicornuate uterus; M Ur = Megalourethra (phallic); P = Phallus; R = Rectum; TB = Tuba1 blockage (bilateral); Ur (E) = Ectopic urethra; UB = Urinary bladder; VS = Vaginal septum.
Fig. 4. Planning of abdomino-perineal operation. UB = Urinary bladder; R = Rectovaginal septum.
CASE REPORTS
Fig. 5. Schematic presentation of reconstructive surgery before excision of phallus. ( I ) Repair of anterior rectal wall at the site of ectopic opening of vagina and urethra; (2) Anterior approximation of puborectalis muscle; (3) Reconstruction of perineal body.
few similar cases have been reported by Grubenmann (1912), Howard & Hinman (I95 I), Bonney et al. (1975) and Thiry et al. (1979); the last two groups of authors claimed theirs to be the 4th and 5th instance in literature but Bellinger & Duckett (1982) and Kounami et al. (1986) recently analysed 19 cases including their own. The clinical features varied widely. The ectopic openings of the vagina and urethra inside the rectum, with accessory phallic urethra as in our patient, have not been described before. This has been confinned by many through personal communication (The Royal Society of Medicine, UK; Royal College of Obstetricians & Gynaecologists, UK; Prof S. S. Ratnam, Singapore; Prof John E. Skandalakis, Georgia, USA; Sir John Stallworthy, UK; Prof Peter L. Williams, UK; Prof Carl Wood, Australia).
Fig. 6. Correction of external genital malformations with ablation of phallic urethra and excision of the phallus.
691
The simple idiopathic group comprises a range of urogenital anomalies and degrees of phallic differentiation (Kounami et al. 1986). A functional double urethra has been recorded in a few cases (Grubenmann 1912; Jones & Scott 197 1; Bonney et al. 1975; Belis & Hrabovsky 1979; Hurwitz & Fitzpatrick 1982), but ‘Y’-type duplication is more frequent (Perloff et a/. 1953; Jones & Scott 1971; Bellinger & Duckett 1982). The phallic urethra was stenotic in most cases with a terminal stricture (Hurwitz & Fitzpatrick 1982). The rectal urethra functioned normally in our patient so that there was no obstructive uropath y. In the normal woman, the ventral corpus spongiosum does not differentiate, resulting in an absence of a phallic urethra and causing a ventral curvature. The clitoral prominence in the present case may be accounted for by phallic urethralization, as suggested by Bellinger & Duckett (1982), which prevents the development of a normal female ventral chordee. The terminal stricture of the phallic urethra, as observed by many (Bonney etal. 1975; Belis & Hrabovsky 1979; Hurwitz & Fitzpatrick 1982), is the result of separate development of this part from an ectodermal groove on the undersurface of the corona glandis (Bellinger & Duckett 1982; Marberger 1975). The megalourethra is the result of abnormal development of the phallus, but there is controversy about the fibro-elastic cylinders (Belis & Hrabovsky 1979; Huben et al. 1981; Kounami et al. 1986). Surgical treatment of the phallic urethra is not often
Fig. 7. A close view of the external genitalia, after reconstructive surgery.
692
C A S E REPORTS
cult to ascertain. Possibilities include a local disturbance of the inductive factor (Howard & Hinman, 1951), resulting from an incomplete union between the Miillerian ducts and the urogenital sinus; this might be responsible for the uninhibited development of an accessory phallic urethra. The urethralization of the female phallus is distinguishable from masculinization in adreno-genital syndrome by the presence of both phallic and vulva1 urethral orifices. Turnock & Rickwood (1987) suggested that this may be due to failure of differentiation of the peri-cloaca1 mesoderm, because of late arrival and incomplete fusion of the Miillerian ducts. Our patient had unique multiple atypical genito-urinary deformities and surgical correction was followed by a successful pregnancy. Such a case has not been described before.
Acknowledgments The author is grateful to staff, Department of Obstetrics & Gynaecology, R.K.M.S.P. Hospital for their assistance; to Dr Asim Kr Dutta, Professor of Anatomy, University College of Medicine, Calcutta for his guidance; the secretarial staff for their support and the Secretary of the hospital for kind permission to use the hospital data. He is thankful to the numerous scientists for personal communication and especially to the Librarians of the Royal College of Obstetricians & Gynaecologists and the Royal Society of Medicine, UK, for computer search and reprints.
References
Fig. 8. Post-operative cysto-urethrogram showing the 4 cm length of the urethra relocated from the rectum.
necessary as little urine usually passes through it (Bellinger & Duckett 1982). Diagnosis is usually at birth or in early childhood because of the ambiguous external genitalia, but there may also be obstructive uropathy, urinary tract infection and frequent dribbling of urine. Supra-pubic cystostomy and cutaneous nephrostomy have been necessary when there was severe urinary tract obstruction (Grubenmann 1912; Belis & Hrabovsky 1979). The treatment is usually directed towards relieving urinary obstruction and correcting the masculinized external genitalia. Clitoridectomy has now been replaced by clitoroplasty relocating normal caudal curvature to reduce the phallic prominence and to preserve normal clitoral sensation. The excess shaft skin has been used to reconstruct the labia minora (Bellinger & Duckett 1982). The internal genitalia rarely require surgical correction, but reduction vaginoplasty and vaginal septotomy (Hurwitz & Fitzpatrick 1982) are recorded. Laparotomy is not necessary for the assessment of gender (Bellinger & Duckett 1982). Uteroplasty and tuboplasty were essential in the present patient as such a major degree of uterine deformity might have not been compatible with term pregnancy. Clitoridectomy was obligatory as clitoroplasty was technically difficult because of the high anatomical location of the phallus. The embryological pathogenesis in the present case is diffi-
Belis J. A. & Hrabovsky E. E. (1979) Idiopathic female intersex with clitoromegaly and urethral duplication. J Urol 122, 805-808. Bellinger M. F. & Duckett J. W. (1982) Accessory phallic urethra in the female patient. J Urol 127, 1159-1164. Blum V. (1904) Die Hypospadie der weiblichen Harnrohre. Monatshericht Urology 9, 522. Bonney W. W., Young H. H., 11, Levin D. & Goodwin W. E. (1975) Complete duplication of the urethra with vaginal stenosis. J Urol 113, 132-137. Goligher J. C. (1958) The treatment of complete prolapse of the rectum by the Roscoe Graham operation. Brit J Surg 45, 343. Goligher J. C. (ed.) (1975) Surgery ofthe Anus, Rectum and Colon. 3rd ed., Baillikre Tindall, London, pp. 292-340. Grubenmann I. (19 12) Eine sagittale Verdoppelung der weiblichen Hamrohre. Frankjurt Ztschr Path 10, 101-128. HowardF. S. &HinmanF., Jr. (1951)Femalepseudohennaphroditism with supplementary phallic urethra: report of two cases. J Urol65, 439-452. Huben R. P., Devine P. C., Harkins G. A. & Stecker J. F., Jr. (1981) Clitoromegaly and megalourethra in idiopathic female intersex. J UI-01126,849-85 1. Hunvitz R. S. & Fitzpatrick T. J. (1982) Vaginal urethra, clitoral hypertrophy and accessory phallic urethra: a rare syndrome of female pseudohennaphroditism. J Urol 127, 1165-1168. Johnson R. J., Palken M., Derrick W. & Bill A. H. (1972) The embryology of high anorectal and associated genito-urinary anomalies in the female. Surg Gynecol Ohstet 135, 759-762. Jones H. W., Jr. & Scott W. W. (1971) Hermaphroditism, Genital Anomalies and Related Endocrine Disorders, 2nd ed., Williams & Wilkins Co., Baltimore, p.275. Klugo R. C., Fisher J. H. & Retik A. B. (1974) Management of urogenital anomalies in cloaca1 dysgenesis. J Urol 112, 832-835. Kounami T., Takeuchi H., Takayama H. & Tomoyoshi T. (1986)
C A S E REPORTS
Vaginal and phallic urethra with prominent clitoris in female pseudohermaphroditism.J Urol 136,915’)18. Marberger M. J., Jr. (1975) Accessory phallic urethra in female pseudohermaphrodite. Urology 5,505-507. Park I. J., Johanson A., Jones H. W. & Blizzard R. (1972) Special female hermaphrodite associated with multiple disorders. Obst
693
Thiry A. J., Wincqz P. J. & Schulman C. C. (1979) Female pseudohermaphroditism with supplementary phallic urethra. Eur Urol 5, 285-286.
Tumock R. R. & Rickwood A. M. M. (1987) Urethralisation of the female phallus: a rare form of intersex. Brit J Urol59(5),481-482.
Gynecol39, 100-105.
Perloff W. H., Conger K. B. & Levy L. M. (1953) Female pseudohermaphroditism: a description of two unusual cases. .I Clin Endocrinol 13. 783-790.
Received 11 September 1990 Resubmitted 5 November 1991 Accepted 19 January 1992
British Journal of Obstetrics and Gynaecology August 1992, Vol. 99, pp. 693-695
Intermittent abdominal decompression: an option for prevention of intrauterine growth retardation S. S H I M O N O V I T Z S. YAGEL D. Z A C U T A. B E N - C H E T R I T D. H O C H N E R C E L N I K I E R M. R O N Department of Obstetrics and Gynecology Hadassah University Hospital Mount Scopus, Jerusalem, Israel
Case reports Patient 1 A 26-year-old healthy woman was admitted in the 12th week of her sixth pregnancy because of her bad obstetric history. All her previous pregnancies had been complicated by hypertension and severe intrauterine growth retardation; the first three pregnancies were terminated between 26 and 32 weeks gestation because of intrauterine fetal death accompanied by moderate to severe hypertension. In her fourth pregnancy caesarean section was performed at 3 1 weeks gestation because of moderate hypertension, a small fetus, oligohydramnios and a positive oxytocin challenge test. The infant, who weighed 700 g at birth, died 4 days later from cardiorespiratory failure. The fifth pregnancy was terminated at 28 weeks gestation, again because of moderate hypertension and the death in-utero of a very small fetus. She was treated in this pregnancy by bed rest, hydralazine, methyldopa, low dose aspirin and prednisolone. No underlying cause was found for the recurrent hyperCorrespondence: S. Shimonovitz MD, Department of Obstetrics & Gynecology,Hadassah Mount Scopus, Jerusalem 91240 POB 24035, Israel.
tension, poor fetal growth and intrauterine death. On admission in her sixth pregnancy her blood pressure was 130/85 mmHg. She was treated with intermittent abdominal decompression using a Heyns’ suit (Heyns 1963) with negative pressure of around 70 mmHg for 30 s every minute for 30 min twice a day. In addition she was treated by methyldopa. Her blood pressure during the pregnancy did not exceed 120/85 mmHg. Fetal growth was monitored by repeated ultrasonographic examinations and was normal. Repeated systolic/ diastolic (S/D) ratios were calculated from the umbilical artery flow velocity waveforms obtained by continuous Doppler ultrasound, values ranged between 3.3 and 3.7. Umbilical artery flow velocity waveforms were not recorded during treatment with decompression because the ultrasound transducer could not be used within the sealed unit. At 38 weeks gestation a healthy 3280 g male infant was born by caesarean section.
Patient 2 A 27-year-old healthy woman was admitted in the 15th week of her fourth pregnancy because of her bad obstetric history. She developed moderate hypertension during her first pregnancy which ended in intrauterine fetal death at 28 weeks. In her second pregnancy labour was induced at 37 weeks because of moderate hypertension and a small fetus. A 1900 g infant was born vaginally. No underlying cause for the recurrent hypertension was found. In her third pregnancy she was treated by bed rest, low dose aspirin (100 mg/day) and dipyridamole (225 mg/day). At 32 weeks gestation intrauterine death occurred. In her fourth pregnancy treatment with intermittent abdominal decompression, as described for patient 1, was initiated at 15 weeks. Her blood pressure was normal throughout the pregnancy and no drug treatment was given. Repeated S/D ratios, calculated during the treatment period from umbilical artery flow velocity waveforms, were below 3.5. At 39 weeks gestation a normal 4100 g male was born spontaneously. In her fifth pregnancy she used the decompression suit at home
