Pratiksha Yadav, MD

 A 12-year-old girl presented with history of enlargement of middle and ring fingers of right hand since birth. Size of the fingers was progressively increased. There was no associated pain or tenderness, skin over the fingers was normal. There was no history of any systemic disease. Radiograph of right hand revealed hypertrophy of all the phalanges of middle and ring fingers with overgrowth of overlying soft tissue and nail.

 Radiograph of right hand, PA and oblique view showing overgrowth of phalanges and soft tissue of middle and ring fingers.

 Macrodactyly is a rare nonhereditary congenital malformation, which causes overgrowth of digits and fibro adipose tissues of one or more fingers and toes. Syndactyly is associated in 10 percent of cases.

 Macrodactyly is a rare benign congenital condition, which is presented with enlarged size of one or more finger or toe. All the elements of fingers or toes are enlarged, e.g. phalanges, muscles, tendons nerves, vessels, subcutaneous tissues, nails and skin. However metacarpals and metatarsals are not involved (1).

True macrodactyly must be differentiated with tumorous enlargement of single element like hamartoma, enchondroma or lymphangioma [2]. There are two types of macrodactyly: static and progressive forms. In static form the overgrowth is present at birth and there is proportionate growth of the digits. In progressive form the overgrowth may or may not present at birth, but begin to enlarge in early childhood frequently with an annular deformity [3]. Hands and feet are affected in equal frequency, slightly male predominance in reported overall cases [4], involvement of more than one digit may occur.

Macrodactyly is an isolated finding in most of the patients, however may be associated with rare syndromes, e.g. Proteus syndrome, Ollier disease, Maffuci syndrome and Bannayan syndrome. Although the cause is not known, three possible factors are responsible for it abnormal nerve supply, abnormal blood supply and abnormal humoral mechanism [3]. Diagnostic investigation is by radiography and CT scan for the extent of bone. Histopathology of macrodactyly is hypertrophy of bone with hamartomatous overgrowth of lipomatous or fibrous tissue.

Various surgeries are described for macrodactyly bulk reduction (defatting), resection of nerves, finger shortening, angulation osteotomy and amputation [1]. Purpose of the surgery in these cases is to improve cosmetic appearance and preserve neurological function. Timing and extent of therapeutic measures should consider carefully for the best functional outcome [2].


1 Kotwal P.P.,Farooque M. J Bone Joint Surg [Br] 1998;80-B:651-3.
2. Firdous Khan, Syed Asif Shah, Naji Ullah Khan, Irfan Ullah, Faheem Ullah. Pak J Surg 2010; 26(3):242-245
3.Sharma S,Vyas S,Negi A,Sood R G,A,Kaushik N K. Macrodactyly:Report of three cases.Indian J Radiol Imaging 2006;16:583-4
4.Kalen V, Burwell DS, Omer GE. Macrodactyly of the hands and feet. J Pediatr Orthop. 1988; 8: 311-15.

Pratiksha Yadav, MD, Assistant Professor, Department of Radiodiagnosis, Padmashree Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India

Articles

Case History: A 12-year-old girl presented with history of enlargement of middle and ring fingers of right hand since birth. 

Macrodactyly

Figure 1: Sagittal image of USG larger right side of the uterus with separate endometrial cavity and separate cervix.

Abstract: Uterine didelphys constitutes approximately 5 percent of mullerian duct anomalies. There is nearly complete non fusion of Mullerian Ducts causing duplication of uterus and cervix. A longitudinal vaginal septum is seen in 75 percent of these cases (1). We report an incidentally detected uterine didelphys in a 23-year-old G2P1 patient came for ultrasound with complaint of vaginal bleeding.

Case history: A 23-year-old female came for ultrasound with complaints of bleeding P/V associated with diffuse pain in abdomen since 18 days, history of consumption of abortion pills after 45 days of missed period, and has 2-year-old male child which was full term normal delivery.

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Figure 2 : Saggital USG image showing smaller left side of uterus with separate endometrial cavity and separate cervix.

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Figure 3b: Transverse USG image of uterus and two separate endometrial cavities. We can see two separate divergent uterine bodies which are not connecting with each other.

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Figure 4 : T2W coronal image of uterine didephyes showing two separate diverging uterine bodies with two separate endometrial cavities (central hyperintense signal).

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Figure 5 : T2W coronal image of uterine didephes showing two separate cervices with longitudinal septum in between the vagina

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Figure 6: T2W saggital image showing larger right uterus body and cervix with separate endometrial cavity.

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Figure 7 : T2W saggital image showing smaller left uterine body and cervix with separate endometrial cavity.

On ultrasound we detected that there were two separate uterus and cervices (Figure 3). The uterus on right side was slightly larger in size than left side (Figures 1 and 2). There were two separate endometrial cavities seen. Endometrium of both the uteri were normal in thickness. No evidence of any other lesion could be seen. Both the ovaries were normal. Both the kidneys ,ureters and bladder were normal.

An MRI was advised for further evaluation. MRI findings have confirmed our USG findings. There were two separate well defined uteri seen with two separate cervices (Figure 4) .The right side uterus measures approx.9.5 (Craniocaudal) x 2.6 (AP) x 3.2 (Transverse) and the left side uterus measures approx. 4 (Craniocaudal) x2.2 (Anteroposterior) x 3 (Transverse).

There is a longitudinal septum seen in the proximal part of vagina (Figure 5). In uterine didephyes two uterine bodies normally are of similar size. However in our case the right uterus is larger than the left (Figures 6 and 7), which might be because patient had one childbirth. Possibly the fetus was on right side and so the right side is of larger size.

Discussion: The Mullerian duct anomalies are uncommon cause of infertility. There are higher incidences of preterm labor, repeated spontaneous abortion or fetal malposition seen in patients with mullerian duct anomalies. Adolescent girls can present with hematocolpos or mucocolpos. Occasionally the anomaly detect incidentally during imaging for another condition or as a normal check up. Most mullerian duct anomalies are associated with normal functioning ovaries and normal external genitalia.

Two paired mullerian ducts develop into the female reproductive tract which include fallopian tubes, uterus, cervix and upper two third of vagina. The ovaries and lower one third of vagina develop from the thickened portion of posterior urogenital sinus called the sinovaginal bulb. Mullerian duct anomalies results from interruption of the normal mullerian duct development at any stage. Intrauterine and extrauterine elements, genetics, and teratogens (eg, 

diethylstilbestrol(Drug information on diethylstilbestrol)

thalidomide(Drug information on thalidomide)

), have been associated with mÃ¼llerian duct anomalies (7).

Mullerian duct anomaly can be Classifed into 7 classes according to the American Fertility Society (AFS) Classification Scheme(2) as follows:
Class I anomalies consist of agenesis and variable degrees of uterovaginal hypoplasia.
Class II anomalies represent unicornuate uteri that may be partial or complete unilateral hypoplasia.
Class III is Uterine didelphys in which complete nonfusion of mullerian ducts resulting in duplication of uterus.
Class IV anomalies are bicornuate uterus, result of the incomplete fusion of the superior segments of the uterovaginal canal.
Class V anomalies are septate uteri resulted from the partial or complete non resorption of uterovaginal septum.
Class VI anomalies represent arcuate uteri that result from near complete resorption of the septum.
Class VII anomalies represent sequelae of in utero DES exposure.(4)

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Figure 8: Classification System of Mullerian Duct anomalies by American Fertility Society (2).

Uterine didephys constitutes approximately 5 percent of mullerian ducts anomalies(3). It is the result of nearly complete failure of fusion of mullerian ducts. There are two complete uterus and cervix with two separate endometrial cavities. A longitudinal vaginal septum seen in 75 percent of these cases.(1) Longitudinal septa can be associated with transverse vaginal septa which can result in hematometrocolpos. Non obstructive Uterine didephys is usually asymptomatic.(3)

Ultrasonography can detect two separate divergent uterine bodies and two separate cervices. Endometrial cavities are separate with no communication.

MRI is the best modality for evaluation of mullerian duct anomalies. Characterization of the uterus, cervix, endometrial cavity are best in MRI. We can evaluate the vagina and vaginal septum also by using MRI. MR imaging provides clear delineation of internal and external uterine anatomy in multiple imaging planes and most importantly reliable depiction of the external uterine contour. MRI also have the ability to detect the frequently associated renal anomalies. (4)

Hysterosalpingography is also a technique for the evaluation of the uterine congenital anomalies however it has major limitation that it can characterize only the endometrial canal and inability to evaluate the external uterine contour. It has the disadvantage of radiation exposure and painful technique.

Didelphic uterus can progress for a successful pregnancy (57 percent) (8) with a fetal survival rate as high as 64 percent(9). Spontaneous vaginal delivery as well as cesarean section at term has been reported(10-11).

Pratiksha Yadav, MD, assistant professor, and Brig. Amarjit Singh, MD, professor and dean, Department of Radiodiagnosis, Padmashree Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India.

Ultrasound, MRI diagnose Mullerian duct anomalies in 23-year-old female. Bleeding P/V, diffuse abdominal pain 18 days; abortion pills use after 45 days of missed period.

Author | Pratiksha Yadav, MD

Articles

Macrodactyly

Uterine Didelphys