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Unusual Diagnosis Of The Week: Digital Mucous Cyst

July 23rd, 2009 by RamonaBatesMD in Better Health Network, Health Tips
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Earlier this month I received an e-mail asking for information.  I have changed it slightly:

I have been pouring over the computer, searching for information on a tumor.  I had removed last week from my left long finger (third finger).  The pathology report came back as a myxoid tumor.  I was told it was a tumor, not a cyst.  Could you help me give me more information?

To begin, most digital myxoid or mucous tumors are cystic in nature.  Very few are not.  It is difficult to find much information in the literature, especially the current literature.

Digital mucous cysts (DMCs) are benign ganglion cysts.  They most often are located at the most distal joint of the finger or in the nail fold.  Physicians call this joint the distal interphalangeal (DIP) joints.   The fingers are most commonly involved, but DMCs may occur on the toes. (photo credit)

The etiology of these cysts is not known.  DMCs are also called myxomatous cutaneous cysts, periungual ganglions, mucous cysts, myxoid cysts, synovial cysts, dorsal cysts, nail cysts, cystic nodules, digital mucoid cysts, digital myxoid cysts, and digital mucinous pseudocysts.

The cyst often has a smooth shiny surface on exam.  If located near the nail, there will often be a groove in the nail as in the photo above.  The cyst’s size may vary.

If the cyst is asymptomatic, then treatment is not required.  Recurrence is common regardless of which treatment is used.

• Aspiration of the contents (72% success rate with multiple aspirations, 2-5 treatments)
• Cyrotherapy (56% to 86% success rate)
• Steroid injection
• Surgical excision (88% to 100%)

Restriction of joint mobility, nail dystrophy, and changes to the contour of the proximal nail fold are potential drawbacks.

When considering a difference diagnosis keep in mind the following:

• Epidermoid cyst
• Heberden node or Rheumatic nodule
• Fibrokeratoma (DMCs may resemble this when they form between the proximal nail fold and the nail and protrude with a keratoticlike tip.)
• Giant-cell tendon sheath tumor
• Myxoid malignant fibrous histiocytoma
• Myxoid variant of liposarcoma (These are less likely to present as firm circumscribed masses and more likely to be deeply seated.)

REFERENCES

Digital Mucous or Myxoid Cyst

Digital mucous cyst – emedicine dermatology, the online textbook

Myxoid Cyst

Cystic Lesions of the Hands; Clinical Advisor, November 12, 2008; Noah S. Scheinfeld, MD, JD

Digital myxoid cysts: a review; Cutis; Feb 1986;37(2):89-94; Sonnex TS.

Myxoid Cysts; JAMA, Dec 1965; 194: 1239; Frederick A. J. Kingery

Ganglion of the Distal Interphalangeal Joint (Myxoid Cyst): Therapy by Identification and Repair of the Leak of Joint Fluid; ARCH DERMATOL/VOL 137, MAY 2001; David de Berker, MRCP; Clifford Lawrence, FRCP

Ray Amputation As A Treatment for Recurrent Myxohyaline Tumor of the Distal Extremity; Plastic and Reconstructive Surgery. 111(4):1573-1574, April 1, 2003; Mowlavi, Arian; Quinn, Brendon M.; Zook, Elvin G.; Milner, Stephen

Soft-Tissue Sarcomas of the Upper Extremity: Surgical Treatment and Outcome; Plastic and Reconstructive Surgery. 113(1):231-232, January 2004; Steinau, Hans-Ulrich; Kuhnen, Cornelius

Soft-Tissue Chondroma in the Thumb; Plastic and Reconstructive Surgery. 110(6):1599-1600, November 2002; Avc, Gülden; Aydogdu, Eser; Ydrm, Serkan; Aköz, Tayfun

*This blog post was originally published at Suture for a Living*

Unusual Syndrome Of The Week: Macrodactyly

July 14th, 2009 by RamonaBatesMD in Better Health Network
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Macrodactyly is an uncommon anomaly of the extremities.  It can affect both the fingers or toes which become abnormally large due to overgrowth of the tissues composing the digit.  All the tissues are involved:   bone and soft tissue-particularly the nerves, fat and skin.  (photo credit)

Other names used for macrodactyly include megalodactyly, overgrowth, gigantism, localized hypertrophy, or macrodactylia fibrolipomatosis.

Hands are more commonly involved than feet. Most of the time (~90%) patients present with unilateral (one side affected) macrodactyly.  Often more than one digit is involved.   The most frequently involved digits of the hand are the index finger, followed by the long finger, thumb, ring, and little fingers.  Syndactyly may be present in 10% of patients.   Men are more often affected than women.

It is not known why macrodactyly occurs.  It does not appear to be an inherited anomaly, but there are some syndromes (ie Proteus Syndrome, Maffuci syndrome, and tuberous sclerosis) which may be associated with enlarged digits. There are some surgeons who believe that macrodactyly is a variant of neurofibromatosis.

Macrodactyly may be either static or progressive.   The progressive type is more common than the static.

• In static the enlarged digit (finger or toe) is present at birth and continues to grow at the same rate as the normal digits of the hand.  The involved digits are generally about 1.5  times the normal length and width of the normal digits.
• In progressive the affected digits begin to grow soon after birth and continue growing faster than the rest of the hand.  The involved digit or digits can become enormous.

There is no medical treatment for this disorder.  It is treated by surgery.   In the hand, the indications for surgery can often be cosmetic in nature as the hand can functionally tolerate a digit with some increased width and length.  In the foot, the enlarged digit can make shoe fitting/wearing difficult.

Surgical treatment of macrodactyly is complex as multiple tissue layers are involved.  It typically will involve debulking, epiphyseal arrest, and shortening.  Multiple surgeries are the norm.

Soft tissue debulking:

• This is done to help correct the width of the digit. This is often done at the same time as the epiphyseal arrest.   The affected fingers are approached volarly with Bruner-type incisions/flaps. The fat is removed from the skin and the tissues are debulked.
• Care is taken to preserve the ulnar and radial digital neurovascular bundles. Sometimes the enlarged nerve branches will need to be sacrificed along with the enlarged subcutaneous tissues.
• When a sufficient amount of tissue has been removed, the skin flaps are overlapped and excised, which allows for tension-free closure.  It needed, skin grafts using healthy skin will be done.
• Debulking is often need to be done in staged procedures.

Shortening procedures:

• This is done to help correct the length of the digit.  Shortening procedures usually involve either surgical excision (removal) of one of the phalanges of the finger or toe, or removal of a metacarpal (hand bone) or metatarsal (foot bone).
• Barsky and Tsuge originally introduced the two most described methods. Barsky’s technique involves removing the distal portion of the middle phalanx and proximal portion of the distal phalanx, thereby reducing the length of the finger while preserving the nail. Tsuge’s technique also preserves the nail by overlapping the dorsal portion of the distal phalanx with the volar portion of the middle phalanx.

Ray resection:

• This may be done in progressive macrodactyly.  It involved the complete removal of the digit or digits.  It is also an option if there is excessive widening of the forefoot, where digital shortening and debulking procedure may not be effective.

Epiphyseal Arrest:

• The timing of the this surgery is critical.  An attempt to “guess” the adult finger length is done by comparing the child’s digits with those of his/her parents.  When growth of the affected digits matches those of the parent, epiphyseal arrest can be performed.   This in effect will stop the bone growth of the digit.
• The epiphyses of the proximal and distal phalanges  are the ones treated by disruption or removal.   The middle phalanx epiphyses is not treated to help preserve motion at the proximal interphalangeal joint.

Other surgical options include amputation and wedge osteotomies.  Amputation is reserved for patients with nonfunctioning digits or digits that are extremely difficult to correct.  Wedge osteotomies are performed in patients who have digits that are grossly deviated.

Complications of macrodactyly surgery include poor healing of flaps secondary to devascularization or undue tension, nerve injury or decreased sensation, infection, stiffness, bony nonunion or malunion, and failure of the epiphysiodesis.

REFERENCES

Wood VE. Macrodactyly. In: Green DP, Hotchkiss RN, eds. Operative Hand Surgery. 4th ed. New York, NY: Churchill Livingston; 1998:533-544.

Treatment of macrodactyly;  Plast Reconstr Surg. 1967; 39:590-599; Tsuge K.

Congenital anomalies of the hand; Cohen M, ed. Mastery of Plastic and Reconstructive Surgery. New York, NY: Little, Brown and Company; 1994; Upton J, Hergrueter C.

Macrodactyly; Boston Children’s Hospital website

Macrodactyly; Wheeless’ Textbook of Orthopaedics

*This blog post was originally published at Suture for a Living*

Congenital Anomaly Of The Month: Poland’s Syndrome

July 3rd, 2009 by RamonaBatesMD in Better Health Network
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Poland’s syndrome is a congenital disorder. The classic ipsilateral features of Poland syndrome include the following: absence of sternal head of the pectoralis major, hypoplasia and/or aplasia of breast or nipple, deficiency of subcutaneous fat and axillary hair, abnormalities of rib cage, and upper extremity anomalies. These upper extremity anomalies include short upper arm, forearm, or fingers (brachysymphalangism). (photo credit)

Additional features of Poland syndrome include the following: hypoplasia or aplasia of serratus, external oblique, pectoralis minor, latissimus dorsi, infraspinatus, and supraspinatus muscles; total absence of anterolateral ribs and herniation of lung; and symphalangism with syndactyly and hypoplasia or aplasia of the middle phalanges. (photo credit)

The name of this condition pays homage to Dr. Alfred Poland of Guy’s Hospital, who in 1841 described a case of these two deformities during the autopsy of a 27-year-old convict, but as this article points out he wasn’t the first to recognize the syndrome.

If you enjoy medical history, then you will enjoy this article. It explores the historical literature to reveal the progression of knowledge about this syndrome. Here is a quick summary of different investigators who contributed to the understanding of Poland’s syndrome. The article goes into more detail of each.

As the authors conclude:

Honoring physicians for notable achievements in the form of eponyms can be viewed as a harmless way to bring a little bit of warmth to an otherwise cold world of facts. The least we can do, though, is to recognize the contributions of those who endeavored to shape our current understanding of disease.

Perhaps if history took another course, Poland’s syndrome would instead be called Frolep’s syndrome or Furst’s syndrome. Or perhaps it might simply have been called pectoral-aplasia-dysdactylia syndrome

REFERENCES

Poland’s Syndrome: Current Thoughts in the Setting of a Controversy; Plastic & Reconstructive Surgery. 123(3):949-953, March 2009; Ram, Ashwin N. B.S.; Chung, Kevin C. M.D., M.S. (subscription required)

*This blog post was originally published at Suture for a Living*

Should Doctors Disclose How Many Procedures They’ve Done?

June 14th, 2009 by RamonaBatesMD in Better Health Network
1 Comment »

Recently the WSJ Health Blog posted “Should Doctors Say How Often They’ve Performed a Procedure?” written by Jacob Goldstein.   It references another guest post by Adam Wolfberg, M.D — “Test Poses Challenge for OB-GYNs”

Dr. Wolfberg writes:

None of the published studies of CVS pitted seasoned physicians against novices; what patient would agree to be randomly assigned to an inexperienced doctor holding a long needle? But several reports from individual hospitals demonstrate that the miscarriage rate declined over time as the hospital’s staff became more experienced.

These reports point to a dilemma: CVS mavens got that way by practicing, so their present-day patients benefit at the expense of previous patients.

When I first began my solo practice 19 years ago, patients often asked how long I had been in practice.  They ask less often these days.  I have never failed to answer.

Patients sometimes ask how many times I have done a procedure, but not often.  Early in my practice, and sometimes even now, if it is a procedure I feel a bit uneasy with or haven’t done in a while I will bring the subject up without being asked.  After all, some procedures you just don’t do every day or even every month.  Some diseases you don’t see every month or even every year.

In my mind, many of the procedures I do are built on basic surgical principles.  I withdrew my privileges for microvascular procedures more than 10 years ago.  I didn’t get enough patients referred to me to feel that my skills were kept sharp.  In private practice, unlike at a university, there are no labs to go do practice work in to maintain those rarely used skills.  I have no doubt that I could regain them given the chance, but at what cost (financially or complications).

Because I gave up my privileges for microvascular procedures, it means I have limited my repertoire of reconstructive procedures important in hand, breast, and other work.  I tell my patients about them.  If a breast reconstruction patient wants a free TRAM flap, then she is referred to someone who does it.  If she  wants to keep me as her surgeon, is there the possibility she is short changing herself on the outcome?  I suppose, but I try (TRY) to be upfront and fair to each patient.

The question asked “should doctors say how often they’ve performed a procedure?” may seem an easy one to answer.   If asked, yes.  If not asked, should it be part of the consent form?  I’m not sure it should for most procedures, but for extremely complex ones, maybe.

What if I did 100 of one type of procedure, but my last one was over a year ago?  What if I have done 50 of a second procedure that is closely related in skill-set?  What if that number is only 15? What if I have never done one and don’t wish to now, but the patient needs the procedure and is not willing to travel to another hospital?  Is it okay that I have “informed” them, but they want to take the risk?  How do I define that risk for them?

How many of which procedure is enough to become proficient?  How often does it need to be done to remain proficient?  Who gets to define proficient?  Who gets to define the “magic” number of how many is enough to be proficient?  Who get to define how often the procedure needs to be done to remain “proficient”?

As Dr Wolfberg noted

What patient would agree to be randomly assigned to an inexperienced doctor holding a long needle?

So how will these questions be answered?

*This blog post was originally published at Suture for a Living*

Discovered On Twitter: Hospitals Recruit Nurses With Free Plastic Surgery

June 11th, 2009 by MotherJonesRN in Better Health Network
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Life is good. I’m settling into my job at UGH (Undisclosed Government Hospital) and I have a couple of days off from work. I’m using my time constructively. My house looks like hell, but I am doing other important things like writing, reading blogs, and visiting Twitter.

Yes, I’m addicted to Twitter. I started tweeting when I hooked up with Pixel RN and Dr. Val at BlogHer last year. They showed the joys of micro-blogging and my life was changed forever. Twitter is great place to meet people using 140 characters at a time. You can hangout in cyberspace with people like Ashton Kutcher, Lance Armstrong, and Stephen Colbert. You can also hangout with a lot of great healthcare providers. I make new “friends” by putting the word “nurse” into the Twitter search engine. Then I sit back and see what pops up.

Yesterday, something very interesting caught my eye. Dr. Hess, a plastic surgeon, tweeted that nurses were being offered free plastic surgery. I love free stuff, so I followed the link in his tweet, and checked out his blog. He wrote a great post. I also checked out the link in his post to the New York Times. The upshot of the story is that some places in Europe are offering plastic surgery as a recruiting tool for nurses. The story talked about the enormous social pressure that some nurses are under to look good. It’s true. Even some hospitals in the United States are using young and beautiful nurses as a marketing tool to entice more patients into their facilities. Age discrimination is rearing its ugly head. I wrote this post about a nurse who lost her job because she was getting old and because she wasn’t pretty anymore.

I tweeted Dr. Hess. I told him that there wasn’t enough plastic on the planet that could make this sow’s ear into a silk purse. I also told him that I look forward to tweeting with him in the future. He wrote back and told me that he thinks that I’m charming. Just wait till he really gets to know me!

I’m going to Twitter my way through life.

*This blog post was originally published at Nurse Ratched's Place*