Reconstructive Plastic Surgery MMK

1. Abdominal wall

2. Breast

3. Colorectal

4. Congenital anomalies

a. Cleft lip and palate

Cleft lip and/or palate is the most common malformation affecting the face, with a global incidence of approximately 1 in 700 births (1). Children treated for cleft deformities have a risk of orthognathic and dental developmental abnormalities as well as aesthetic stigmatization and speech disorders later in life. Various surgical protocols have been suggested over the years but there is to this date no global consensus or golden standard regarding preferential timing and optimal surgical technique for cleft lip and palatal repair.

Study 1.

Background: Cleft palate patients are operated with a 2-stage reconstruction in 4 out of 6 Swedish national cleft centers. In the remaining 2 centers, a 1-stage reconstruction is utilized. The 2-stage protocol include closure of the soft palate at 6 months of age and closure of the hard palate at 2 years of age. In contrast, when the palate is closed in one session, both the soft and hard palate is repaired when the patient is around 1 year. Previous studies and the national cleft registry have failed to show any difference in long term outcomes between the two surgical protocols, including speech development and maxillary growth (2-4). This study aim to investigate if there are any differences in burden of care and short term complications. Burden of care has to our knowledge not been previously assessed in this context.

Materials: At Karolinska University Hospital cleft palate patients were operated with the 1-stage procedure up until 2012 and thereon after the 2-stage procedure has been practiced. In this study patients operated between 2002-2012 with the 1-stage technique will be compared with patients operated between 2012-2022 with the 2-stage technique. An estimated 800 cleft patients will be included in the study. Background variables including cleft type, syndromes, age at operation, cleft width and operating surgeon will be recorded. Burden of care in terms of operating time, hospital stay, re-admissions and number of contacts with the cleft team within 4 weeks postoperatively will be assessed. Occurrence of bleeding (requiring blood transfusion or reoperation) and postoperative palatal fistulas will also be recorded and compared between the two cohorts.

Impact: Since no differences in long term outcomes has been found between the 1- and 2-stage palatal repair approaches, burden of care aspects are especially important to assess and consider when establishing national and international guidelines for cleft palate patients.

The Global Occurrences of Cleft Lip and Palate in Pediatric Patients and Their Association with Demographic Factors: A Narrative Review. Putri et al, Children (Basel). 2024 Mar 8;11(3):322
Årsrapport avseende data och aktiviteter 2022, Svenska LKG-registret (https://lkg-registret.se/publikationer-och-forskning/arsrapporter).
A systematic review of differences in outcome between one and two stage palate Repair in cleft lip and palate. Cornefjord et al, Journal of Plastic Surgery and Hand Surgery, 2023, VOL. 58, 132–141
Är en- eller tvåstegsoperation att föredra vid rekonstruktion av gommen för läpp-käk-gomspalt? HTA-rapport, Region Skåne, HTA Syd, Rapport 2022:3

b. Microtia

The term microtia describes a spectrum of congenital anomalies of the auricle of the ear. These can range from mild structural abnormalities to complete absence of the ear (anotia). Previous population-based studies show a wide range in prevalence of microtia, depending on geographical region. Prevalence ranges between 0.83 and 4.34 per 10,000 births. (1) Bilateral microtia is more often associated with other anomalies or syndromes. (2) To date, no causal genetic mutation has been associated with isolated microtia, although individual gene mutations have been identified in syndromic cases. Sporadic cases are likely to be multifactorial. Suggested risk factors for microtia include male sex, low birth weight, advanced maternal age, high parity (>4), maternal diabetes mellitus, and Hispanic ethnicity. (1, 2) There is also some evidence for an association between intrauterine exposure to certain medications, including well-known teratogens such as retinoids and thalidomide. (3)

The occurrence of microtia is of public health concern for several reasons. It is a highly visible condition and is associated with potential psychosocial sequelae and stigma, as well as the burden of undergoing multiple surgeries. (4, 5, 6) In addition to the aesthetic considerations, associated moderate to severe hearing loss may affect communication, exacerbating psychosocial problems. The societal cost associated with managing microtia, and the possible sequelae, are likely to be considerable due to the need for management of hearing impairment and the need for multiple surgical procedures.

The availability of comprehensive patient registers in Sweden presents a unique opportunity to study this relatively rare congenital anomaly.

This PhD research project is divided into five sub-studies:

Prevalence and birth characteristics of patients with microtia in Sweden

This registry-based cohort study will study birth prevalence, abortion trends and survival over time in Sweden for children with microtia. It will describe birth characteristics of the children and other possible associated malformations/syndromes as well as the prevalence of hearing loss.

Maternal risk factors for microtia

This case-control study will investigate possible risk factors in pregnancy and risk of microtia in the foetus compared to controls.

The risk of neuropsychiatric disorders in individuals born with microtia

This case-control study will study the risk of developing a neuropsychiatric diagnosis in individuals born with microtia compared to controls.

Education level and socio-economic status in individuals with microtia

This case-control study will study the educational level and socio-economic status of people born with microtia with the aim of mapping the highest educational attainment and wage level of individuals born with microtia, as a proxy for the long-term psycho-social impact of the malformation.

Quality of life in patients with microtia

This prospective cohort study will review the Stockholm cohort of microtia patients between 2000-2023 to study quality of life through validated questionnaires. The results from the different domains of quality of life will be compared in groups with individuals who have undergone ear reconstruction surgery, patients with microtia who have opted against reconstruction and healthy individuals' estimation of the same form.

Through this epidemiological and long-term follow-up project we hope to identify risk factors, co-morbidities, and the possible long-term impact of the congenital malformation microtia. Microtia is a relatively uncommon condition and large studies are challenging. However, due to the extensive data available to us through national patient registries in Sweden we hope to be able to describe one of the largest cohorts in the literature. The research contained within this project will contribute to increasing knowledge about the underlying causes and the long-term consequences of being born with microtia. We hope to identify preventive measures and thus be able to provide better and more accurate information in future pre- and post-natal counselling. This is of great significance for affected children and their families as well as leading to potential healthcare financial benefits.

References

The epidemiology of anotia and microtia.
Harris J, Källén B, Robert E
J Med Genet 1996 Oct;33(10):809-13

Microtia: epidemiology and genetics.
Luquetti DV, Heike CL, Hing AV, Cunningham ML, Cox TC
Am J Med Genet A 2012 Jan;158A(1):124-39

Carey JC, Park AH, Muntz HR. 2006. External ear. In: Stevenson RE, editor. Human malformations and related anomalies. Oxford, New York: Oxford University Press. pp. 329–338.

Psychosocial outcomes among microtia patients of different ages and genders before ear reconstruction.
Li D, Chin W, Wu J, Zhang Q, Xu F, Xu Z, Zhang R
Aesthetic Plast Surg 2010 Oct;34(5):570-6

A prospective evaluation of psychosocial outcomes following ear reconstruction with rib cartilage in microtia.
Steffen A, Wollenberg B, König IR, Frenzel H
J Plast Reconstr Aesthet Surg 2010 Sep;63(9):1466-73

Psychosocial status of patients with unilateral and bilateral microtia before auricular reconstruction surgery.
Fan Y, Liu W, Fan X, Niu X, Chen X
Int J Pediatr Otorhinolaryngol 2021 Dec;151():110928

5. Facial palsy

6. Head and neck cancer and microvascular reconstruction

Malignancies in the head and neck region account for 2.3% of malignancies in Sweden which translates into about 1500 incident cases each year. Of these, 450 are cancers within the oral cavity. The incidence of these cancers has increased over time and the patients have a five-year survival rate of 62%, indicating highly malignant disease.

Treatment of these cancers include surgery, radiation and sometimes chemotherapy. Obtaining a surgical margin is important but can be very challenging due to the localization of the tumour in an area with multiple vital structures and little excess tissue available for wound closure. In some cases, reconstructive plastic surgery is therefore necessary. Immediate free flap reconstruction is the method of choice after major head and neck cancer. Reconstructive surgery can allow for large tumors to be resected, satisfying oncologic, functional and cosmetic goals.

In this PhD project, we will assess the quality of life of these patients over time. The flap donor sites also pose particular challenges to the surgeon, the patient and the health care system as these sites often take long to heal. A novel technique to repair donor sites will be assessed in two RCTs. Health economical evaluation from the health care providers perspective will also be undertaken.

7. Lymphedema

8. Melanoma

9. Radiation and tissue effects

10. Registers

a. Microsurgery registry

b. Breast implant registry

11. Sarcoma

Sarcoma, often referred to as ‘the loneliest cancer’ due to a prevalence of 1% among adult malignancies, encompasses a diverse group of primary malignancies with their origin in connective tissues. They are divided into soft tissue sarcomas (STS) and bone sarcomas (BS) depending on histological origin, with further subclassifications. Treatment is based upon combinations of surgical resection, radiation therapy, and chemotherapy according to the susceptibility of each sarcoma type. While radicality is a main principle of oncological surgery, sarcoma surgeons also aim to preserve functionality of the affected body part. Prognosis is relatively poor with estimates of overall 5-year survival being around 65%.

Postoperative complications following sarcoma surgery such as surgical site infection, seroma, reoperation, and delayed wound healing are common.

This project involves partners from reconstructive plastic surgery and orthopaedic surgeons and general surgeons at the Stockholm Sarcoma Centre and it represents one of the areas where work in Sweden is combined with work in Uganda. Two PhD students, Emmy Nyqvist and Madeleine Nguyen Hoang, are working on the project which includes several substudies. A few are outlined below.

Study 1. ERAS in sarcoma surgery

In this study, 300 study participants (15 years or older, with primary sarcoma of the extremities and trunk) will be recruited. The first half will receive standard care. The second half will receive care with an addition of an ERAS protocol. Outcomes will be followed up to one year postoperatively and include postoperative complications and quality of life among other things. Health economical evaluation of standard care versus ERAS will be performed.

Study 2. Angiosarcoma, surgical margins and reconstructive plastic surgery

Angiosarcoma (AS) is a rare and highly malignant form of soft tissue sarcoma. Many cases are secondary to previous radiation therapy. Few studies address the subject from a technical perspective, warranting a baseline investigation of 1) adequate excision margins during primary surgery and 2) the optimal reconstructive technique. This is a retrospective study that is carried out in partnership with the Stockholm Sarcoma Centre as well as partners at the university hospitals in Gothenburg and Lund.

Study 3. Sarcoma in secondary and tertiary level hospitals in Uganda

While sarcoma is a rare disease it still poses considerable challenges to orthopaedic and general surgeons in Uganda as the patients accumulate in orthopaedic centres. In this retrospective and primarily descriptive study, all patient managed for sarcoma of the extremities and trunk at the department of orthopaedics in Jinja Regional Referral Hospital and Mulago National Referral Hospital between 2014 and 2024 will be included. We expect that the sample will be 100 – 200 patients. Data will be collected from the operation theatre records, from the in-patient and the outpatient records. Data will include all available information which is most likely limited to age and sex of the patients, type of sarcoma, stage of disease and what treatment that was recommended and administered.